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Aplastic Anemia
Aplastic anemia is a bone marrow failure
syndrome characterized by peripheral
pancytopenia and marrow hypoplasia.
Fanconi anemia
Dyskeratosis congenita
Shwachman-Diamond syndrome
Familial aplastic anemia
Acquired:
1. Drugs
- Cytotoxic drugs
- Antibiotics
- Chloramphenicol
- Anti-inflammatory
- Anti-convulsant
- Sulphonamides
- 2-3 months usually between exposure and the development of aplastic anemia.
Inevitable:
Idiosyncratic:
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
skin pigmentation,
short stature,
microcephaly,
hypogonadism,
mental retardation,
skeletal anomalies.
FBC
Reticulocyte count
Blood film.
B12/folate.
Liver function tests
Virology
Bone marrow aspirate & trephine
PNH screen.
BM Aspiration
BM Biopsy
BM biopsy
hypocellular ,increased fat spaces
Hb
<10g/dL
ANC
<1,5 G/L
PL
<100 G/L
2. Bone marrow histology and cytology
- decreased marrow cellularity (< 25%)
- increased fat cells component
- no extensive fibrosis
- no malignancy or storage disease
Pancytopenia
Acute Myelogenous Leukemia
Anemia
Aplastic Anemia
Hairy Cell Leukemia
Paroxysmal Nocturnal Hemoglobinuria
Immune pancytopenias in connective tissue
disorders (eg, systemic lupus
erythematosus, refractory anemia)
Causes of pancytopenia
1.Failure of production of blood cells
a) bone marrow infiltration
- acute leukemias
- hairy cell leukemia
- multiple myeloma
- lymphoma
- myelofibrosis
- metastatic carcinoma
b) aplastic anemia
2. Ineffective hematopoesis
- myelodysplastic syndrome
- vit.B12 and folate deficiency
Androgens
Growth factors
- Antilymphocyte glob.
- Anti T cells abs.
2. Restrictions
- age below 40
- suitable donor available in less than 30% (sibling)
- 25-40% risk of GVHD
- 5-15% risk of graft failure in multitransfused patients
- high mortality after MUD-HSCT
- solid tumors (12%)
Immunosuppressive therapy
Indicated for patients > 40 years
Patients with no HLA matched sibling
donors.
Anti-Thymocyte Globulin(ATG) or antilymphocyte globulin (ALG), cyclosporin,
methylprednisolone.
Best results are for combination therapy.
Response is slow, 4-12 weeks to see early
improvement.
Immunosuppressive therapy
Immunosuppressive therapy
Antithymocyte globulin, equine (Atgam) - 10-20
mg/kg/day for 8-14 days.
Antithymocyte globulin, rabbit (Thymoglobulin) - 0,75
mg/kg/day for 8 days.
Cyclosporine (Sandimmune, Neoral) - 1.5-2 mg/kg IV
q12h,
Methylprednisolone (Medrol, Solu-Medrol) - :5 mg/kg IV
on days 1-8; then tapered using PO 1 mg/kg on days 9-14;
further tapering over days 15-29. Stop after 1 mo except in
evidence of serum sickness.
Cyclophosphamide (Cytoxan) : 45 mg/kg/d IV for 4 d.
Immunosuppressive therapy 2
Response rates 60-70%
Relapses are common and continued
supportive care needed.
Up to 50% of relapsed patients will respond
to 2nd course of immunosuppressive
therapy.
Infections
Bleeding
Iron overload
Complications of BMT
Graft versus host disease
Graft failure