also called Felty syndrome, (FS) is rare autoimmune disease characterized by the triad of rheumatoid arthritis, enlargement of the spleen and too few neutrophils in the blood.
All ACUTE LYMPHOBLASTIC LEUKEMIA BY DR MAGDI SASIcardilogy
1. The document provides information about acute lymphocytic leukemia (ALL), the most common type of cancer in children. It describes the symptoms, signs, classification, and risk factors of ALL.
2. Common symptoms of ALL include fever, fatigue, easy bruising, frequent infections, and bone/joint pain. Physical signs may include pallor, organ enlargement, and lymphadenopathy.
3. ALL is classified according to the type of lymphocytes involved (B-cell vs T-cell lineage) and cellular features. The vast majority of ALL cases involve B-cell lymphocytes. Risk factors for ALL are generally unknown for most people.
Myelodysplastic syndromes are a group of myeloid disorders characterized by peripheral blood cytopenias and dysplasia in bone marrow cells. MDS results from clonal stem cell disorders that impair differentiation, causing low blood cell counts and risk of acute myeloid leukemia. The disease mainly affects the elderly and risk factors include genetic mutations, toxic exposures, and certain genetic syndromes. Diagnosis involves blood tests showing low counts of one or more cell types and bone marrow biopsy demonstrating dysplastic changes. Prognosis ranges from long-term survival to rapid progression; treatment focuses on supportive care like transfusions as well as hypomethylating agents or stem cell transplantation in high-risk cases.
This document discusses cryoglobulinemic vasculitis and renal disease. It begins with objectives and history of cryoglobulins and cryoglobulinemic disease. It then covers definitions, classifications, etiology, epidemiology, pathogenesis, clinical features, diagnosis, prognosis, and management of cryoglobulinemic vasculitis. The pathogenesis involves cryoglobulin precipitation, immune complex formation, and inflammation mediated by infections like HCV, autoimmune diseases, and malignancies. Clinical features commonly include purpura, arthralgia, weakness, and renal involvement.
This document discusses secondary glomerular nephropathies caused by various autoimmune diseases and systemic conditions. It describes how systemic lupus erythematosus is a common cause of glomerulonephritis mediated by circulating immune complexes. It also discusses Goodpasture's disease, Schönlein-Henoch purpura, cryoglobulinemic vasculitis, and how they can result in various types of glomerular injury and nephropathies. The prognosis and treatment of these secondary glomerular nephropathies depends on the underlying disease and severity of renal involvement.
This document summarizes a study on acute myeloid leukemia (AML) in children. It discusses:
1. AML arises from mutations in hematopoietic precursors that confer proliferation/survival advantages and impair differentiation/apoptosis.
2. Treatment involves intensive chemotherapy induction followed by consolidation therapy, with improved outcomes over decades from 60% survival rates.
3. However, questions remain about optimal drug doses and timing, as well as the role of stem cell transplantation and risk-directed therapy.
Acute lymphoblastic leukemia in children ch2tilayem
This document summarizes acute lymphoblastic leukemia (ALL) in children. It discusses the classification of ALL which includes morphological, immunological, and genetic classification. Morphological classification includes the FAB system which defines three categories of lymphoblasts. Immunological classification is based on monoclonal antibodies and distinguishes between B-lineage, T-lineage, and myeloid phenotypes. Genetic classification identifies cytogenetic abnormalities which are important for diagnosis, risk assessment, treatment and prognosis, with some abnormalities associated with favorable prognosis and others with poor prognosis.
1. Secondary glomerular nephropathies are glomerular injuries caused by multiorgan diseases rather than primarily affecting the kidneys. Common causes include systemic lupus erythematosus, vasculitis, Goodpasture's disease, and Schönlein-Henoch syndrome.
2. Systemic lupus erythematosus commonly involves the kidneys and is a leading cause of morbidity and mortality. Lupus nephropathy can be classified into six types based on pathology findings. Clinical manifestations vary depending on nephropathy type.
3. Schönlein-Henoch syndrome is characterized by skin, joint, and digestive manifestations often accompanied by glomerul
Chronic lymphocytic leukemia (CLL) is the most common type of leukemia in adults. It is characterized by the accumulation of abnormal B lymphocytes that are immuno-incompetent. CLL typically presents insidiously and is often diagnosed incidentally on routine blood tests. The diagnosis is based on blood tests showing mature lymphocytosis with characteristic cell markers. Prognosis depends on disease stage - stage A patients have a normal life expectancy while stage C patients have a median survival of 2-3 years. Initial treatment typically involves oral chemotherapy such as chlorambucil for patients requiring treatment.
All ACUTE LYMPHOBLASTIC LEUKEMIA BY DR MAGDI SASIcardilogy
1. The document provides information about acute lymphocytic leukemia (ALL), the most common type of cancer in children. It describes the symptoms, signs, classification, and risk factors of ALL.
2. Common symptoms of ALL include fever, fatigue, easy bruising, frequent infections, and bone/joint pain. Physical signs may include pallor, organ enlargement, and lymphadenopathy.
3. ALL is classified according to the type of lymphocytes involved (B-cell vs T-cell lineage) and cellular features. The vast majority of ALL cases involve B-cell lymphocytes. Risk factors for ALL are generally unknown for most people.
Myelodysplastic syndromes are a group of myeloid disorders characterized by peripheral blood cytopenias and dysplasia in bone marrow cells. MDS results from clonal stem cell disorders that impair differentiation, causing low blood cell counts and risk of acute myeloid leukemia. The disease mainly affects the elderly and risk factors include genetic mutations, toxic exposures, and certain genetic syndromes. Diagnosis involves blood tests showing low counts of one or more cell types and bone marrow biopsy demonstrating dysplastic changes. Prognosis ranges from long-term survival to rapid progression; treatment focuses on supportive care like transfusions as well as hypomethylating agents or stem cell transplantation in high-risk cases.
This document discusses cryoglobulinemic vasculitis and renal disease. It begins with objectives and history of cryoglobulins and cryoglobulinemic disease. It then covers definitions, classifications, etiology, epidemiology, pathogenesis, clinical features, diagnosis, prognosis, and management of cryoglobulinemic vasculitis. The pathogenesis involves cryoglobulin precipitation, immune complex formation, and inflammation mediated by infections like HCV, autoimmune diseases, and malignancies. Clinical features commonly include purpura, arthralgia, weakness, and renal involvement.
This document discusses secondary glomerular nephropathies caused by various autoimmune diseases and systemic conditions. It describes how systemic lupus erythematosus is a common cause of glomerulonephritis mediated by circulating immune complexes. It also discusses Goodpasture's disease, Schönlein-Henoch purpura, cryoglobulinemic vasculitis, and how they can result in various types of glomerular injury and nephropathies. The prognosis and treatment of these secondary glomerular nephropathies depends on the underlying disease and severity of renal involvement.
This document summarizes a study on acute myeloid leukemia (AML) in children. It discusses:
1. AML arises from mutations in hematopoietic precursors that confer proliferation/survival advantages and impair differentiation/apoptosis.
2. Treatment involves intensive chemotherapy induction followed by consolidation therapy, with improved outcomes over decades from 60% survival rates.
3. However, questions remain about optimal drug doses and timing, as well as the role of stem cell transplantation and risk-directed therapy.
Acute lymphoblastic leukemia in children ch2tilayem
This document summarizes acute lymphoblastic leukemia (ALL) in children. It discusses the classification of ALL which includes morphological, immunological, and genetic classification. Morphological classification includes the FAB system which defines three categories of lymphoblasts. Immunological classification is based on monoclonal antibodies and distinguishes between B-lineage, T-lineage, and myeloid phenotypes. Genetic classification identifies cytogenetic abnormalities which are important for diagnosis, risk assessment, treatment and prognosis, with some abnormalities associated with favorable prognosis and others with poor prognosis.
1. Secondary glomerular nephropathies are glomerular injuries caused by multiorgan diseases rather than primarily affecting the kidneys. Common causes include systemic lupus erythematosus, vasculitis, Goodpasture's disease, and Schönlein-Henoch syndrome.
2. Systemic lupus erythematosus commonly involves the kidneys and is a leading cause of morbidity and mortality. Lupus nephropathy can be classified into six types based on pathology findings. Clinical manifestations vary depending on nephropathy type.
3. Schönlein-Henoch syndrome is characterized by skin, joint, and digestive manifestations often accompanied by glomerul
Chronic lymphocytic leukemia (CLL) is the most common type of leukemia in adults. It is characterized by the accumulation of abnormal B lymphocytes that are immuno-incompetent. CLL typically presents insidiously and is often diagnosed incidentally on routine blood tests. The diagnosis is based on blood tests showing mature lymphocytosis with characteristic cell markers. Prognosis depends on disease stage - stage A patients have a normal life expectancy while stage C patients have a median survival of 2-3 years. Initial treatment typically involves oral chemotherapy such as chlorambucil for patients requiring treatment.
Recent advances in treatment of Myelodysplastic Syndrome. Dr. Zhijian Xiaospa718
The document discusses recent advances in the treatment of myelodysplastic syndrome (MDS). It covers several key points:
1) Patient characteristics like age, performance status, and comorbidities are important considerations in determining treatment approach and ability to tolerate intensive therapies.
2) Treatment strategies for lower-risk and higher-risk MDS aim to improve blood counts, quality of life, delay disease progression, and prolong survival.
3) Supportive care focuses on reducing morbidity from low blood counts while active therapies aim to alter the natural history of MDS and improve survival.
4) Allogeneic stem cell transplantation remains the only potentially curative treatment but the decision to transplant requires considering age,
Austin Arthritis is an open access, peer reviewed, scholarly journal dedicated to publish articles covering all areas of Arthritis.
The journal aims to promote research communications and provide a forum for doctors, researchers, physicians and healthcare professionals to find most recent advances & happenings in all areas of Arthritis. Austin Arthritis accepts original research articles, reviews, mini reviews, case reports and rapid communication covering all aspects of Arthritis.
Austin Arthritis strongly supports the scientific up gradation and fortification in related scientific research community by enhancing access to peer reviewed scientific literary works. Austin Publishing Group also brings universally peer reviewed journals under one roof thereby promoting knowledge sharing, mutual promotion of multidisciplinary science
Leukaemia is a cancer of the white blood cells characterized by the overproduction of abnormal white blood cells in the bone marrow. There are four main types of leukaemia - acute lymphocytic leukaemia, chronic lymphocytic leukaemia, acute myeloid leukaemia, and chronic myeloid leukaemia. The document discusses the signs, symptoms, diagnosis, and treatment for each type of leukaemia. It also covers oral manifestations that may be seen and dental management considerations for patients with leukaemia.
- Whipple's disease is a rare infection caused by the bacterium Tropheryma whipplei. It was first reported in 1907 but the bacterium was not identified until 1991 and not cultured until 2000.
- Classic symptoms include weight loss, diarrhea, arthralgia or arthritis. Neurological involvement occurs in 10-40% of cases and can include cognitive changes, supranuclear ophthalmoplegia, and altered consciousness.
- T. whipplei can also cause heart infections in 17-55% of cases, with infiltration of the pericardium, myocardium, and endocardium. Endocarditis from T. whipplei is a potential cause of sudden
Dr. Feroze Momin presents Chronic Lymphocytic Leukemia - Review and new Insights.
To read about Dr. Feroze Momin: http://conquercancers.com/ourdoctorso1.html
To read about Cancer Treatment Center in Michigan:
http://conquercancers.com
This document provides an overview of the myeloid malignancies, including myeloproliferative neoplasms (MPNs), myelodysplastic syndrome (MDS), and acute myeloid leukemia (AML). It describes key characteristics of each condition, such as increased mature cells in MPNs, decreased blood cells in MDS, and presence of immature cells in AML. Diagnostic criteria and classification systems for AML, including the 2008 WHO classification, are reviewed. Risk stratification in AML and standard treatment approaches are also summarized. Two clinical cases are then presented and discussed in detail.
This document provides an overview of lymphoid leukemias. It begins with an introduction to lymphoid leukemias and compares them to myeloid leukemias. It then discusses the subtypes of acute and chronic lymphoid leukemias in more detail. Key points include distinguishing between B-cell and T-cell acute lymphoblastic leukemias, important genetic alterations in ALL, and initial therapy approaches. Chronic lymphoid leukemias such as CLL are also reviewed, covering topics like diagnostic criteria, prognostic factors, and standard treatment regimens.
an overview of Lupus for journalist
Lupus has a wide spectrum of manifestation. Some mild but in most cases it has a high impact of life and quality of life
1. Stroke is a common complication in cancer patients that can be caused by direct tumor effects, coagulation disorders, infections, or cancer therapies.
2. Studies have found strokes occur in 3.5-15% of cancer patients, with ischemic strokes being more common than hemorrhagic. Risk factors include certain cancers like lung cancer or blood cancers.
3. Diagnosis involves blood tests, imaging like MRI, and treatment focuses on anticoagulation though established treatments are still lacking. Differentiating between causes like coagulopathies or conventional vascular risk factors is important.
This document discusses post-transplant lymphoproliferative disorder (PTLD) in renal transplant recipients. It defines PTLD as a complication of solid organ transplantation and hematopoietic stem cell transplantation caused by Epstein-Barr virus infection or reactivation. The document covers the clinical presentation, diagnosis, risk factors, histopathological subtypes, management including reduction of immunosuppression, antiviral therapy, monoclonal antibodies, chemotherapy, and radiation therapy of PTLD.
1. Systemic sclerosis is a disease characterized by abnormalities of blood vessels, fibrosis of skin and internal organs, and activation of the immune system.
2. It is classified as limited or diffuse cutaneous systemic sclerosis based on the extent of skin involvement and rate of progression.
3. Clinical features include Raynaud's phenomenon, skin thickening, joint and muscle involvement, as well as pulmonary, cardiac, gastrointestinal, and renal complications.
Leukemia is a cancer of the blood or bone marrow characterized by an abnormal increase of immature white blood cells. There are several types classified by how quickly the disease develops (acute or chronic) and the affected blood cell type (lymphoid or myeloid). Common types include acute lymphoblastic leukemia, acute myeloid leukemia, chronic lymphocytic leukemia, and chronic myeloid leukemia. Treatment involves chemotherapy, radiation therapy, or stem cell transplantation and varies depending on the leukemia type and stage.
The document discusses recent advances in myelodysplastic syndromes (MDS), including new risk stratification models, prognostic factors, and therapeutic options for lower-risk and higher-risk MDS such as lenalidomide for lower-risk MDS and azacitidine or allogeneic stem cell transplantation for higher-risk MDS. Clinical trials have shown that lenalidomide can induce transfusion independence in patients with MDS and del(5q) abnormality and azacitidine improves overall survival compared to
Transient leukemia in down syndrome report of two casesRogelio Cotaya
This document reports on two cases of transient leukemia in infants with Down syndrome. Both infants presented with high white blood cell counts and many blast cells in their blood. No specific treatment was given and in both cases the blast cells disappeared and blood counts returned to normal within a few months. Transient leukemia is a unique condition seen in 10% of newborns with Down syndrome where leukemia develops in the first few days of life but resolves spontaneously within a few months without treatment.
Down's syndrome is associated with a 10-20 fold increased risk of leukaemia, especially acute myeloid leukaemia (AMKL). Nearly all cases of AMKL and transient leukaemia in Down's syndrome patients have mutations in the GATA1 gene, which is important for differentiation of blood cells. The mutations lead to expression of a shortened GATA1 protein and block differentiation. While a GATA1 mutation is necessary, it is not sufficient to cause leukaemia by itself, suggesting an additional genetic "hit" is required along with the presence of trisomy 21. Future research aims to identify the second genetic change and better understand how trisomy 21 contributes to leukaemic transformation.
Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon lung disease that typically affects young adult smokers. It is characterized by inflammatory lesions surrounding small airways and blood vessels that can progress to interstitial fibrosis. Common symptoms include cough, dyspnea, and weight loss. Diagnosis involves imaging showing nodules and cysts predominantly in the mid and upper lung zones. Transbronchial biopsy can confirm the presence of Langerhans cells. Cessation of smoking is the primary treatment and can lead to remission of symptoms.
This document summarizes information about chronic myeloid leukemia (CML), including its history, clinical presentation, diagnosis, treatment and case study. It notes that CML is characterized by the Philadelphia chromosome, which was first observed in 1960. Most patients present with fatigue and splenomegaly and are diagnosed via blood tests showing leukocytosis. Treatment of chronic phase CML initially involves tyrosine kinase inhibitors like imatinib or nilotinib, with the goals of achieving complete hematological and cytogenetic responses and major molecular response. A case study described a 35-year-old female patient who achieved MR4 status after 6 months of nilotinib treatment with no side effects.
Management of acute lymphoblatic leukemia with light on etiology, clinical features, diagnosis and different aspects of management including chemotherapy and radiation therapy
Rheumatological diseases can affect any organ system and cause life-threatening complications requiring intensive care. The document discusses several key rheumatological conditions that intensivists should be aware of including:
1) Macrophage activation syndrome, a potentially lethal complication seen in rheumatological diseases characterized by uncontrolled inflammation.
2) Scleroderma renal crisis, a rheumatological emergency caused by thickening of renal arteries leading to hypertension and kidney failure.
3) Catastrophic antiphospholipid syndrome, a rare but severe form of antiphospholipid antibody syndrome involving rapid multi-organ failure.
Early recognition and management of these rheumatological conditions is important to prevent poor outcomes for patients in intensive care
The document provides information on the medical treatment of ulcerative colitis. It discusses the use of 5-aminosalicylic acid compounds like sulfasalazine and newer formulations like mesalamine as first-line treatments. Corticosteroids are described as potent agents used for acute treatment of moderate to severe flare-ups. The goal of treatment is to decrease inflammation and symptoms to improve quality of life, selecting options based on severity and side effects.
Recent advances in treatment of Myelodysplastic Syndrome. Dr. Zhijian Xiaospa718
The document discusses recent advances in the treatment of myelodysplastic syndrome (MDS). It covers several key points:
1) Patient characteristics like age, performance status, and comorbidities are important considerations in determining treatment approach and ability to tolerate intensive therapies.
2) Treatment strategies for lower-risk and higher-risk MDS aim to improve blood counts, quality of life, delay disease progression, and prolong survival.
3) Supportive care focuses on reducing morbidity from low blood counts while active therapies aim to alter the natural history of MDS and improve survival.
4) Allogeneic stem cell transplantation remains the only potentially curative treatment but the decision to transplant requires considering age,
Austin Arthritis is an open access, peer reviewed, scholarly journal dedicated to publish articles covering all areas of Arthritis.
The journal aims to promote research communications and provide a forum for doctors, researchers, physicians and healthcare professionals to find most recent advances & happenings in all areas of Arthritis. Austin Arthritis accepts original research articles, reviews, mini reviews, case reports and rapid communication covering all aspects of Arthritis.
Austin Arthritis strongly supports the scientific up gradation and fortification in related scientific research community by enhancing access to peer reviewed scientific literary works. Austin Publishing Group also brings universally peer reviewed journals under one roof thereby promoting knowledge sharing, mutual promotion of multidisciplinary science
Leukaemia is a cancer of the white blood cells characterized by the overproduction of abnormal white blood cells in the bone marrow. There are four main types of leukaemia - acute lymphocytic leukaemia, chronic lymphocytic leukaemia, acute myeloid leukaemia, and chronic myeloid leukaemia. The document discusses the signs, symptoms, diagnosis, and treatment for each type of leukaemia. It also covers oral manifestations that may be seen and dental management considerations for patients with leukaemia.
- Whipple's disease is a rare infection caused by the bacterium Tropheryma whipplei. It was first reported in 1907 but the bacterium was not identified until 1991 and not cultured until 2000.
- Classic symptoms include weight loss, diarrhea, arthralgia or arthritis. Neurological involvement occurs in 10-40% of cases and can include cognitive changes, supranuclear ophthalmoplegia, and altered consciousness.
- T. whipplei can also cause heart infections in 17-55% of cases, with infiltration of the pericardium, myocardium, and endocardium. Endocarditis from T. whipplei is a potential cause of sudden
Dr. Feroze Momin presents Chronic Lymphocytic Leukemia - Review and new Insights.
To read about Dr. Feroze Momin: http://conquercancers.com/ourdoctorso1.html
To read about Cancer Treatment Center in Michigan:
http://conquercancers.com
This document provides an overview of the myeloid malignancies, including myeloproliferative neoplasms (MPNs), myelodysplastic syndrome (MDS), and acute myeloid leukemia (AML). It describes key characteristics of each condition, such as increased mature cells in MPNs, decreased blood cells in MDS, and presence of immature cells in AML. Diagnostic criteria and classification systems for AML, including the 2008 WHO classification, are reviewed. Risk stratification in AML and standard treatment approaches are also summarized. Two clinical cases are then presented and discussed in detail.
This document provides an overview of lymphoid leukemias. It begins with an introduction to lymphoid leukemias and compares them to myeloid leukemias. It then discusses the subtypes of acute and chronic lymphoid leukemias in more detail. Key points include distinguishing between B-cell and T-cell acute lymphoblastic leukemias, important genetic alterations in ALL, and initial therapy approaches. Chronic lymphoid leukemias such as CLL are also reviewed, covering topics like diagnostic criteria, prognostic factors, and standard treatment regimens.
an overview of Lupus for journalist
Lupus has a wide spectrum of manifestation. Some mild but in most cases it has a high impact of life and quality of life
1. Stroke is a common complication in cancer patients that can be caused by direct tumor effects, coagulation disorders, infections, or cancer therapies.
2. Studies have found strokes occur in 3.5-15% of cancer patients, with ischemic strokes being more common than hemorrhagic. Risk factors include certain cancers like lung cancer or blood cancers.
3. Diagnosis involves blood tests, imaging like MRI, and treatment focuses on anticoagulation though established treatments are still lacking. Differentiating between causes like coagulopathies or conventional vascular risk factors is important.
This document discusses post-transplant lymphoproliferative disorder (PTLD) in renal transplant recipients. It defines PTLD as a complication of solid organ transplantation and hematopoietic stem cell transplantation caused by Epstein-Barr virus infection or reactivation. The document covers the clinical presentation, diagnosis, risk factors, histopathological subtypes, management including reduction of immunosuppression, antiviral therapy, monoclonal antibodies, chemotherapy, and radiation therapy of PTLD.
1. Systemic sclerosis is a disease characterized by abnormalities of blood vessels, fibrosis of skin and internal organs, and activation of the immune system.
2. It is classified as limited or diffuse cutaneous systemic sclerosis based on the extent of skin involvement and rate of progression.
3. Clinical features include Raynaud's phenomenon, skin thickening, joint and muscle involvement, as well as pulmonary, cardiac, gastrointestinal, and renal complications.
Leukemia is a cancer of the blood or bone marrow characterized by an abnormal increase of immature white blood cells. There are several types classified by how quickly the disease develops (acute or chronic) and the affected blood cell type (lymphoid or myeloid). Common types include acute lymphoblastic leukemia, acute myeloid leukemia, chronic lymphocytic leukemia, and chronic myeloid leukemia. Treatment involves chemotherapy, radiation therapy, or stem cell transplantation and varies depending on the leukemia type and stage.
The document discusses recent advances in myelodysplastic syndromes (MDS), including new risk stratification models, prognostic factors, and therapeutic options for lower-risk and higher-risk MDS such as lenalidomide for lower-risk MDS and azacitidine or allogeneic stem cell transplantation for higher-risk MDS. Clinical trials have shown that lenalidomide can induce transfusion independence in patients with MDS and del(5q) abnormality and azacitidine improves overall survival compared to
Transient leukemia in down syndrome report of two casesRogelio Cotaya
This document reports on two cases of transient leukemia in infants with Down syndrome. Both infants presented with high white blood cell counts and many blast cells in their blood. No specific treatment was given and in both cases the blast cells disappeared and blood counts returned to normal within a few months. Transient leukemia is a unique condition seen in 10% of newborns with Down syndrome where leukemia develops in the first few days of life but resolves spontaneously within a few months without treatment.
Down's syndrome is associated with a 10-20 fold increased risk of leukaemia, especially acute myeloid leukaemia (AMKL). Nearly all cases of AMKL and transient leukaemia in Down's syndrome patients have mutations in the GATA1 gene, which is important for differentiation of blood cells. The mutations lead to expression of a shortened GATA1 protein and block differentiation. While a GATA1 mutation is necessary, it is not sufficient to cause leukaemia by itself, suggesting an additional genetic "hit" is required along with the presence of trisomy 21. Future research aims to identify the second genetic change and better understand how trisomy 21 contributes to leukaemic transformation.
Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon lung disease that typically affects young adult smokers. It is characterized by inflammatory lesions surrounding small airways and blood vessels that can progress to interstitial fibrosis. Common symptoms include cough, dyspnea, and weight loss. Diagnosis involves imaging showing nodules and cysts predominantly in the mid and upper lung zones. Transbronchial biopsy can confirm the presence of Langerhans cells. Cessation of smoking is the primary treatment and can lead to remission of symptoms.
This document summarizes information about chronic myeloid leukemia (CML), including its history, clinical presentation, diagnosis, treatment and case study. It notes that CML is characterized by the Philadelphia chromosome, which was first observed in 1960. Most patients present with fatigue and splenomegaly and are diagnosed via blood tests showing leukocytosis. Treatment of chronic phase CML initially involves tyrosine kinase inhibitors like imatinib or nilotinib, with the goals of achieving complete hematological and cytogenetic responses and major molecular response. A case study described a 35-year-old female patient who achieved MR4 status after 6 months of nilotinib treatment with no side effects.
Management of acute lymphoblatic leukemia with light on etiology, clinical features, diagnosis and different aspects of management including chemotherapy and radiation therapy
Rheumatological diseases can affect any organ system and cause life-threatening complications requiring intensive care. The document discusses several key rheumatological conditions that intensivists should be aware of including:
1) Macrophage activation syndrome, a potentially lethal complication seen in rheumatological diseases characterized by uncontrolled inflammation.
2) Scleroderma renal crisis, a rheumatological emergency caused by thickening of renal arteries leading to hypertension and kidney failure.
3) Catastrophic antiphospholipid syndrome, a rare but severe form of antiphospholipid antibody syndrome involving rapid multi-organ failure.
Early recognition and management of these rheumatological conditions is important to prevent poor outcomes for patients in intensive care
The document provides information on the medical treatment of ulcerative colitis. It discusses the use of 5-aminosalicylic acid compounds like sulfasalazine and newer formulations like mesalamine as first-line treatments. Corticosteroids are described as potent agents used for acute treatment of moderate to severe flare-ups. The goal of treatment is to decrease inflammation and symptoms to improve quality of life, selecting options based on severity and side effects.
Chronic lymphocytic leukemia (CLL) is the most common type of leukemia in adults. It has an average incidence of 2.7 per 100,000 people in the US and is more common in older men. CLL accounts for about 0.8% of all cancers and 30% of leukemias. The neoplastic cells are typically B-cell lymphocytes. Farming exposure and hepatitis C may play a role in etiology. CLL has various genetic abnormalities that affect prognosis. Clinical features include lymphadenopathy, fatigue, infections. Diagnosis requires a sustained lymphocytosis above 5000/uL. Treatment is indicated for symptomatic disease, doubling time under 6 months, or disease complications. Prognostic factors
Leptospirosis - clinical manifestations and diagnosis.pdfJim Jacob Roy
Leptospirosis is a commonly encountered infection , especially in tropical regions.
In this document , the clinical manifestations and diagnosis of leptospirosis is described.
The modified FAINE'S criteria is also described at the end.
Non-Hodgkin lymphoma (NHL) represents an abnormal proliferation of B- or T-lymphocytes that can be low or high-grade. The incidence increases with age and is rising overall by 3% per year. NHL is often disseminated at diagnosis and can involve lymph nodes, bone marrow, gut, and other organs. Diagnostic tests include immunophenotyping, immunoglobulin levels, and HIV testing. Treatment depends on grade, with chemotherapy such as CHOP plus rituximab for high-grade NHL, while low-grade NHL may only require monitoring. Prognosis ranges from 10-year survival for low-grade to 75% 5-year survival for low-risk high
Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by autoantibodies against nuclear antigens. It can affect multiple organ systems. The presentation, pathogenesis, clinical features, investigations, management, and prognosis of SLE are discussed in detail in this document. Key points include that SLE predominantly affects women and certain races disproportionately. Genetic and environmental factors contribute to its pathogenesis. A variety of clinical manifestations are described affecting the skin, joints, kidneys, lungs, and other organs. Management involves controlling symptoms, preventing organ damage, and maintaining remission, often through medications like hydroxychloroquine and corticosteroids. Prognosis has improved over time but risks of infection and accelerated
This document summarizes recent updates on lupus nephritis (LN), a form of kidney disease caused by systemic lupus erythematosus (SLE) that can lead to kidney failure. It discusses the epidemiology of SLE and LN, noting higher rates in women and certain racial groups. Genetic factors that may influence susceptibility and disease course are also reviewed. The pathogenesis of LN is complex and involves interferon response, neutrophil activation, and impaired clearance of cellular debris. Timely diagnosis via urine testing and kidney biopsy is important for treatment and preventing long-term kidney damage.
Sarcoidosis and IgG4-related diseases are inflammatory conditions characterized by granuloma formation. Sarcoidosis is a multisystem disorder involving lungs in over 90% of cases and skin, eyes, and liver in about a third of patients each. It is thought to be triggered by an infectious or environmental agent in a genetically susceptible host. IgG4-related disease is a fibroinflammatory condition that can affect virtually any organ, forming tumefactive lesions. Treatment for both conditions typically involves corticosteroids, with immunosuppressants used for chronic or resistant cases.
This document discusses acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML). It describes the epidemiology, pathogenesis, classification, clinical presentation, diagnosis, prognostic factors, management and outcomes of both ALL and AML in children. ALL is more common and has a better prognosis than AML. Prognostic factors for ALL include age, white blood cell count, specific genetic mutations and response to initial treatment. Treatment involves induction, consolidation, central nervous system prophylaxis and maintenance therapy over 2-3 years.
This document provides information on mixed connective tissue disease (MCTD). It discusses the definition, etiology, pathophysiology, diagnosis, treatment and prognosis of MCTD. MCTD is a rare autoimmune disease with overlapping features of at least two connective tissue diseases like SLE, SSc, PM and DM. It is characterized by the presence of anti-U1 RNP antibodies. Symptoms can affect multiple organ systems. Diagnosis involves assessing clinical features and antibody levels. Treatment aims to control symptoms and is tailored based on organ involvement. Prognosis varies, with some patients experiencing complete resolution while others face life-threatening complications like pulmonary hypertension.
This document provides an overview of rheumatoid arthritis (RA). It discusses the history and first descriptions of RA. The epidemiology section notes that RA affects 0.5-1% of adults worldwide and is more common in females. Genetics plays a role, as the risk is increased in relatives and twin studies show a 60% genetic contribution. The HLA-DRB1 gene confers the greatest risk. Environmental factors like smoking can also increase risk. The pathogenesis involves a breakdown of self-tolerance leading to autoantibodies like rheumatoid factor and anti-CCP antibodies. T cells and cytokines like TNF-alpha promote inflammation and joint damage. Clinical features include variable onset patterns and progressive joint damage over time, sometimes leading
This document provides an overview of systemic lupus erythematosus (SLE) and neuropsychiatric SLE (NPSLE). It discusses the pathogenesis, epidemiology, classification, clinical manifestations, diagnosis, and management approaches for NPSLE. Regarding management, it describes general treatment approaches as well as specific therapies aimed at inflammatory NPSLE such as corticosteroids, immunosuppressants, and intravenous immunoglobulin. It also covers primary and secondary prevention strategies as well as treatments targeting ischemic events like antiplatelet agents and anticoagulants.
8-Guideline for elaborate SLE management.pptBosan Khalid
1. The document provides information about systemic lupus erythematosus (SLE) including its epidemiology, symptoms, organ involvement, diagnostic tests, and disease management.
2. SLE most commonly affects women of childbearing age and has a variety of clinical manifestations involving the skin, joints, kidneys, and other organ systems.
3. Diagnosis involves assessing clinical signs and symptoms along with serological tests like ANA and anti-DNA antibodies. Disease activity and organ involvement help guide treatment approaches.
Systemic lupus erythematosus (SLE) is the prototypic multisystem autoimmune disorder with a broad spectrum of clinical presentations encompassing almost all organs and tissues.
The extreme heterogeneity of the disease has led some investigators to propose that SLE represents a syndrome rather than a single disease.
Lupus was first recognised as a systemic disease with visceral manifestations by Moriz Kaposi (1837–1902).
This document provides information on acute rheumatic fever (RF), including its causes, presentation, diagnosis, treatment, and prognosis. RF is an autoimmune disease that occurs after a streptococcal throat infection in genetically susceptible individuals. It commonly affects children ages 5-15 and can involve the heart, joints, brain, and skin. Diagnosis requires supporting evidence of a recent streptococcal infection via antibody testing. Treatment involves anti-inflammatory drugs like aspirin and corticosteroids. Recurrences increase the risk of rheumatic heart disease, so long-term antibiotic prophylaxis is important. The prognosis depends on initial severity and recurrences, with carditis carrying the greatest risk of permanent heart damage.
Focal segmental glomerulosclerosis (FSGS) accounts for 20-40% of nephrotic syndrome cases in adults and children. It can be primary/idiopathic or secondary to various causes. Histologic variants include classic, collapsing, tip, perihilar, and cellular forms. Prognosis depends on factors like proteinuria level, kidney function, and fibrosis. While untreated FSGS often leads to kidney failure, treatment with steroids, immunosuppressants, or plasmapheresis can induce remission. Recurrence is a risk after kidney transplantation, so candidates are warned though transplantation is not precluded.
Systemic lupus erythematosus (SLE) is an autoimmune disease that can damage any body organ by generating autoantibodies and immune complexes. It predominantly affects women aged 15-65 and has the highest prevalence in African-American and Afro-Caribbean women. While the cause is unknown, genetic, environmental, and hormonal factors likely play a role. Symptoms vary but can include rashes, fatigue, joint pain, and organ involvement. Diagnosis involves clinical criteria and serological markers. Treatment focuses on controlling disease activity and inflammation using medications like NSAIDs, antimalarials, corticosteroids, and immunosuppressants.
Systemic Lupus Erythematosus (SLE) is a multi-gene autoimmune disease caused by a combination of genetic and environmental factors. It is characterized by abnormal immune responses that result in inflammation and damage to various organs. Diagnosis requires meeting 4 out of 11 classification criteria relating to clinical symptoms and blood markers. Management aims to induce remission of acute flares, maintain improvements to suppress symptoms, and prevent organ damage. Treatment choices depend on the severity and potential reversibility of manifestations. The goal is controlling symptoms without cure since complete sustained remission is rare.
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There is increasing confidence that cell therapies will soon play a role in the treatment of autoimmune disorders, but the extent of this impact remains to be seen. Early readouts on autologous CAR-Ts in lupus are encouraging, but manufacturing and cost limitations are likely to restrict access to highly refractory patients. Allogeneic CAR-Ts have the potential to broaden access to earlier lines of treatment due to their inherent cost benefits, however they will need to demonstrate comparable or improved efficacy to established modalities.
In addition to infrastructure and capacity constraints, CAR-Ts face a very different risk-benefit dynamic in autoimmune compared to oncology, highlighting the need for tolerable therapies with low adverse event risk. CAR-NK and Treg-based therapies are also being developed in certain autoimmune disorders and may demonstrate favorable safety profiles. Several novel non-cell therapies such as bispecific antibodies, nanobodies, and RNAi drugs, may also offer future alternative competitive solutions with variable value propositions.
Widespread adoption of cell therapies will not only require strong efficacy and safety data, but also adapted pricing and access strategies. At oncology-based price points, CAR-Ts are unlikely to achieve broad market access in autoimmune disorders, with eligible patient populations that are potentially orders of magnitude greater than the number of currently addressable cancer patients. Developers have made strides towards reducing cell therapy COGS while improving manufacturing efficiency, but payors will inevitably restrict access until more sustainable pricing is achieved.
Despite these headwinds, industry leaders and investors remain confident that cell therapies are poised to address significant unmet need in patients suffering from autoimmune disorders. However, the extent of this impact on the treatment landscape remains to be seen, as the industry rapidly approaches an inflection point.
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4. Risk factors for FS include the following:
RF positivity in high titers
Long-standing disease
Aggressive and erosive synovitis – Patient with FS may present with mild RA,
but FS is clearly associated with severe disease and extra-articular
manifestations
Human leukocyte antigen (HLA)-DR4 positivity and DR4 homozygosity –
This may be due to the presence of HLA-DR4 in patients who have severe
disease
Extra-articular RA manifestations
5. Pathophysiology of FS associated neutropenia is multifactorial
including
increased neutrophil sequestration secondary to splenomegaly,
peripheral destruction of neutrophils,
and failure of bone marrow to produce neutrophils.
6. 1-3 % of RA patients are complicated with FS with an estimated prevalence
of 10 per 100,000 populations .
Comparing to the prevalence for systemic lupus erythematosus (SLE) (52
cases per 100,000 population),
It is not plausible to estimate the real incidence of FS among RA population
since a large proportion of patients with FS are asymptomatic.
Evidence use of immunosuppressive agents in RA global incidence of FS is
decreasing.
FS is rare in African-American patients
Caucasians are affected more than blacks .
Early occurrence of FS is more probable in males than females, but FS is
three times more common in females than the males.
HLA-DR4 genotype is strongly associated with FS, but this association is
not found in LGL leukemia without arthritis.
Mean age of patients with FS is within fifth to seventh decades.
7. Although FS is a severe form of RA, it can be asymptomatic.
Polyarthicular joint disease in the setting of severe seropositive
RA complicated with neutropenia and infection is the hallmark
of FS.
Erosive RA is evident on radiographic images of involved
joints.
Splenomegaly is not always present . Splenomegaly can occur
in RA without FS or may indicate other complications such as
liver cirrhosis, brucellosis, visceral leishmaniasis, bacterial
endocarditis, histoplasmosis or amyloidosis.
Abdominal pain can be a manifestation of splenomegaly or
splenic infarcts.
Weight loss may be prominent.
Extraarticular manifestation of RA could be evident;
hepatomegaly, lymphadenopathy, episcleritis, sicca syndrome,
eye lid necrosis , pleuritis, neuropathy, portal hypertension,
skin hyperpigmentation, leg ulcers, and vasculitis are common
in FS.
CLINICAL MANIFESTATIONS AND COMPLICATIONS
8. Liver involvement such as portal fibrosis and cirrhosis can
be another extra articular manifestation of FS . Infection is
more common in FS than RA and skin, pulmonary tract
and mouth are among the most common sites of infection.
hypocomplementemia, leg ulcers and concomitant steroid
use, impaired phagocytosis and intracellular killing,
impaired chemotaxis and superoxide production may also
play some role to increase the risk of infections ..
Procalcitonin could also indicate concurrent infection in
neutropenia .
Liver involvement is not common in Felty’s syndrome, but
if present, combination of hepatic nodular regenerative
hyperplasia, portal hypertension, and variceal bleeding are
characteristic findings in FS .
9. Overall risk of all cancers is increased in FS patients.
There is a two-fold greater chance of non-Hodgkin’s
lymphoma (NHL) in RA patients, which is probably
directly related to the severity of RA. Patients with FS
have more risk for developing NHL than RA
population.This higher risk is similar to the increased
risk of Sjogren’s syndrome in RA .
10. Neutropenia is the hallmark of FS.
anemia of chronic disease
thrombocytopenia.
High titers of rheumatoid factor presents in most patients.
Antinuclear antibodies (ANA), antiperinuclear antibodies, and anti-keratin antibodies
can be positive in both RA and FS patients. (neither sensitive nor specific to be used for
diagnosis and treatment follow up).
Antihistone antibodies are detectable in 83% of patients with Felty's syndrome, and
presence of antihistone antibody in a known case of RA is almost always indicative of FS .
Erythrocyte sedimentation rate is constantly elevated.
Complement components are usually depressed and circulatory immune complexes are
usually elevated.
Antineutrophil cytoplasmic antibodies (ANCA) of atypical type can be positive in 77% of
patients.
Recent studies have shown that immunoglobulin G autoantibodies in patients with FS,
avidly binds to deiminated histones, (citrullinated histones) and neutrophil extracellular
chromatin traps (NETosis) . These antibodies can easily differentiate subclinical FS from
bland RA. Owing to strong association of antibodies against cyclic citrullinated peptide
(ACPA or anti-CCP) and extra-articular manifestation of RA, high titers of ACPA is
expected in FS as well. Positivity rate of ACPA in FS is not clear yet
LABORATORY INVESTIGATION
11. Bone marrow aspiration and biopsy are recommended
to have a better assessment of the hematopoiesis and
rule out bone marrow involvement by other
hematological and nonhematological malignancies
but bone marrow findings are not specific.
Bone marrow can be normocellular but myeloid
hyperplasia and maturation arrest are the most
common findings.
Unlike SLE, both relative and absolute
granulocytopenia are common in FS. However, the
granulocytopenia can be transient and spontaneous
remission has been reported.
12.
13. Complications of FS include the following:
Splenic rupture
Life-threatening infection
Toxicity due to immunosuppressive regimens
Portal hypertension and gastrointestinal bleeding due
to nodular regenerative hyperplasia of the liver
14. Felty’s syndrome can be a differential diagnosis of any
systemic disease with neutropenia.
LGL leukemia , T-LGL maybehave as a chronic inflammatory
disease, and is the main differential diagnosis of any RA patient
who presents with neutropenia .
Hodgkin and non-Hodgkin lymphomas are among theneoplastic
differential diagnosis .
• Cirrhosis
• Myeloproliferative Disease
• Non-Hodgkin Lymphoma
• Sarcoidosis
• Sjogren Syndrome
• Systemic Lupus Erythematosus (SLE)
• Tuberculosis
15. There is no definitive treatment for FS and no randomized clinical trials available
for FS.
Isolated neutropenia is not essentially an indication for specific treatment unless
granulocyte count < 1000/mm3.
Untreated cases of RA with granulocytopenia usually respond well to conventional
disease modifying anti-rheumatic drugs.
constitutional symptoms (fever) and RA. Administration of broad-spectrum
antibiotics covering the most important microbial or fungal agents.
Low dose methotrexate (MTX), a folate antagonist inhibiting purine synthesis that
is widely accepted treatment for classic RA, is the most commonly used drug as the
initial treatment
Cyclophosphamide (with active metabolite phosphoramide mustard) is seldom an
initial choice owing to potential role of inducing neutropenia and harboring risk of
infection.
corticosteroids are empirically used in FS with good initial response, but its long
term use is a major issue due to increased risk of infection and these agents should
probably be viewed as a second-line treatment modality .
16. hydroxychloroquine had a long lasting effect and
increased the neutrophil count after a few days .
Leflunamide had disappointing results in FS.
Continuous production of autoantibodies has been
observed in FS and
B cell depletion therapy with rituximab may have
some potential role in FS management.
biological agents mainly TNF- alpha blocking agents
such as etanercept, infliximab, and adalimumab
increased risk of neutropenia and infection, these
biological agents are not good options.
17. granulopoietic growth factors, such as granulocyte
colony-stimulating factor (G-CSF) and granulocyte-
macrophage colony-stimulating factor (GM-CSF),
effectively and quickly raise the granulocyte count,
which is important in patients with life-threatening
infections. Initial treatment of patients with FS and
life-threatening infections should include
administration of a growth factor. Long-term use of G-
CSF appears to be well tolerated, though
hypersensitivity vasculitis and flareups of the
underlying RA have been reported.
18. splenectomy can be avoided in the majority of FS patients.
It is important to monitor patient clinical condition rather
than following laboratory values, considering this
important fact that neutropenia does not predispose every
patient to infectious complications. Thus, prophylactic
splenectomy is not recommended and splenectomy is
always the last therapeutic modality for FS patients who
have severe neutropenia (ANC < 500/mm3) and frequent
infections.
Splenectomy can improve neutropenia, but it does not
provide a long-lasting effect. Almost all patients show some
improvement in neutrophil counts after splenectomy but
neutropenia reoccurs in approximately 25% of the patients