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MED 341 Anemia

MED 341 Anemia. Abdul kareem Al Momen, MD, FRCPC Professor of Medicine- Hematology King Saud University (Jan 26, 2014). Definition.

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MED 341 Anemia

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  1. MED 341Anemia Abdul kareem Al Momen, MD, FRCPC Professor of Medicine- Hematology King Saud University (Jan 26, 2014)

  2. Definition • Anemia is defined as reduction in the level of Hb/Hct (PCV) in relation to the normal level of tested healthy population at there own normal circumstances (Age, type of Hb, Oxygen tension).

  3. Average normal Hb/Hct (PCV) level in various populations

  4. Symptoms

  5. Symptoms & signs • Symptoms: (non-specific) fatigue, weakness, ↓ exercise tolerance, impaired memory, comprehension, appetite, Pica ( craving for non-food substances such as mud, sand, ice). • Signs: Depend on severity: pallor (↓ circulation to skin), • Symptoms and signs vary greatly and correlate with severity (severe ˃ mild) and onset/chronicity ( acute ˃ chronic) • Adaptation to anemia: • ↑ Heart rate, • ↑ stroke volume, • ↑ 2,3 DPG (→↓ affinity)

  6. Lack of energy

  7. Pallor

  8. Koilonychia, Papillary atrophy, erythema and angular stomatitis

  9. Classification • Congenital/ hereditary Or Acquired • Morphological : MCV (Microcytic, normocytic, macrocytic) , RDW (↑) • Etiological: Blood loss, Nutritional deficiencies, Hemolysis (RBC Destruction), Erythropoietin deficiency, Chronic inflammation, ( Infection, Malignancy, Connective tissue disease).

  10. Morphological Classification of Anemia • Low MCV (< 84 fl): IDA, Thalassemia traits, some cases of chronic illnesses, lead poisoning • High MCV (> 96 fl): Megaloblastic (Folate & B12 defeciency), Aplastic anemia, Myelodysplastic syndrome, Cytotoxic drugs ( e.g. hydroxyurea) • Normal MCV (84-94 fl): Acute blood loss, Erythropoietin deficiency, some cases of chronic illnesses

  11. Normocytic anemia

  12. Normal red cell morphology

  13. Microcytic anemia

  14. Macrocytic anemia

  15. Hereditary Anemia • 1- Hemoglobinopathies: a- Sickle cell anemia (homozygous) due to replacement of glutamic acid at position 6 in the beta chain → change of Hb character when Oxygen tension is low →↓ solubility, crystallization, fiber formation, cell rigidity, Obstruction of microcirculation, hemolysis, painful crises, thrombosis.

  16. a-sickle cell anemia • Painful episodes are precipitated by ↓ Oxygen tension in circulation ( high altitude, basements, use of charcoal in heating, pulmonary diseases, cardiac diseases) • ↑ viscosity (↑ Hb, WBC, Platelets, dehydration, infection, ) • Unknown causes

  17. a-sickle cell anemia

  18. a-sickle cell anemia

  19. a-sickle cell anemia • Prevention of disease: premarital testing, contraception, fetal selection (IVF) • Prevention of painful episodes: daily hydroxyurea, avoidance of hypoxia, dehydration, infection. • Management of painful crises; Hydration, pain killers, (Rarely transfusion)

  20. a-sickle cell anemia • Other complications of Sickle cell anemia; Hemolytic crises ( severe episodes), Splenic sequestration ( medical emergency), aplastic crisis (parvovirus B19 infection), Priapism (continuous, painful erection), stroke (cerebral infarction) • Treat accordingly

  21. b-Thalassemia • Alpha thalassemia • - aaa = alpha thalassemia trait type 2 (silent), • --aa = alpha thalassemia trait type 1(↓ MCV), • ---A = Hb H disease • ---- = Hydropes fetalis (incompatible with life)

  22. b-Thalassemia

  23. b-Thalassemia • Beta thallassemia: • -b = thalassemia trait (↓ MCV), • -- = (Homozygous) thalassemia major: severe anemia with severe intramedullary hemolysis, bone marrow expansion, hepato-splenomegally, growth and sexual retardation,

  24. b-Thalassemia • Prevention: premarital screening, early abortion (in some countries). • Treatment: regular blood transfusion ( Q 2-4 weeks), • Splenectomy ( after vaccination) at 6 years of age ( to reduce transfusion requirements), • Iron chelation. • Stem cell transplantation ( in some cases)

  25. c-enzymopathies • G6PD deficiency (x-linked)→↓ production of the antioxidant glutathione, → Hemolysis due oxygen free radicals that can be produced by certain foods and drugs e.g. fava beans. • Prevention: avoidance of oxidative foods & drugs

  26. d-membrane defects • Hereditary spherocytosis: → ↓ RBC survival, hemolysis, reticulocytosis, splenomegaly, jaundice, gall stones. • Treatment: Splenectomy

  27. Nutritional Anemia • a- Iron deficiency anemia (IDA; the commonest acquired anemia) • Causes: Blood loss, impaired iron absorption (↓ pH ), Increased requirement (growth, pregnancy, EPO therapy). • Functional IDA (Suppression of erythropoiesis due to inflammation), Erythropoietin deficiency

  28. a- Iron deficiency anemia • Treatment: Ferrous sulphate/ gluconate/ fumerate, or iron polymaltose orally, • Intravenous iron succharate or iron dextran (only when oral iron is intolerable, unabsorpable, or ineffective) • Liver ( good for iron & B12 deficiency)

  29. b-megaloblastic anemia • i-Folate deficiency: MTHFR mutation→ hyperhomocysteinemia due to defective folate metabolism, Eating only cooked food, lack of vegetables and fruits, Hemolytic anemia anti-folate drugs (e.g. methotrexate), • Treatment: folate supplement

  30. b-megaloblastic anemia • Ii-Vitamin B12/Cobalamin deficiency: vegetarian diet, Gastric, pancreatic or terminal elium resection, lack of gastric acidity, lack of intrinsic factor, intestinal bacterial overgrowth, fish worm (Diphyllobothrium latum), Crohn’s diseases, metformin

  31. Role of MTHFR

  32. MTHFR mutation

  33. Cobalamin/B12 absorption

  34. Hemolytic anemia • i-Autoimmune: IgG (Warm): -1ry, -2ry: autoimmune diseases, lymphoid malignancies, drug-induced • ii-Autoimmune: IgM (cold), • Iii-Non-immune: RBC abnormalities (Sickle, thalassema, spherocytosis, enzyme deficiency, • MAHA

  35. Hemolytic anemia • Treatment of 1ry warm, autoimmune hemolytic anemia ; steroids, IgG, Rituximab (anti-CD 20), splenectomy • 2ry: treatment of underlying disease

  36. Anemia of Chronic Illnesses • Functional iron deficiency+ Erythropoietic inhibitors • Infection, • Connective tissue/autoimmune diseases • Malignancies • Treatment: Transfusion, Treat underlying illnesses, mega doses of erythropoietic stimulants

  37. Summery

  38. Diagnosis • Gender, Age, Nutrition (meats, vegetables), blood loss, illnesses, drugs (ASA, Anticoagulants, Anti-inflammatory) • Physical examination, • Laboratory investigations, CBC, ESR, Reticulocytes, Blood film, Serum Fe , TIBC (transferrin), Ferritin, Creatinine, Folate level (serum + RBC), B12, Hb electrophoresis, • Diagnosis of underlying illnesses ; Autoimmune, infection, cancer, etc.

  39. Treatment of Anemia • Improve nutrition, • Prevent blood loss, • Treat underlying illnesses, • Specific therapy: ( Iron, folic acid, vitamin B12, Erythropoietin)

  40. Iron therapy • Hem iron • Non-hem iron • Oral iron (Ferrous sulphate, gluconate, fumerate ), Iron polymaltose, • Absorption enhancers & inhibitors, • Parenteral iron therapy (indications, iron saccharate, iron dextran) • Response/ Complications

  41. Folate , B12 & Epo • Folate treatment, • Folate supplement, • Polyglutamate/monoglutamate • B12 (IM) • Erythropoietin (Epo): • a-Chronic renal failure (hemodialysis), b-Anemia of cancer, AIDS, chronic illnesses

  42. Treatment failures • Occult continuous blood loss • Occult deficiencies, • Erythropoietic inhibitors

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