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Paroxysmal nocturnal hemolobinurea (PNH) is a rare disease where red blood cells break down earlier than normal due to missing a gene called PIG-A that helps proteins stick to cells and protect them. Without this, red blood cells leak hemoglobin into the blood and urine, especially at night or in the morning. It has symptoms like abdominal pain, dark urine, and easy bruising. Tests like blood counts and urine analysis are used to diagnose. Treatment includes drugs to suppress the immune system and prevent cell breakdown, blood transfusions, iron/folic acid, and possibly bone marrow transplant. Complications can include blood clots, leukemia, or death.
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APLASTIC ANEMIA
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Polycythemia vera is a chronic myeloproliferative disorder characterized by increased red blood cell production which leads to thickened blood and risk of blood clots. Diagnosis is based on criteria including elevated red blood cell count and spleen enlargement. Treatment aims to reduce blood thickness and prevent clots through regular blood removal and medications to suppress bone marrow activity.
Polycythemia vera
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ALGORITHM FOR PANCYTOPENIA
TREATMENT
APLASTIC ANEMIA
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Polycythemia is a blood disorder where the body produces too many red blood cells. It can be primary, caused by problems in bone marrow production of red blood cells, or secondary, caused by factors like lung or heart diseases that result in low oxygen levels triggering high red blood cell production. Symptoms include feeling tired, shortness of breath, headaches, and itchy skin. Diagnosis involves blood tests showing high red blood cell counts and hematocrit levels. Treatment focuses on phlebotomy to reduce red blood cell counts through blood removal, along with medications in some cases to suppress bone marrow production of red blood cells.
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Hemolytic anemias are caused by the premature destruction of red blood cells, either inside or outside of blood vessels. They can be congenital/hereditary or acquired. Hemolytic anemias may be chronic like sickle cell disease or acute like those caused by drugs or infections. Membrane defects or metabolic abnormalities inside red blood cells, hemoglobinopathies, or immune or non-immune reactions outside cells can all cause hemolysis. Patients experience symptoms of anemia along with jaundice and dark urine. Diagnosis involves blood tests showing elevated reticulocytes, LDH, indirect bilirubin and a peripheral smear with fragmented cells. Treatment focuses on transfusion, hydration
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Polycythemia Vera is a chronic myeloproliferative disorder characterized by an overproduction of red blood cells from bone marrow due to a mutation in the JAK2 gene. This leads to thickening of the blood and complications like blood clots and spleen enlargement. It is diagnosed through blood tests showing increased red blood cells. While there is no cure, treatment focuses on reducing blood cell counts through regular blood removal and medications to suppress bone marrow production and stimulate the immune system.
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Polycythemia vera is a chronic myeloproliferative disorder characterized by increased red blood cell mass, leukocytosis, thrombocytosis, and elevated hemoglobin levels. It typically occurs in middle-aged adults and can cause a variety of symptoms including fullness, headaches, and pruritus. Treatment may involve phlebotomy to reduce red blood cell counts, myelosuppressive drugs to control the disease, and medications to reduce complications like uric acid levels and thrombosis. Nursing care focuses on monitoring for complications, educating patients, and ensuring safety during treatments.
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Polycythemia vera is a proliferative disorder where the bone marrow overproduces red blood cells, white blood cells, and platelets. It is caused by a mutation in the JAK2 enzyme. Common symptoms include headache, dizziness, and an enlarged spleen due to spleen enlargement and increased blood viscosity. Treatment focuses on phlebotomy to reduce red blood cell counts and medications like hydroxyurea to suppress bone marrow production and reduce risk of blood clots. Patients also need lifestyle changes and monitoring for complications like thrombosis.
Polycythemia vera
Polycythemia Vera is a chronic myeloproliferative disorder characterized by an overproduction of red blood cells from bone marrow due to a mutation in the JAK2 gene. This leads to thickening of the blood and complications like blood clots and spleen enlargement. It is diagnosed through blood tests showing increased red blood cells. While there is no cure, treatment focuses on reducing blood cell counts through regular blood removal and medications to suppress bone marrow production and stimulate the immune system.
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Hemolytic anemia occurs when the destruction of red blood cells outpaces their production. It can develop intrinsically due to abnormalities in red blood cells, such as in sickle cell anemia or thalassemia. Or it can occur extrinsically due to infection, autoimmune disorders, medications or splenic sequestration of normal red blood cells. Symptoms include pallor, fatigue, jaundice and dark urine. Treatment depends on the underlying cause but may include blood transfusions, medications to suppress the immune system, splenectomy or stem cell transplant.
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Aplastic Anaemia
It a bone marrow failure syndrome. Patients with severe Anaemia and neutropenia exhibiting a yellow hypercellular marrow on postmortem examination.
Features are. Pancytopenia, reticulocytopenia, bone marrow hypercellularity, heart murmur, pale-skin, gum, nail bus, epistaxis, mouth or throat infection, Anaemia etc. No gender differences based on the aetiology classified into two types Acquired and hereditary.
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David Snead on The use of digital pathology in the primary diagnosis of histo...
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IVMS-CV-Pharmacology- Anti-hypertensive Agents
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Case stuies in Lymphomas
This document summarizes several case studies involving lymphomas:
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Sickle cell anemia is a genetic blood disorder caused by a mutation in the beta-globin gene that results in abnormal hemoglobin S. When deoxygenated, hemoglobin S polymerizes and causes red blood cells to take on a sickle shape, which can block small blood vessels and damage tissues. Common complications include painful vasoclusive crises, acute chest syndrome, strokes, and infections. Treatment focuses on prevention of sickling through hydration and pain management of crises with opioids when they occur.
Sickle cell anemia
Sickle cell anemia is a genetic blood disorder where red blood cells become stiff and sticky and form into a sickle shape. This causes blockages in blood vessels, pain crises, and organ damage. It is caused by a mutation that changes hemoglobin in red blood cells. Symptoms include anemia, pain crises, fatigue, infections, and stroke. Treatment focuses on managing pain, preventing infections, treating complications, and sometimes blood transfusions or hydroxyurea. People with sickle cell anemia require lifelong monitoring and treatment.
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Hemolytic anemias are caused by increased destruction of red blood cells. This document discusses several types of hereditary and autoimmune hemolytic anemias including hereditary spherocytosis, glucose-6-phosphate dehydrogenase deficiency, autoimmune hemolytic anemia (warm and cold types), and paroxysmal nocturnal hemoglobinuria. Signs of hemolysis include jaundice, gallstones, splenomegaly, red blood cell abnormalities, increased LDH and reticulocytes, and a positive Coombs test. Management depends on the specific condition but may include folic acid, steroids, splenectomy, immunosuppression, plasmapheresis or treating any underlying
Anemia
Anemia is a condition where the hemoglobin concentration in the blood is lower than normal, reducing its oxygen carrying capacity. It can be caused by blood loss, deficiencies in red blood cell production, or excessive red blood cell destruction. Types of anemia include iron deficiency, pernicious, folic acid deficiency, thalassemia, and aplastic anemia. Treatment depends on the underlying cause and may involve supplements, medications, blood transfusions, or bone marrow transplants. Nursing care focuses on managing fatigue, maintaining nutrition and tissue perfusion, and promoting compliance with prescribed therapies.
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Sickle cell anaemia
1. Sickle-cell anemia is a genetic blood disorder caused by a mutation in the hemoglobin gene, resulting in rigid, sickle-shaped red blood cells and chronic hemolysis. It is characterized by severe pain crises, anemia, and life expectancy is shortened.
2. Complications include organ damage from blocked blood flow, infections due to spleen dysfunction, stroke, and bone/joint problems. Painful vaso-occlusive crises are the most common acute symptom.
3. Management involves folic acid, antibiotics, pain medications, blood transfusions, and hydroxyurea. The heterozygous form known as sickle cell trait does not cause symptoms.
Anaemia PNH
PNH is caused by a somatic mutation in the PIG-A gene in a hematopoietic stem cell, resulting in blood cells that lack glycosylphosphatidylinositol (GPI)-anchored proteins. These proteins include complement regulatory factors CD55 and CD59. As a result, PNH red blood cells are susceptible to complement-mediated hemolysis, leading to chronic hemolytic anemia. PNH often presents with hemoglobinuria, abdominal pain, dysphagia, and cytopenias. It is associated with aplastic anemia and carries a risk of thrombosis. The defect arises from a mutation in the PIG-A gene, which codes for an enzyme essential for GPI
Anaemia
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired blood disorder characterized by complement-induced breakdown of red blood cells in the bloodstream. This leads to anemia, red urine, and an increased risk of blood clots. PNH results from a genetic defect causing a lack of protective proteins on red blood cells and other cell surfaces, making them targets for destruction by the immune system's complement system. Treatment options include long-term anticoagulation therapy to prevent clots, transfusions to address anemia, and the monoclonal antibody eculizumab which inhibits complement system activity and reduces symptoms.
Hereditary spherocytosis
Hereditary spherocytosis is a genetically transmitted form of anemia where red blood cells are sphere-shaped rather than disk-shaped, making them prone to hemolysis. Symptoms include fatigue, pallor, jaundice, and in acute cases hypoxemia and hyperbilirubinemia. Diagnosis involves observing the lack of central pallor in red blood cells and increased fragility in hypotonic solutions. The condition is caused by defects in genes coding for membrane proteins, preventing red blood cells from maintaining their disk shape and surviving passage through the spleen. Treatment options include blood transfusions, folic acid supplementation, and splenectomy.
Hereditary elliptocytosis
Hereditary elliptocytosis is an inherited blood disorder where red blood cells (RBCs) are elliptical rather than biconcave. It is caused by defects in the RBC membrane and has an autosomal pattern of inheritance. Most people with hereditary elliptocytosis are asymptomatic, but 5-10% experience a clinically significant hemolytic anemia. The disorder exists on a spectrum from mild to severe. Treatment involves folic acid supplementation for those with significant hemolysis; most require no treatment. The prognosis is generally good except for those with very severe disease.
Dyserythropoietic anaemia
Congenital dyserythropoietic anemia (CDA) is a rare blood disorder characterized by ineffective red blood cell production resulting in anemia. There are four main types of CDA which are inherited in an autosomal recessive pattern. Patients with CDA require frequent blood transfusions and iron chelation therapy to remove excess iron from the body. Bone marrow transplant and gene therapy may cure CDA but carry risks. Recent gene therapy trials have shown promise in curing beta-thalassemia.
Anaemia
Anemia is a decrease in red blood cells or hemoglobin in the blood. It can be caused by blood loss, excessive red blood cell destruction, or deficient red blood cell production. Common symptoms include fatigue, weakness, and shortness of breath. Anemia is diagnosed through a complete blood count and examination of a blood smear. It can be classified based on red blood cell size as microcytic, normocytic, or macrocytic. A more detailed classification involves evaluating red blood cell production, destruction, and loss through parameters like reticulocyte count and mean corpuscular volume. Determining the underlying cause requires additional tests depending on the classification and symptoms.
Anaemia of chronic disease
Anemia of chronic disease, also known as anemia of inflammatory response, is a common type of anemia seen in patients with chronic illnesses like infections, immune disorders, or cancers. Recent research has found that the liver protein hepcidin, which regulates iron metabolism, plays a central role in causing this anemia by blocking the release of iron stores during inflammation. Hepcidin increases during inflammation and prevents the release of iron, leading to insufficient iron availability for red blood cell production. While locking up iron is beneficial in the short term for fighting infection, prolonged inflammation and iron sequestration can severely limit the bone marrow's ability to produce red blood cells. The ideal treatment is resolving the underlying chronic disease, but otherwise patients
Thalassemia
Thalassemia is a group of inherited blood disorders caused by mutations that reduce or eliminate globin chain production, leading to abnormal hemoglobin and anemia. There are two main types: alpha thalassemia affects alpha chain production while beta thalassemia affects beta chain production. Thalassemia is most common in populations from regions where malaria was endemic, as carriers have some protection against the disease. Treatment involves blood transfusions, iron chelation therapy, and potentially bone marrow transplantation.
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- 1. ليبالتوفيق ادعو Page 1 Paroxysmal nocturnal hemolobinurea (PNH) Email this page to a friendBookmark & SharePrinter-friendly version Paroxysmal nocturnal hemolobinurea is a rare disease in which red blood cells break down earlier than normal. Causes Persons with this disease have blood cells that are missing a gene called PIG-A. This gene allows a substance called glycosyl-phosphatidylinositol (GPI) to help certain proteins stick to cells. Without PIG-A, important proteins cannot connect to the cell surface and protect the cell from substances in the blood called complement. As a result, red blood cells break down too early. The red cells leak hemoglobin into the blood, which can pass into the urine. This can happen at any time, but is more likely to occur during the night or early morning. The disease can affect people of any age. It may lead to aplastic anemia, myelodysplastic syndrome, or acute myelogenous leukemia. Risk factors, except for prior aplastic anemia, are not known. Symptoms Abdominal pain Back pain Blood clots -- may form in some people Dark urine -- comes and goes Easy bruising or bleeding Headache Shortness of breath Exams and Tests Red and white blood cell counts and platelet counts may be low.
- 2. ليبالتوفيق ادعو Page 2 Red or brown urine signals the breakdown of red blood cells and that hemoglobin is being released into the body's circulation and eventually into the urine. Tests that may be done to diagnose this condition may include: Complete blood count (CBC) Coombs' test Flow cytometry to measure certain proteins Ham's (acid hemolysin) test Serum hemoglobin and haptoglobin Sucrose hemolysis test Urinalysis Urine hemosiderin Treatment Steroids or other drugs that suppress the immune system may help slow the break down of red blood cells. Blood transfusions may be needed. Supplemental iron and folic acid are provided. Blood thinners may also be needed to prevent clot formation. Soliris (eculizumab) is a drug used to treat PNH. It blocks the breakdown of red blood cells. Bone marrow transplantation can cure this disease. All patients with PNH should receive vaccinations against certain types of bacteria to prevent infection. Ask your doctor which ones are right for you. Outlook (Prognosis) The outcome varies. Most people survive greater than 10 years after their diagnosis. Death can result from complications such as blood clot formation (thrombosis) or bleeding. In rare cases, the abnormal cells may decrease over time. Possible Complications Acute myelogenous leukemia
- 3. ليبالتوفيق ادعو Page 3 Aplastic anemia Blood clots Death Hemolytic anemia Iron deficiency anemia Myelodysplasia