Aplastic anemia.pptx

Aplastic anemia and its management

1. Aplastic anemia Dr. Muhammad Adnan

2. Case scenario 7 years old boy presented to p aeds opd with pallor, easy fatigability and multiple bruises on whole body. Patient have history of multiple blood transfusions in past. On examination patient was pale looking having pale conjunctivae,increased heart rate and multiple bruises mainly on abdomen and lower extremities

3. Investigations: CBC Hb 2.9 gm/dl TLC 2410 /uL RBC 0.81 millions /uL Platelets 4000/ uL Diagnosis? Bone marrow exam findings? Management?

4. Definition Pancytopenia with hypocellularity ( Aplasia ) of Bone Marrow Aplastic anemia can present initially with a single cell line failure before the onset of pancytopenia It may be inherited or acquired.

5. Clinical features Anemia: pallor and/or signs of congestive heart failure, such as shortness of breath. • Thrombocytopenia: bruising ( eg , ecchymoses , petechiae ) on the skin, gum bleeding, or nosebleeds. Neutropenia :fever, cellulitis , pneumonia, or sepsis jaundice and evidence of clinical hepatitis in subset of patients adenopathy or organomegaly should suggest an alternative diagnosis.

6. cont In any case of aplastic anemia, look for physical stigmata of inherited marrow failure syndromes such as: • skin pigmentation short stature microcephaly hypogonadism mental retardation skeletal anomalies

7. Investigations CBC Peripheral Smear Reticulocyte count B12/ folate . Liver function tests Virology Bone marrow aspirate & biopsy

8. cont Anemia is common, and red cells appear morphologically normal. The reticulocyte count usually is less than 1%. Thrombocytopenia, with a paucity of platelets in the blood smear. Agranulocytosis ( ie . decrease in all granular white blood cells, including neutrophils , eosinophils , and basophils ) and a decrease in monocytes are observed. A relative lymphocytosis occurs. The degree of cytopenia is useful in assessing the severity of aplastic anemia.

9. Bone marrow exam A bone marrow biopsy is performed in addition to the aspiration. In aplastic anemia, these specimens are hypocellular . Only fat cells, fibrous stroma , scattered lymphocytes and plasma cells presents Bone marrow revels little materials (“Dry Tap”)

10. Management Supportive Care Supportive care manage the symptoms of aplastic anemia. This approach includes the use of: Blood transfusion Iron chelation to treat iron overload Growth factors Antibiotics

11. cont Definitive Therapy Acquired aplastic anaemia can be treated with either Hematopoietic stem cell transplantation (HSCT) Immunosuppressive therapy: ATG ( antithymocyte globulin) or ALG ( antilymphocyte globulin) •Cyclosporine