Aplastic anemia
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Aplastic anemia

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Aplastic anemia Aplastic anemia Presentation Transcript

  • Aplastic Anemia Dr. Kalpana Malla MD Pediatrics Manipal Teaching HospitalDownload more documents and slide shows on The Medical Post [ www.themedicalpost.net ]
  • PANCYTOPENIA:• Simultaneous presence of anaemia, leukopenia, thrombocytopenia• Causes: aplastic anemia Subleukemic leukemia cytotoxic drugs radiotherapy bone marrow infiltration hypersplenism megaloblastosis SLE
  • APLASTIC ANEMIA:• Failure of two or more cell lines• Anaemia, leukopenia, thrombocytop enia + Hypoplasia or aplasia of the marrow
  • Pathology:• Reduction in the amount of haemopoietic tissue- inability to produce mature cells for discharge into the bloodstream• Patchy areas of normo/hypercellularity betn areas of hypocellularity
  • CLASSIFICATION:• Idiopathic• Secondary: idiosyncratic drug reaction chemical exposure infectious hepatitis paroxysmal nocturnal haemoglobinuria• Constitutional
  • CLASSIFICATIONCONSTITUTIONAL/CONGENITAL• Diamond-Blackfan syndrome• Shwachmann-Diamond syndrome• Fanconi anemia• Dyskeratosis Congenita• TAR (thrombocytopenia with absent radii)• Amegakaryocytic thrombocytopenia
  • FANCONI ANEMIA• Familial• AR• M:F=1.3:1• Onset in 1st decade of life• Diminished capacity for DNA repair and increased random chromosome breakage during mitosis
  • C/F:• Facies – microphthalmia,depressed nasal bridge,epicanthic fold,micrognathia• Hyperpigmentation, café-au-lait spots• Absent / hypoplastic thumb• Skeletal and renal lesions• Short stature,• Microcephaly , subnormal intelligence• Hypogonadism,ano malies of urinary tract• Predisposition to leukemias• Poor prognosis
  • DYSKERATOSIS CONGENITA• X-linked, AR, AD• M:F= 4.3:1• Hyperpigmentation• Nail dystrophy, early loss of teeth• Leukoplakia• Ocular abnormalities: cataract etc• Short stature but No skeletal/renal lesions (diff from FA)
  • DIAMOND BLACKFAN SYNDROME• Congenital pure red cell aplasia• AD, AR, Sporadic• Familial in 15 %• 90 % diagnosed in 1st year of life• Intrinsic defect in RBC, early apoptosis• Macrocytic anemia, reticulocytopenia, absence of RBC precursors in an otherwise normocellular bone marrow
  • DIAMOND BLACKFAN SYNDROME• Eye - Wide set eyes, blue sclera,glaucoma,epicanthic fold,cataract,strabismus• Thick upper,cleft lip palate in some cases• lip,intelligent expression• Upperlimb anomalies – flattening of thenar eminence, Triphalangeal thumb
  • C/F:• Profound anemia at 2-6 months of age• Short stature• Renal anomalies and hypogonadism maybe present
  • ACQUIRED APLASTIC ANEMIA - CAUSES• Radiation • Immune diseases:• Drugs and chemicals - eosinophilic fascitis - chemotherapy - thymoma - benzene • Pregnancy - chloramphenicol • PNH - antiepileptics • Marrow replacement:• Viruses: - leukemia - CMV - myelofibrosis - EBV - myelodysplasia - Hep B, C,D - HIV
  • PATHOPHYSIOLOGY• Direct destruction of haemopoietic progenitors• Disruption of marrow micro-environment• Immune –mediated suppression of marrow elements  Cytotoxic T cells in blood and marrow release gamma IFN and TNF -> inhibit early and late progenitor cells
  • Pathology:• Hallmark: peripheral pancytopenia with hypoplastic/ aplastic bone marrow
  • CLINICAL FEATURESRBC (anemia)• Progressive and persistent pallor• Anemia related symptomsWBC (Leucopenia/neutropenia)• Prone to infections - Pyodermas, OM, pneumonia, UTI, GI infections, sepsisPlatelets (Thrombocytopenia)• Petechiae, purpura, ecchymoses• Hematemesis, hematuria, epistaxis, gingival bleed• IC bleed- headache, irritability, drowsiness, coma
  • NO HEPATOMEGALY NO SPLENOMEGALY NO LYMPHADENOPATHYFailure of entire RES. No extramedullary hematopoesis
  • Blood picture:• Anemia-normocytic, normochromic• Leukopenia (neutropenia)• Relative lymphocytosis• Thrombocytopenia• Absolute reticulocyte count low• Mild to moderate anisopoikilocytosis
  • Other investigations• BM : dry aspirate, hypocellular with fat (>70% yellow marrow)
  • SEVERITYSEVERE APLASTIC ANEMIA• Granulocyte count <500/cu.mm• Platelet count <20,000/cu.mm• Reticulocytes <1%• BM < 25% of hematopoeitic cellsVERY SEVERE/ EXTREME APLASTIC ANEMIA• Above + Granulocyte count <200/cu.mm
  • DIFFERENTIAL DIAGNOSIS• ITP• LEUKEMIA• MYELOID METAPLASIA
  • Management:• Identification and elimination of underlying cause• Supportive therapy:1. Red cell transfusion for anemia2. Prevention and treatment of haemorrhage3. Prevention and treatment of infection
  • SUPPORTIVE CARE• Prophylactic antibiotics• If infection: cephalosporins + aminoglycosides + metronidazole• Antifungals: amphotericin B, fluconazole (if fever >10 days despite antibiotics)
  • THERAPYDEFINITIVE• BONE MARROW TRANSPLANTATION• IMMUNOSUPPRESSION
  • 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
  • IMMUNOSUPPRESSION• Antithymocyte globulin (ATG)• Antilymphocyte glubulin (ALG)• Cyclosporin• Intensive immunosupression : cyclophosphamide• Corticosteroids
  • ATG administration:• IV administration of Ig preparations containing antibody to human thymocytes• Improvement in haematological indices in one-half of subjects• Anaphylaxis
  • ATG AND ALG• Dose : 40 mg/kg/day X 4 days• Hematologic response rate 45%• Survival rate 60%• Side effects: serum sickness
  • 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
  • Corticosteroids:• High dose steroids combined with anabolic agents• Uncertain benefits• Side-effects
  • HAEMATOPOIETIC GROWTH FACTORS• GM-CSF• G-CSF• IL-3• IL-1• IL-6
  • Thank youDownload more documents and slide shows on The Medical Post [ www.themedicalpost.net ]