This document defines anemia and provides classifications of anemia. It begins by defining anemia as a condition where the number of red blood cells or amount of hemoglobin is lower than normal, causing inadequate oxygen delivery. It then lists normal hemoglobin and red blood cell reference values. Anemia is classified based on cell size (microcytic, normocytic, macrocytic), color (hypochromic, normochromic, hyperchromic), and pathophysiology (red blood cell loss, decreased production). Common causes of anemia are discussed including iron deficiency, thalassemia, sickle cell disease, and blood loss. Diagnostic findings and treatments for various specific anemias are also summarized.
Title: Understanding Anemia: Causes, Types, Clinical Features, and Diagnostic Investigations
Anemia is a condition characterized by a deficiency in the number or quality of red blood cells (RBCs) or hemoglobin in the blood, leading to reduced oxygen-carrying capacity. It is a prevalent global health issue affecting people of all ages, genders, and socioeconomic backgrounds. Understanding the causes, types, clinical features, and diagnostic investigations of anemia is crucial for effective management and treatment.
**Causes of Anemia:**
Anemia can result from various factors that disrupt the production, lifespan, or function of red blood cells. Some common causes include:
1. **Iron Deficiency:** Insufficient intake or absorption of iron, essential for hemoglobin synthesis, is a primary cause of anemia globally. It can stem from poor dietary intake, chronic blood loss (e.g., menstruation, gastrointestinal bleeding), or increased demand during pregnancy.
2. **Vitamin Deficiencies:** Deficiencies in vitamins such as vitamin B12 (cobalamin) or folate (vitamin B9) can impair RBC production, leading to megaloblastic anemia.
3. **Chronic Diseases:** Conditions like chronic kidney disease, inflammatory disorders (e.g., rheumatoid arthritis), and infections can disrupt erythropoiesis (RBC production) or accelerate RBC destruction, causing anemia.
4. **Hemolytic Disorders:** Inherited or acquired conditions that increase the breakdown (hemolysis) of red blood cells, such as sickle cell disease, thalassemia, or autoimmune hemolytic anemia, can result in anemia.
5. **Bone Marrow Disorders:** Diseases affecting the bone marrow, including leukemia, myelodysplastic syndromes, and aplastic anemia, can lead to decreased RBC production and anemia.
**Types of Anemia:**
Anemia is classified based on the underlying mechanism or etiology, leading to several types:
1. **Iron-Deficiency Anemia:** Characterized by low iron levels, resulting in decreased hemoglobin synthesis and microcytic (small-sized) RBCs.
2. **Megaloblastic Anemia:** Caused by impaired DNA synthesis in RBC precursors due to deficiencies in vitamin B12 or folate, leading to macrocytic (large-sized) RBCs.
3. **Hemolytic Anemia:** Occurs due to increased RBC destruction, either intravascularly (within blood vessels) or extravascularly (outside blood vessels), leading to various subtypes like autoimmune hemolytic anemia, hereditary spherocytosis, and sickle cell disease.
4. **Anemia of Chronic Disease:** Associated with chronic inflammation, infections, or malignancies, leading to impaired iron metabolism and decreased RBC production.
5. **Aplastic Anemia:** Results from bone marrow failure, leading to decreased production of all blood cell types, including RBCs.
**Clinical Features of Anemia:**
The clinical presentation of anemia can vary depending on its severity, underlying cause, and individual factors. Common clinical features include:
anemia is a condition in which you lack enough healthy red blood cells to carry adequate oxygen to your body's tissues. Having anemia, also referred to as low hemoglobin, can make you feel tired and weak. There are many forms of anemia, each with its
Tom Selleck Health: A Comprehensive Look at the Iconic Actor’s Wellness Journeygreendigital
Tom Selleck, an enduring figure in Hollywood. has captivated audiences for decades with his rugged charm, iconic moustache. and memorable roles in television and film. From his breakout role as Thomas Magnum in Magnum P.I. to his current portrayal of Frank Reagan in Blue Bloods. Selleck's career has spanned over 50 years. But beyond his professional achievements. fans have often been curious about Tom Selleck Health. especially as he has aged in the public eye.
Follow us on: Pinterest
Introduction
Many have been interested in Tom Selleck health. not only because of his enduring presence on screen but also because of the challenges. and lifestyle choices he has faced and made over the years. This article delves into the various aspects of Tom Selleck health. exploring his fitness regimen, diet, mental health. and the challenges he has encountered as he ages. We'll look at how he maintains his well-being. the health issues he has faced, and his approach to ageing .
Early Life and Career
Childhood and Athletic Beginnings
Tom Selleck was born on January 29, 1945, in Detroit, Michigan, and grew up in Sherman Oaks, California. From an early age, he was involved in sports, particularly basketball. which played a significant role in his physical development. His athletic pursuits continued into college. where he attended the University of Southern California (USC) on a basketball scholarship. This early involvement in sports laid a strong foundation for his physical health and disciplined lifestyle.
Transition to Acting
Selleck's transition from an athlete to an actor came with its physical demands. His first significant role in "Magnum P.I." required him to perform various stunts and maintain a fit appearance. This role, which he played from 1980 to 1988. necessitated a rigorous fitness routine to meet the show's demands. setting the stage for his long-term commitment to health and wellness.
Fitness Regimen
Workout Routine
Tom Selleck health and fitness regimen has evolved. adapting to his changing roles and age. During his "Magnum, P.I." days. Selleck's workouts were intense and focused on building and maintaining muscle mass. His routine included weightlifting, cardiovascular exercises. and specific training for the stunts he performed on the show.
Selleck adjusted his fitness routine as he aged to suit his body's needs. Today, his workouts focus on maintaining flexibility, strength, and cardiovascular health. He incorporates low-impact exercises such as swimming, walking, and light weightlifting. This balanced approach helps him stay fit without putting undue strain on his joints and muscles.
Importance of Flexibility and Mobility
In recent years, Selleck has emphasized the importance of flexibility and mobility in his fitness regimen. Understanding the natural decline in muscle mass and joint flexibility with age. he includes stretching and yoga in his routine. These practices help prevent injuries, improve posture, and maintain mobilit
Title: Sense of Taste
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the structure and function of taste buds.
Describe the relationship between the taste threshold and taste index of common substances.
Explain the chemical basis and signal transduction of taste perception for each type of primary taste sensation.
Recognize different abnormalities of taste perception and their causes.
Key Topics:
Significance of Taste Sensation:
Differentiation between pleasant and harmful food
Influence on behavior
Selection of food based on metabolic needs
Receptors of Taste:
Taste buds on the tongue
Influence of sense of smell, texture of food, and pain stimulation (e.g., by pepper)
Primary and Secondary Taste Sensations:
Primary taste sensations: Sweet, Sour, Salty, Bitter, Umami
Chemical basis and signal transduction mechanisms for each taste
Taste Threshold and Index:
Taste threshold values for Sweet (sucrose), Salty (NaCl), Sour (HCl), and Bitter (Quinine)
Taste index relationship: Inversely proportional to taste threshold
Taste Blindness:
Inability to taste certain substances, particularly thiourea compounds
Example: Phenylthiocarbamide
Structure and Function of Taste Buds:
Composition: Epithelial cells, Sustentacular/Supporting cells, Taste cells, Basal cells
Features: Taste pores, Taste hairs/microvilli, and Taste nerve fibers
Location of Taste Buds:
Found in papillae of the tongue (Fungiform, Circumvallate, Foliate)
Also present on the palate, tonsillar pillars, epiglottis, and proximal esophagus
Mechanism of Taste Stimulation:
Interaction of taste substances with receptors on microvilli
Signal transduction pathways for Umami, Sweet, Bitter, Sour, and Salty tastes
Taste Sensitivity and Adaptation:
Decrease in sensitivity with age
Rapid adaptation of taste sensation
Role of Saliva in Taste:
Dissolution of tastants to reach receptors
Washing away the stimulus
Taste Preferences and Aversions:
Mechanisms behind taste preference and aversion
Influence of receptors and neural pathways
Impact of Sensory Nerve Damage:
Degeneration of taste buds if the sensory nerve fiber is cut
Abnormalities of Taste Detection:
Conditions: Ageusia, Hypogeusia, Dysgeusia (parageusia)
Causes: Nerve damage, neurological disorders, infections, poor oral hygiene, adverse drug effects, deficiencies, aging, tobacco use, altered neurotransmitter levels
Neurotransmitters and Taste Threshold:
Effects of serotonin (5-HT) and norepinephrine (NE) on taste sensitivity
Supertasters:
25% of the population with heightened sensitivity to taste, especially bitterness
Increased number of fungiform papillae
Report Back from SGO 2024: What’s the Latest in Cervical Cancer?bkling
Are you curious about what’s new in cervical cancer research or unsure what the findings mean? Join Dr. Emily Ko, a gynecologic oncologist at Penn Medicine, to learn about the latest updates from the Society of Gynecologic Oncology (SGO) 2024 Annual Meeting on Women’s Cancer. Dr. Ko will discuss what the research presented at the conference means for you and answer your questions about the new developments.
Recomendações da OMS sobre cuidados maternos e neonatais para uma experiência pós-natal positiva.
Em consonância com os ODS – Objetivos do Desenvolvimento Sustentável e a Estratégia Global para a Saúde das Mulheres, Crianças e Adolescentes, e aplicando uma abordagem baseada nos direitos humanos, os esforços de cuidados pós-natais devem expandir-se para além da cobertura e da simples sobrevivência, de modo a incluir cuidados de qualidade.
Estas diretrizes visam melhorar a qualidade dos cuidados pós-natais essenciais e de rotina prestados às mulheres e aos recém-nascidos, com o objetivo final de melhorar a saúde e o bem-estar materno e neonatal.
Uma “experiência pós-natal positiva” é um resultado importante para todas as mulheres que dão à luz e para os seus recém-nascidos, estabelecendo as bases para a melhoria da saúde e do bem-estar a curto e longo prazo. Uma experiência pós-natal positiva é definida como aquela em que as mulheres, pessoas que gestam, os recém-nascidos, os casais, os pais, os cuidadores e as famílias recebem informação consistente, garantia e apoio de profissionais de saúde motivados; e onde um sistema de saúde flexível e com recursos reconheça as necessidades das mulheres e dos bebês e respeite o seu contexto cultural.
Estas diretrizes consolidadas apresentam algumas recomendações novas e já bem fundamentadas sobre cuidados pós-natais de rotina para mulheres e neonatos que recebem cuidados no pós-parto em unidades de saúde ou na comunidade, independentemente dos recursos disponíveis.
É fornecido um conjunto abrangente de recomendações para cuidados durante o período puerperal, com ênfase nos cuidados essenciais que todas as mulheres e recém-nascidos devem receber, e com a devida atenção à qualidade dos cuidados; isto é, a entrega e a experiência do cuidado recebido. Estas diretrizes atualizam e ampliam as recomendações da OMS de 2014 sobre cuidados pós-natais da mãe e do recém-nascido e complementam as atuais diretrizes da OMS sobre a gestão de complicações pós-natais.
O estabelecimento da amamentação e o manejo das principais intercorrências é contemplada.
Recomendamos muito.
Vamos discutir essas recomendações no nosso curso de pós-graduação em Aleitamento no Instituto Ciclos.
Esta publicação só está disponível em inglês até o momento.
Prof. Marcus Renato de Carvalho
www.agostodourado.com
Basavarajeeyam is an important text for ayurvedic physician belonging to andhra pradehs. It is a popular compendium in various parts of our country as well as in andhra pradesh. The content of the text was presented in sanskrit and telugu language (Bilingual). One of the most famous book in ayurvedic pharmaceutics and therapeutics. This book contains 25 chapters called as prakaranas. Many rasaoushadis were explained, pioneer of dhatu druti, nadi pareeksha, mutra pareeksha etc. Belongs to the period of 15-16 century. New diseases like upadamsha, phiranga rogas are explained.
ARTIFICIAL INTELLIGENCE IN HEALTHCARE.pdfAnujkumaranit
Artificial intelligence (AI) refers to the simulation of human intelligence processes by machines, especially computer systems. It encompasses tasks such as learning, reasoning, problem-solving, perception, and language understanding. AI technologies are revolutionizing various fields, from healthcare to finance, by enabling machines to perform tasks that typically require human intelligence.
263778731218 Abortion Clinic /Pills In Harare ,sisternakatoto
263778731218 Abortion Clinic /Pills In Harare ,ABORTION WOMEN’S CLINIC +27730423979 IN women clinic we believe that every woman should be able to make choices in her pregnancy. Our job is to provide compassionate care, safety,affordable and confidential services. That’s why we have won the trust from all generations of women all over the world. we use non surgical method(Abortion pills) to terminate…Dr.LISA +27730423979women Clinic is committed to providing the highest quality of obstetrical and gynecological care to women of all ages. Our dedicated staff aim to treat each patient and her health concerns with compassion and respect.Our dedicated group ABORTION WOMEN’S CLINIC +27730423979 IN women clinic we believe that every woman should be able to make choices in her pregnancy. Our job is to provide compassionate care, safety,affordable and confidential services. That’s why we have won the trust from all generations of women all over the world. we use non surgical method(Abortion pills) to terminate…Dr.LISA +27730423979women Clinic is committed to providing the highest quality of obstetrical and gynecological care to women of all ages. Our dedicated staff aim to treat each patient and her health concerns with compassion and respect.Our dedicated group of receptionists, nurses, and physicians have worked together as a teamof receptionists, nurses, and physicians have worked together as a team wwww.lisywomensclinic.co.za/
New Directions in Targeted Therapeutic Approaches for Older Adults With Mantl...i3 Health
i3 Health is pleased to make the speaker slides from this activity available for use as a non-accredited self-study or teaching resource.
This slide deck presented by Dr. Kami Maddocks, Professor-Clinical in the Division of Hematology and
Associate Division Director for Ambulatory Operations
The Ohio State University Comprehensive Cancer Center, will provide insight into new directions in targeted therapeutic approaches for older adults with mantle cell lymphoma.
STATEMENT OF NEED
Mantle cell lymphoma (MCL) is a rare, aggressive B-cell non-Hodgkin lymphoma (NHL) accounting for 5% to 7% of all lymphomas. Its prognosis ranges from indolent disease that does not require treatment for years to very aggressive disease, which is associated with poor survival (Silkenstedt et al, 2021). Typically, MCL is diagnosed at advanced stage and in older patients who cannot tolerate intensive therapy (NCCN, 2022). Although recent advances have slightly increased remission rates, recurrence and relapse remain very common, leading to a median overall survival between 3 and 6 years (LLS, 2021). Though there are several effective options, progress is still needed towards establishing an accepted frontline approach for MCL (Castellino et al, 2022). Treatment selection and management of MCL are complicated by the heterogeneity of prognosis, advanced age and comorbidities of patients, and lack of an established standard approach for treatment, making it vital that clinicians be familiar with the latest research and advances in this area. In this activity chaired by Michael Wang, MD, Professor in the Department of Lymphoma & Myeloma at MD Anderson Cancer Center, expert faculty will discuss prognostic factors informing treatment, the promising results of recent trials in new therapeutic approaches, and the implications of treatment resistance in therapeutic selection for MCL.
Target Audience
Hematology/oncology fellows, attending faculty, and other health care professionals involved in the treatment of patients with mantle cell lymphoma (MCL).
Learning Objectives
1.) Identify clinical and biological prognostic factors that can guide treatment decision making for older adults with MCL
2.) Evaluate emerging data on targeted therapeutic approaches for treatment-naive and relapsed/refractory MCL and their applicability to older adults
3.) Assess mechanisms of resistance to targeted therapies for MCL and their implications for treatment selection
These simplified slides by Dr. Sidra Arshad present an overview of the non-respiratory functions of the respiratory tract.
Learning objectives:
1. Enlist the non-respiratory functions of the respiratory tract
2. Briefly explain how these functions are carried out
3. Discuss the significance of dead space
4. Differentiate between minute ventilation and alveolar ventilation
5. Describe the cough and sneeze reflexes
Study Resources:
1. Chapter 39, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 34, Ganong’s Review of Medical Physiology, 26th edition
3. Chapter 17, Human Physiology by Lauralee Sherwood, 9th edition
4. Non-respiratory functions of the lungs https://academic.oup.com/bjaed/article/13/3/98/278874
2. DEFINITION
• any condition in which the number of red cells or the
amount of hemoglobin in the blood is less than
normal.
• pathophysiological condition in which the body
cannot meet its demands for oxygen.
3. BLOOD REFERENCE VALUES
• Hemoglobin (M: 13.0 – 17.0 g/dl and F: 12.0 – 15.0 g/dl)
• RBC count RBC count Males range 5.5 x 1012/L, Females 4.5 x 1012/L.
• Mean cell volume MCV (80 – 100 fl)
• Mean cell hemoglobin (27 – 32 pg)? the average mass of hemoglobin per red
blood cell in a sample of blood. MCH value is diminished in hypochromic
anemias.
• Mean cell hemoglobin concentration MCHC (30-35 g/dl).
4. CLASSIFICATION OF ANEMIA
• By color
• Hypochromic
• Normochromic
• Hyperchromic
• By volume
• Microcytic
• Normocytic
• Macrocytic
• Pathophysiologic classification
• RBC loss
• Decreased RBC production
5. CLASSIFICATION BY VOLUME
I. microcytic anemia (MCV <80 fl)
1. iron deficiency anemia
2. thalassemia syndromes
3. anemia of chronic disease
4. sideroblastic anemia
II. normocytic anemia (MCV 80-100 fl)
1. anemia of blood loss
2. hemolytic anemia
III. macrocytic anemia (MCV >100 fl)
1. megaloblastic anemia
6. PATHOPHYSIOLOGIC
CLASSIFICATION
I RBC loss
• Blood loss
• Increased RBC destruction
a. intrinsic abnormality
b. extrinsic abnormality
II Decreased RBC production
• Stem cell abnormality
• erythroblast abnormality
• Unknown/multiple mechanism
7. PATHOPHYSIOLOGIC
CLASSIFICATION
I RBC loss
• Blood loss
• acute : trauma, massive hemorrhage
• chronic : GI lesion, GYN lesion
• Increased RBC destruction
a. intrinsic abnormality
b. extrinsic abnormality
8. PATHOPHYSIOLOGIC
CLASSIFICATION
I RBC loss
• Blood loss
• Increased RBC destruction
a. intrinsic abnormality
i. membrane disorder (e.g. hereditary spherocytosis)
ii. enzyme disorder (e.g. G6PD deficiency & PKD)
iii. Hgb synthesis disorder (e.g. Thalassemia & sickle cell disease)
iv. acquired membrane defect
a. extrinsic abnormality
9. PATHOPHYSIOLOGIC
CLASSIFICATION
I RBC loss
• Blood loss
• Increased RBC destruction
a. intrinsic abnormality
b. extrinsic abnormality
a. mechanical trauma (e.g. TTP/HUS, DIC)
b. chemical injury (e.g. lead poisoning)
c. Infection (e.g. malaria)
d. immunologic injury (e.g. autoimmune hemolytic anemia)
10. PATHOPHYSIOLOGIC
CLASSIFICATION
I RBC loss
• Blood loss
• Increased RBC destruction
a. intrinsic abnormality
b. extrinsic abnormality
II Decreased RBC production
• Stem cell abnormality
• erythroblast abnormality
• Unknown/multiple mechanism
11. PATHOPHYSIOLOGIC
CLASSIFICATION
I RBC loss
• Blood loss
• Increased RBC destruction
a. intrinsic abnormality
b. extrinsic abnormality
II Decreased RBC production
• Stem cell abnormality (aplastic anemia)
• erythroblast abnormality (megaloblastic anemia & IDA)
• Unknown/multiple mechanism (sideroblastic anemia)
12. PATHOPHYSIOLOGIC
CLASSIFICATION
I RBC loss
• Blood loss
• Increased RBC destruction
a. intrinsic abnormality
i. membrane disorder (hereditary spherocytosis & elliptocytosis)
ii. enzyme disorder (e.g. G6PD deficiency & PKD)
iii. Hgb synthesis disorder (Thalassemia & sickle cell disease)
iv. acquired membrane defect (PNH)
a. extrinsic abnormality
13. HEREDITARY SPHEROCYTOSIS
• Deficiency of Beta Spectrin or Ankyrin Loss of membrane surface
area becomes more spherical Destruction in Spleen
• Clinical findings:
2. splenomegaly
3. marked compensatory erythroid hyperplasia in BM
4. jaundice, pigment cholelithiasis (↑ bilirubin)
• Laboratory findings : normal MCV with increased MCHC
• Treatment: splenectomy
14. HEREDITARY ELLIPTOCYTOSIS
• Abnormally large number of patient’s erythrocytes are
elliptical rather than typical biconcave shape.
• Inherited disease (autosomal dominant)
• Impaired aggregation of spectrin.
• majority require no treatment.
15. PATHOPHYSIOLOGIC
CLASSIFICATION
I RBC loss
• Blood loss
• Increased RBC destruction
a. intrinsic abnormality
i. membrane disorder (hereditary spherocytosis & elliptocytosis)
ii. enzyme disorder (e.g. G6PD deficiency & PKD)
iii. Hgb synthesis disorder (Thalassemia & sickle cell disease)
iv. acquired membrane defect (PNH)
a. extrinsic abnormality
16. G6PD DEFICIENCY
• G6PD deficiency is an X-linked inherited condition in which the body
doesn't have enough of the enzyme glucose-6-phosphate
dehydrogenase
• Function? Pivotal enzyme in HMP Shunt & produces NADPH to
protect RBC against oxidative stress
• Most common enzymopathy -10% world’s population
• Neonatal jaundice
• Hemolysis after exposure to oxidant stress
• Drugs
• Fava beans
18. G6PD DEFICIENCY
• Lab findings:
• Heinz bodies : precipitates of denatured hemoglobin material
• “bite cells”? When a macrophage in the spleen identifies a RBC with
a Heinz body, it removes the precipitate and a small piece of the
membrane, leading to characteristic "bite cells“
• Treatment:
• Stop the precipitating drug or treat the infection
• Acute transfusions if required
20. PYRUVATE KINASE DEFICIENCY
• also called erythrocyte pyruvate kinase deficiency, is an inherited metabolic
disorder of the enzyme pyruvate kinase which affects the survival of red blood cells
due to inability to maintain normal ATP levels.
• Splenomegaly
• Diagnosis: 1. fluorescent spot test 2. enzyme assay
• patients tolerate anemia rather well ( result in: high levels of 2,3-DPG)
phosphoenol pyruvate pyruvate lactat
e
P
K
ADP ATP *NADH NAD
21. PATHOPHYSIOLOGIC
CLASSIFICATION
I RBC loss
• Blood loss
• Increased RBC destruction
a. intrinsic abnormality
i. membrane disorder (hereditary spherocytosis & elliptocytosis)
ii. enzyme disorder (G6PD deficiency & PKD)
iii. Hgb synthesis disorder (Thalassemia & sickle cell disease)
iv. acquired membrane defect (PNH)
a. extrinsic abnormality
22. THALASSEMIA SYNDROMES
• heterogeneous hemolytic disorders characterized by
quantitative abnormalities of hemoglobin synthesis.
• genetic defect in globin production > inadequate Hgb
formation > hypochromic anemia (low hemoglobin in
RBC)
• Types:
• α-thalassemia : α-chain deficiency
• β-thalassemia : β-chain deficiency
23. THALASSEMIA SYNDROMES
• There are many forms of thalassemia. Each type has
many different subtypes. Both alpha and beta
thalassemia include the following two forms:
• Thalassemia major (gene defect from both parents)
• Thalassemia minor (faulty gene from only one parent)
• α thalassemia major causes stillbirth
• Children born with β thalassemia major (Cooley's
anemia) are normal at birth, but develop severe
anemia after 6 months where HbF must be replaced
with HbA .
24. α-Thalassemia
• There are a total of 4 alpha globin genes, alpha
thalassemia result from the deletions of some or all
of the genes.Disease Defect Hb A % HbH % MCV,fl
Silent carrier (asymptomatic) One deletion 97 0 90
Thalassemia traits Two deletions 90-95 rare 70-80
HbH (B4) Three deletions 70-95 5-30 60-70
Hb Bart (hydrops fetalis) Four deletions
Death in utero
0 5-10
25. β Thalassemia Major
• Severe, homozygous
• Childhood, growth delay, chipmunk face.
• Severe anemia , hepatosplenomegaly
• Treatment:
• Require transfusion once to twice a month
• Treat iron overload (due to chronic transfusion, give deferasirox or
deferoxamine)
• splenectomy reduce hemolysis
• Bone marrow tranplantation might be used.
• Diagnosis:
• P. Smear- severe microcytosis, target cells
• Hb electrophoresis: HbF - 90-96 % HbA2- 3.5 %- 5.5% HbA - 0 %
26. β Thalassemia Intermedia
• Similar stigmata like major
• Survive without transfusion
• Less severe than major
• Moderate anemia, microcytosis, hypochromia
• Diagnosis:
• P. Smear- severe microcytosis, target cells
• Hb electrophoresis: HbF - 20% - 100% HbA2 3.5 % -
5.5% HbA 0% - 30%
28. SICKLE CELL DISEASE
• Severe hereditary form of anemia (recessive) in which
an abnormal hemoglobin (hemoglobin S) leads to
chronic hemolytic anemia, pain and organ failure.
• Structurally abnormal hemoglobin (hemoglobin S)
result from a point mutation in the β chain of
hemoglobin molecule (glutamic acid valine).
• If only one parent has sickle cell; their child would have
sickle cell trait. People with sickle cell trait do not have
the symptoms of sickle cell anemia.
• Symptoms appears after 8 to 10 weeks of age, when
the HbF has been replaced by HbS.
29. SICKLE CELL DISEASE
• There are two major consequences of RBC sickling
• Chronic hemolytic anemia.
• Blood vessels occlusion.
• Premature destruction of RBC in the spleen causes
hemolysis and anemia.
• Vessels occlusion disrupt blood flow causing tissue
ischemia and pain crisis.
• Other symptoms: Fatigue, paleness, rapid heart rate and
shortness of breath
• Splenic infraction may lead to splenic dysfunction which
predispose the patient to infections.
30. SICKLE CELL DISEASE
• Neonatal diagnosis of sickle cell disease is made on
the basis of clinical findings and hemoglobin
electrophoresis.
• The goal of treatment is to manage and control
symptoms, and to limit the number of crises.
31. PATHOPHYSIOLOGIC
CLASSIFICATION
I RBC loss
• Blood loss
• Increased RBC destruction
a. intrinsic abnormality
i. membrane disorder (e.g. hereditary spherocytosis)
ii. enzyme disorder (G6PD deficiency & PKD)
iii. Hgb synthesis disorder (Thalassemia & sickle cell disease)
iv. Acquired membrane defect (PNH)
a. extrinsic abnormality
32. PAROXYSMAL NOCTURNAL
HEMOGLOBINURIA
• Is a rare disease in which red blood cells break down
earlier than normal.
• 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.
• Risk Factors: Unknown!
33. PATHOPHYSIOLOGIC
CLASSIFICATION
I RBC loss
• Blood loss
• Increased RBC destruction
a. intrinsic abnormality
b. extrinsic abnormality
a. mechanical trauma (e.g. TTP/HUS, DIC)
b. chemical injury (e.g. lead poisoning)
c. Infection (e.g. malaria)
d. immunologic injury (e.g. autoimmune hemolytic anemia)
34. MECHANICAL TRAUMA
• Mechanical heart valves, Arterial grafts: cause
shear stress damage
• March hemoglobinuria: Red cell damage in
capillaries of feet
• Thermal injury: burns
• Microangiopathic hemolytic anemia (MAHA):
by passage of RBC through fibrin strands
deposited in small vessels disruption of RBC
35. PATHOPHYSIOLOGIC
CLASSIFICATION
I RBC loss
• Blood loss
• Increased RBC destruction
a. intrinsic abnormality
b. extrinsic abnormality
a. mechanical trauma (e.g. TTP/HUS, DIC)
b. chemical injury (e.g. lead poisoning)
c. Infection (e.g. malaria)
d. immunologic injury (e.g. autoimmune hemolytic anemia)
36. LEAD POISONING
• inhibition of 5’-nucleotidase and
sodium-potassium pump decreased
RBC survival.
• Other chemical injury by drugs: oxidant
denaturation of hemoglobin e.g:
Dapsone, sulphasalazine, Arsenic gas,
Cu, Nitrates & Nitrobenzene.
37. PATHOPHYSIOLOGIC
CLASSIFICATION
I RBC loss
• Blood loss
• Increased RBC destruction
a. intrinsic abnormality
b. extrinsic abnormality
a. mechanical trauma (e.g. TTP/HUS, DIC)
b. chemical injury (e.g. lead poisoning)
c. Infection (e.g. malaria)
d. immunologic injury (e.g. autoimmune hemolytic anemia)
38. MALARIA
• intracellular protozoan parasite
(Plasmodium)
• intravascular hemolysis: severe called
‘Blackwater fever’
• worldwide infection affecting 100 million
people 1 to 1.5 million deaths each year.
39. PATHOPHYSIOLOGIC
CLASSIFICATION
I RBC loss
• Blood loss
• Increased RBC destruction
a. intrinsic abnormality
b. extrinsic abnormality
a. mechanical trauma (e.g. TTP/HUS, DIC)
b. chemical injury (e.g. lead poisoning)
c. Infection (e.g. malaria)
d. immunologic injury (e.g. autoimmune hemolytic anemia)
40. AUTOIMMUNE HEMOLYTIC
ANEMIA
• Result from RBC destruction due to RBC
autoantibodies: Ig G, M, E, A
• Most commonly-idiopathic
• Classification
• Warm AI hemolysis: Ab binds at 37degree
Celsius
• Cold AI Hemolysis: Ab binds at 4 degree
Celsius
41. Warm Al Hemolysis
• Can occurs at all age groups
• F > M
• Causes:
• 50% Idiopathic
• Rest - secondary causes: Lymphoid neoplasm, Solid
Tumors, Autoimmune and drugs like Alpha methyl
DOPA.
• Investigations:
• Normocytic, Increased LDH, Reticulocytosis, decreased
haptoglobin
• Increased indirect Bilirubin.
• Direct Coomb’s Test / Antiglobulin test
Direct antiglobulin test
demonstrating the presence of
autoantibodies (shown here) or
complement on the surface of
the red blood cell.
complement
42. Warm Al Hemolysis
•Treatment:
• Correct the underlying cause
• Prednisolone 1mg/kg orally until Hb reaches 10mg/dl then
taper slowly and stop
• Transfusion: for life threatening problems
• If no response to steroids > Splenectomy or
immunosuppressive: Azathioprine, Cyclophosphamide
43. Cold AI Hemolysis
• Usually IgM
• Acute or Chronic form
• Chronic:
• C/F:
• Elderly patients
• Cold , painful & often blue fingers, toes, ears, or nose ( Acrocyanosis)
• Investigations:
• e/o hemolysis
• P Smear: Microspherocytosis
• Ig M with specificity to I or I Ag
44. Cold AI Hemolysis
• Treatment:
• Treatment of the underlying
cause
• Keep extremities warm
• Steroids treatment
• Blood transfusion