1. Aplastic Anemia
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Dr. Kalpana Malla
MD Pediatrics
Manipal Teaching Hospital
8. Pathology:
1. Absent or defective stem cells (marrow
failure)
2. Abnormal marrow microenvironment
3. Inhibition by abnormal clone of
hematopoietic cells
4. Immune mediated suppression of
hematopoietic cells –
Cytotoxic T cells in blood and marrow release
gamma IFN and TNF -> inhibit early and late
progenitor cells
17. Differential Diagnosis
Pancytopenia with hypocellular bone
marrow
1. Acquired aplastic anemia
2. Inherited aplastic anemia
3. Hypoplastic MDS
4. Hypoplastic AML
18. Pancytopenia with cellular bone
marrow
1. Primary bone marrow
diseases PNH
2. Bone marrow
lymphoma SLE,
Sjogren’s disease
3. Vitamin B12 and folate
deficiency Overwhelming
infection Brucellosis
1. MDS
2. Myelofibrosis
3. Hairy cell leukemia
4. Hypersplenism
5. Alcoholism
6. Sarcoidosis
19. Hypocellular bone marrow with or without
cytopenia
1. Q fever
2. Legionaires
disease
3. Mycobacteria
4. Tuberculosis
5. Hypothyroidism
6. Anorexia
nervosa
20.
21. Management:
• Identification and elimination of underlying
cause
• Supportive therapy:
1. Blood transfusion
2. Iron chelation to treat iron overload
3. Antibiotics
4. Growth factors
22. Blood Transfusion
• The 2 types of transfusion typically used for
aplastic anemia patients are:
• Red blood cell transfusion
• Hb < 6
• Platelet transfusion
• <10,000 / bleeding
• Maintain > 30,000 prior to ATG infusion
• White blood cells live for a very short time. So
patients with a low white count rarely get
transfusions of white blood cells.
23. Iron Chelation Therapy for Iron Overload
• Blood iron level checked regularly if patient get red
blood cell transfusions.
• Iron overload can start to become a problem after as few
as 20 units of red blood cells.
• ferritin of over 1,000 - treatment
• Deferasirox
• Deferoxamine
• Diferiprone
26. Definitive Therapy
Acquired aplastic anaemia can be treated with
either
•Hematopoietic stem cell transplantation (HSCT)
•Immunosuppressive therapy.
27. BONE MARROW TRANSPLANTATION
• Treatment of choice
• HLA matched donor. Usually siblings
• Long term survival rates: 60-70%
• Donor stem cells > 4 X 108 cells/kg
29. ATG administration:
• IV administration of Ig preparations containing
antibody to human thymocytes
• Improvement in haematological indices in one-half
of subjects
• Anaphylaxis
30. ATG AND ALG
• Dose : 40 mg/kg/day X 4 days
• Hematologic response rate 45%
• Survival rate 60%
• Side effects: serum sickness
31. ANDROGENS
• No longer have primary role
• Increase erythopoietin producion
• Stimulate erythroid stem cells
• Increase Hb levels in normal males
• methyl testosterone, testesterone enanthate,
oxymetholone, danazol
• Oral dose : 2-5mg/kg/day, IM dose: 1-2mg/kg/wk
• Side effects: cholestatic jaundice, masculinization
33. Treatment summary
These are the most common treatments for
aplastic anemia:
•Blood transfusions
•Growth factors
•Antibiotics
•Iron chelation
•Immunosuppressive drug therapy
•Stem cell transplantation
34.
35. Thank you
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Medical Post [ www.themedicalpost.net ]