Anemia

1. Anemia Kristine Krafts, M.D. | October 28, 2013

2. Anemia Outline • Background facts about blood • Anemia: general information • Anemia: specific types

3. Anemia Outline • Background facts about blood

4. Normal blood cells

6. Complete Blood Count (CBC) Hematocrit RBC Hemoglobin

7. Complete Blood Count (CBC) MCV MCHC hypochromic normochromic microcytic normocytic macrocytic

8. Additional Red Blood Cell Properties Size variation Shape anisocytosis poikilocytosis

9. Normal red blood cells

10. Anemia Outline • Background facts about blood • Anemia: general information

11. An (without) -emia (blood): a reduction below normal in hemoglobin or red blood cell number.

12. Symptoms of Anemia Pale skin, mucous membranes Jaundice (if hemolytic) Tachycardia Breathlessness Dizziness Fatigue

13. Anemia Outline • Background facts about blood • Anemia: general information • Anemia: specific types

14. Three Ways to Get Anemic Loseblood Destroy too much blood • Extracorpuscular reasons • Intracorpuscular reasons Make too little blood • Too few building blocks • Too few erythroblasts • Not enough room

15. Three Ways to Get Anemic Loseblood

16. Anemia of Blood Loss • Cause: traumatic, acute blood loss • At first, hemoglobin is normal! • After 2-3 days, see reticulocytes • Chronic blood loss is different (it causes iron deficiency anemia). Things you must know

17. Reticulocytes

18. Three Ways to Get Anemic Lose blood Destroy too much blood • Extracorpuscular reasons • Intracorpuscular reasons

19. Hemolytic Anemias • Intracorpuscular vs. extracorpuscular • Chronic vs. acute • Signs of destruction: ↑ bilirubin, ↑ LDH, ↓ haptoglobin • Signs of production: ↑ reticulocytes, nucleated red cells in blood

20. Reticulocytes (supravital stain)

21. Three Ways to Get Anemic Lose blood Destroy too much blood • Extracorpuscular reasons

22. Microangiopathic Hemolytic Anemia Things You Must Know • Physical trauma to red cells • Schistocytes • Find out why!

23. Red cells snagged on fibrin strand

24. Schistocytes

25. Triangulocyte

26. Causes of MAHA • Artificial heart valve • Malignancy • Obstetric complications • Sepsis • Trauma

27. Autoimmune Hemolytic Anemia • Warm AIHA • IgG • Spleen • Spherocytes • Cold AIHA • IgM, complement • Intravascular hemolysis • Agglutination Things You Must Know

28. Warm AIHA

29. Warm AIHA

30. Warm AIHA

31. Cold AIHA

32. Cold AIHA

33. patient red cells + AHG = agglutination Direct antiglobulin test (DAT)

34. Three Ways to Get Anemic Lose blood Destroy too much blood • Extracorpuscular reasons • Intracorpuscular reasons

35. Sickle Cell Anemia • Hemoglobinopathy (qualitative defect in hemoglobin) • Single amino acid substitution in beta chain of hemoglobin • Can be heterozygous or homozygous • Sickle cells are nasty: • Fragile (burst easily) • Get stuck in vessels Things You Must Know

36. Hemoglobin

37. Point mutation in  chain gene abnormal  chains (substitution of valine for glutamate) Hgb S Nasty! Aggregates and polymerizes on deoxygenation Red cell becomes sickle shaped Sickles clog up vessels… …plus, they are fragile

38. Sickle cell anemia

40. Sickle cell anemia: foot lesion

41. Sickle cell anemia: spleen

42. Clinical Findings in Sickle Cell Anemia • Blacks (8% are heterozygous) • Severity of disease is variable • Chronic hemolysis, vaso-occlusive disease, and  infections (autosplenectomy) • Treatment: prevent triggers, vaccinate, transfuse

43. Thalassemia Things You Must Know • Quantitativedefect in hemoglobin • Can’t make enough α or β chains • Variable disease severity • Hypochromic, microcytic anemia with increased RBC and target cells

44. α β γ δ birth Hgb F = α2γ2 Hgb A2 = α2δ2 Hgb A = α2β2 Hemoglobin chain development

45. Thalassemia

46. Thalassemia: Medullary expansion

47. Hereditary Spherocytosis Things You Must Know • Tons of spherocytes • Spectrin defect • Splenectomy is curative

48. z

49. Hereditary spherocytosis

50. Splenomegaly in hereditary spherocytosis