1. Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan Outcome : The student will know : -The causes and pathogenesis of aplastic anemia. -The diagnosis of aplastic anemia. -The laboratory tests needed for diagnosis

2. Aplastic Anaemia.

3. APLASTIC ANEMIA Aplastic anemia is a severe, life threatening syndrome in which production of erythrocytes, WBCs, and platlets has failed. Aplastic anemia may occur in all age groups and both genders. The disease is characterized by peripheral pancytopenia and accompanied by a hypocellular bone marrow.

4. Definition of aplastic anaemia. Peripheral blood pancytopenia and a hypocellular marrow in which normal marrow is replaced by fat cells. Abnormal cells are not found.

5. Normal Haemopoiesis. Red cell life span 120 days. Platelet life span 6 days. Granulocyte life span < 24 hours. Constant marrow activity needed to replace dead cells.

6. Pathogenesis. Primary defect or damage to haematopoietic stem cell. possible Immunological attack on stem cells.. Defective microenvironment (i.e. marrow stromal defect)

7. Haematopoietic stem cell.

8. Pathophysiology of aplastic anemia

9. Aetiology.(Causes ) INHERITED(20%) oFanconi Anaemia oDyskeratosis congenita oShwachman-Diamond syndrome oDiamond-Blackfan anaemia. ACQUIRED(80%) oIdiopathic oDrug induced oViral (hepatitis, EBV) oIonising radiation oToxins (pesticides, benzene, arsenic) oPregnancy oleukaemic

10. Clinical Features. Anaemia; tiredness & fatigue, palpitations. Low white count; recurrent infections. Low platelets; easy bruising and bleeding.

11. Investigations. CBC ( Hb < 10 g /dl, TLC < 4000 /cmm, platelets < 100,000 /cmm ) Reticulocyte count ( decreased ) Blood film. B12/folate. Liver function tests Virology Bone marrow aspirate & trephine

12. APLASTIC ANEMIA – Lab findings » Severe pancytopenia with relative lymphocytosis (lymphocytes live a long time) » Normochromic, normocytic RBCs » Mild to moderate anisocytosis and poikilocytosis » Decreased reticulocyte count » Hypocellular bone marrow with > 70% yellow marrow

13. Severe Aplastic Anaemia Peripheral Blood oGranulocytes <0.5 x 10 9 /l oPlatelets <20 x 10 9 /l oReticulocytes <1% Marrow trephine Markedly hypocellular <25% normal

14. Marrow trephine

15. HYPOCELLULAR BONE MARROW IN APLASTIC ANEMIA

16. OTHER HYPOPROLIFERATIVE ANEMIAS Renal disease – due to decreased erythropoietin Endocrine deficiencies – may lead to decreased erythropoietin production. For example: hypothyroidism leads to decreased demand for oxygen from tissues; decreased androgens in males; decreased pituitary function

17. Assignement : TopicStudent Names Acquired aplastic anaemia حماده محمد عشري

18. Training questions : What are the laboratory findings in aplastic anaemia ? How to diagnose Aplastic anaemia ? Reference book : Essential Hematology. Dacie.