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

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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. ...

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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, 0.4 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 (RAEB-t), and chronic myelomonocytic leukemia (CMML). The World Health Organization classification (2002) recognizes that the distinction between RAEB-t and acute myeloid leukemia is arbitrary and groups them together as acute leukemia, notes that CMML behaves as a myeloproliferative disease, and separates refractory anemias with dysmorphic change restricted to erythroid lineage from those with multilineage changes (Table 102-5).
Table 102-5 World Health Organization Classification of Myelodysplastic Syndromes Disease Freque Blood Bone Prognos ncy Findings Marrow is Findings Refractory 5–10% Anemia Erythro Protract anemia (RA) id dysplasia ed course No or only rare blasts Leukem
(RARS) ≥15% ia in ~1–2% ringed sideroblasts
ringed sideroblasts nias (2 or 3 ia in ≥10% of (RCMD-RS) lineages) cells in ≥2 lineages No or rare blasts ≥15% ringed No sideroblasts Auer rods
109/L Auer rods monocytes Refractory Cytope Uniline Progress anemia with excess nias age or ive BM failure blasts-2 (RAEB-2) multilineage 5–19% Leukem dysplasia blasts ia in ~33% 10– ±Auer 19% blasts rods ±Auer
Auer rods No Auer rods MDS with Unkno Anemia Nl or Long isolated del(5q) wn increased survival