tailieunhanh - Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 12)

Pure Red Cell Aplasia: Treatment History, physical examination, and routine laboratory studies may disclose an underlying disease or a suspect drug exposure. Thymoma should be sought by radiographic procedures. Tumor excision is indicated, but anemia does not necessarily improve with surgery. The diagnosis of parvovirus infection requires detection of viral DNA sequences in the blood (IgG and IgM antibodies are commonly absent). The presence of erythroid colonies has been considered predictive of response to immunosuppressive therapy in idiopathic PRCA. Red cell aplasia is compatible with long survival with supportive care alone: a combination of erythrocyte transfusions and iron chelation. . | Chapter 102. Aplastic Anemia Myelodysplasia and Related Bone Marrow Failure Syndromes Part 12 Pure Red Cell Aplasia Treatment History physical examination and routine laboratory studies may disclose an underlying disease or a suspect drug exposure. Thymoma should be sought by radiographic procedures. Tumor excision is indicated but anemia does not necessarily improve with surgery. The diagnosis of parvovirus infection requires detection of viral DNA sequences in the blood IgG and IgM antibodies are commonly absent . The presence of erythroid colonies has been considered predictive of response to immunosuppressive therapy in idiopathic PRCA. Red cell aplasia is compatible with long survival with supportive care alone a combination of erythrocyte transfusions and iron chelation. For persistent B19 parvovirus infection almost all patients respond to intravenous immunoglobulin therapy for example g kg daily for 5 days although relapse and retreatment may be expected especially in patients with AIDS. The majority of patients with idiopathic PRCA respond favorably to immunosuppression. Most first receive a course of glucocorticoids. Also effective are cyclosporine ATG azathioprine cyclophosphamide and the monoclonal antibodydaclizumab an antibody to the IL-2 receptor. PRCA developing on erythropoietin therapy should be treated with immunosuppression and withdrawal of erythropoietin. Myelodysplasia Definition The myelodysplasias MDSs are a heterogeneous group of hematologic disorders broadly characterized by cytopenias associated with a dysmorphic or abnormal appearing and usually cellular bone marrow and by consequent ineffective blood cell production. A clinically useful nosology of these entities was first developed by the French-American-British Cooperative Group in 1983. Five entities were defined refractory anemia RA refractory anemia with ringed sideroblasts RARS refractory anemia with excess blasts RAEB refractory anemia with excess blasts in transformation .