Aplastic anemia and its management

1. Dr. Muhammad Adnan

2. Case scenario
 7 years old boy presented to paeds 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.

6. 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.

7. 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

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

9. 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.

10. 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”)

12. 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

13. 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