Uploaded byAsif Zeb
Aplastic anemia
1. Aplastic anemia is a condition characterized by pancytopenia (low red blood cells, white blood cells, and platelets) due to bone marrow failure. 2. It can be caused by exposure to toxins, radiation, viruses, or immune system attacks on the bone marrow. The bone marrow is hypocellular with fatty replacement of hematopoietic tissue. 3. Symptoms include anemia, increased risk of infection, bruising/bleeding due to low blood cell counts. Diagnosis involves blood tests showing pancytopenia and a bone marrow biopsy revealing a hypocellular marrow. Treatment options include supportive care, immunosuppressants, bone marrow transplant, or androgens
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Aplastic anemia
- 1. Aplastic anemia Muhammad AsifZeb Lecturer Hematology Khyber Medical University Peshawar
- 2. Just like normaland aplastic marrow
- 3. The Bone Marrowis the blood Factory May be exposed to damage or failure
- 5. 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
- 6. Pancytopenia Primary bonemarrow 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.
- 7. Aplastic Anemia Definition: Pancytopeniawith hypocellularity (Aplasia) of Bone Marrow Aplastic anemia is a severe, life threatening syndrome in which production of erythrocytes, WBCs, and platlets has failed.
- 8. Aplastic anemia mayoccur in all age groups and both genders. Failure of the bone marrow percursors to produce mature cells. Characterized by hypocellular marrow and pancytopenia.
- 9. 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)
- 10. Idiosyncratic: Unpredictable to drugse.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
- 11. 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)
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- 13. substantial reductionin 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.
- 14. Pathogenesis Potential mechanisms: • Absentor 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.
- 15. Pathogenesis (Cont…) The latesttheory 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.
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- 18. Figure 4:Red spotsFigure 5:Bruise
- 21. Hematological findings: CBC: Pancytopenia: initiallyonly 1 or 2 parameters WBC < 2.0, Hb < 10. Plt. < 100. No gross morphological abnormalities. Anemia is usually NCNC. Reticulocytopenia.
- 22. BM Aspiration BMBiopsy
- 23. BM biopsy hypocellular ,increasedfat spaces
- 25. Hematological findings: (Cont…) BoneMarrow: – 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
- 26. Treatment Withdrawal ofetiological agents. Supportive. Restoration of marrow activity: – Bone marrow transplant – Immunosuppressive treatment - Prednisolone - Antilymphocyte glob. - Cyclosporin - Anti T cells abs. - Splenectomy – Androgens – Growth factors
- 27. 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.
- 28. 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.
- 29. Clinical Features ofFanconi’s Anemia Common Findings: Low birth weight Short stature Microcephaly Microphthalmia Microstomia Skeletal abnormalities, particularly of thumbs and radii Generalized increased pigmentation of skin
- 33. PURE RED CELLAPLASIA 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.