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Aplastic anemia

Aplastic anemia is one of the stem cell disorder which leads to pancytopenia in the peripheral blood and decrease production of all cell line in bone marrow. it require bone marrow transplantation to cure the patient.

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Aplastic anemia

  1. 1. Aplastic anemia Muhammad Asif Zeb Lecturer Hematology Khyber Medical University Peshawar
  2. 2. Just like normal and aplastic marrow
  3. 3. The Bone Marrow is the blood Factory May be exposed to damage or failure
  4. 4. Pancytopenia – What??  Therefore it is the combination of anemia, leukopenia, and thrombocytopenia :- – Hb < 13.5 in males & 11.5 in females – Leucocyte count < 4x109 /l – Platelet count < 100x109 /l
  5. 5. Pancytopenia  Primary bone marrow disease 1. MDS 2. PNH 3. Myelofibrosis 4. Mylophthisis 5. Hairy cell leukemia 6. Aleukemic leukemia  Secondary to systemic disease 1. SLE 2. B12 or folate difficiency 3. Hypersplenism 4. Overwhelming infection 5. Brucellosis 6. Sarcoidosis 7. T.B.
  6. 6. Aplastic Anemia Definition:  Pancytopenia with hypocellularity (Aplasia) of Bone Marrow  Aplastic anemia is a severe, life threatening syndrome in which production of erythrocytes, WBCs, and platlets has failed.
  7. 7. Aplastic anemia may occur in all age groups and both genders.  Failure of the bone marrow percursors to produce mature cells. Characterized by hypocellular marrow and pancytopenia.
  8. 8. Etiology Acquired: More common Inherited: Fanconi anemia Acquired: Idiopathic: 65% 1. Drugs Inevitable: Dose related , reversible.g. cytotoxic drugs, ionizing radiation. The timing, duration of aplasia and recovery depend on the dose. Recovery is usual except with whole body irradiation. e.g., chemotherapeutic drugs)
  9. 9. Idiosyncratic: Unpredictable to drugs e.g., anti-inflammatory antibiotics, anti-epileptic, these agents usually do not produce marrow failure in the majority of persons exposed to these agents. - Cytotoxic drugs - Antibiotics - Chloramphenicol - Anti-inflammatory - Anti-convulsant - Sulphonamides
  10. 10. Aplastic Anemia: (Cont.) Acquired:  Radiations  Chemicals e.g., Benzene and pesticides  Viruses: – Hepatitis A, Non-A and Non-B – Herpes simplex – E-B virus – Parvovirus: Transient  Immune: SLE, RA (rheumatoid arthritis)
  11. 11. INHERITED(20%) o Fanconi Anaemia
  12. 12.  substantial reduction in the number of haemopoietic pluripotential stem cells, and a fault in the remaining stem cells or an immune reaction against them, which makes them unable to divide and differentiate sufficiently to populate the bone marrow.  A primary fault in the marrow microenviromnent has also been suggested but the success of stem cell transplantation (SCT) shows this can only be a rare cause because normal donor stem cells are usually able to thrive in the recipient's marrow cavity.
  13. 13. Pathogenesis Potential mechanisms: • Absent or defective stem cells (stem cell failure). • Abnormal marrow micro-environment. • Inhibition by an abnormal clone of hemopoietic cells. • Immune mediated suppression of hematopoiesis. It is believed that genetic factors play a role. There is a higher incidence with HLA (11) histo comp. Antigen. Immune mechanism is involved.
  14. 14. Pathogenesis (Cont…) The latest theory is: there is an intrinsic derangement of hemopoietic proliferative capacity, which is consistent with life. The immune mechanism I,e autoreactive T cells attempt to destroy the abnormal cells (self cure) and the clinical course and complications depend on the balance. If the immune mechanism is strong, there will be severe pancytopenia. If not, there will be myelodysplasia.
  15. 15. Clinical Features
  16. 16. Clinical Features
  17. 17. Figure 4:Red spots Figure 5:Bruise
  18. 18. Hematological findings: CBC: Pancytopenia: initially only 1 or 2 parameters WBC < 2.0, Hb < 10. Plt. < 100. No gross morphological abnormalities. Anemia is usually NCNC. Reticulocytopenia.
  19. 19. BM Aspiration BM Biopsy
  20. 20. BM biopsy hypocellular ,increased fat spaces
  21. 21. Hematological findings: (Cont…) Bone Marrow: – Hypocellular: <50% of normal cellularity Trephine biopsy is the most important for diagnosis. – Most of the cells present are lymphocytes, plasma cells. – Iron stores: increased
  22. 22. Treatment  Withdrawal of etiological agents.  Supportive.  Restoration of marrow activity: – Bone marrow transplant – Immunosuppressive treatment - Prednisolone - Antilymphocyte glob. - Cyclosporin - Anti T cells abs. - Splenectomy – Androgens – Growth factors
  23. 23. Inherited Anemia Fanconi’s Anemia:  The most common type of inherited aplastic anemias.  Associated with anomalies e.g., skeletal, skin.  Autosomal recessive. Genetics:  Increased sensitivity of the cells to chromosomal damage by DNA cross linking agents.  13 genes are responsible  IV54 mutation, is associated with multiple dysmorphism, severe pancytopenia, higher incidence of AML.
  24. 24. Inherited Anemia Clinical Features:  Skeletal and skin anomalies seen at birth e.g., microcephally.  Manifestations of marrow failure, usually later at age 5-10 yrs. Present as anemia, mucusal bleeding e.g. nasal.
  25. 25. Clinical Features of Fanconi’s Anemia Common Findings:  Low birth weight  Short stature  Microcephaly  Microphthalmia  Microstomia  Skeletal abnormalities, particularly of thumbs and radii  Generalized increased pigmentation of skin
  26. 26. PURE RED CELL APLASIA  PURE RED CELL APLASIA  Pure red cell aplasia is characterized by a selective decrease in erythroid precursor cells in the bone marrow. WBCs and platlets are unaffected.  — Acquired  Transitory with viral or bacterial infections  Patients with hemolytic anemias may suddenly halt erythropoiesis  Patients with thymoma – T-cell mediated responses against bone marrow erythroblasts or erythropoietin are sometimes produced.  — Congenital  Diamond-Blackfan syndrome – occurs in young children and is progressive. It is probably due to an intrinsic or regulatory defect in the committed erythroid stem cell.

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Aplastic anemia is one of the stem cell disorder which leads to pancytopenia in the peripheral blood and decrease production of all cell line in bone marrow. it require bone marrow transplantation to cure the patient.

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