Text book Treatment Treatment of underlying cause –if possible Removal of cause Supportive care –Blood & platelet transfusion –Infection: Broad spectrum antibiotics –Asepsis Bone Marrow Transplant (SCT) –patient age <40yrs, availability of a HLA- identical sibling marrow donor
Immunosuppression: –Cyclosporine, –Glucocorticoids : in cong Pure Red Cell Aplasia –Antilymphocyte or Antithymocyte globulin (ALG / ATG) –Cyclophosphomide Androgens Thymectomy : for Adult Pure Red Cell Aplasia
Case History: My first post-Cyclophosphamide white cells appeared 10 days after treatment. I had 6. I ordered them all little party hats, and got to know them personally:-)
Clinical Features Mouth infection,Sore throat ( Mucositis) Ulcers of : Mouth & throat, Skin, Anus Features of Sepsis (Gm +ve &–ve): –Fever +/- –Hypotension, –MODS In prolonged neutropenia Fungal infections are likely to develop: Candida (Oral), Aspergillus(Pulm)
Investigations and Treatment The peripheral blood smear shows a marked decrease or absence of neutrophils. The bone marrow may show myeloid hypoplasia or absence of myeloid precursors. In many cases, the bone marrow is cellular with a maturation arrest at the promyelocyte stage. On occasion, the marrow may be hypercellular.