Successful Treatment with Alternating Pulse Methylprednisolone and High-Dose Intravenous .GAMMA.-Globulin in a Case of Kasabach-Merritt Syndrome.
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A 1-month-old male infant was admitted to our hospital for petechiae over the extremities. Physical examination showed a firm mass at the left upper part of the abdomen. Laboratory findings were; hemoglobin 6.0 g/dl, platelet count 4, 000/μ1, prothrombin time 18.0 sec (control 11.3 sec), activated partial thromboplastin time 57.0 sec (control 33.6 sec), fibrinogen 33 mg/dl, and fibrinfibrinogen degradation products 40 μg/ml. Because ultrasound, CT, MRI scans and angiography suggested a pancreatic hemangioma, Kasabach-Merritt syndrome was diagnosed. Unresponsive to treatment with prednisolone, gabexate mesilate and heparin, thrombocytopenia and hypofibrinogenemia persisted, and the tumor size remained unchanged. Soon after the initiation of alternating pulse methylprednisolone and high-dose intravenous γ-globulin therapy, platelet counts rapidly increased. During the treatment platelet antibody became negative, and platelet-associated IgG (PAIgG) decreased in an inverse relation to platelet counts. Subsequently blood coagulation data returned to normal, and the mass gradually reduced in size. The histology of biopsied tumor specimens proved the illness to be capillary hemangioma. Further regression of the mass was achieved by continuation of the therapy.
- 特定非営利活動法人 日本小児血液・がん学会の論文
特定非営利活動法人 日本小児血液・がん学会 | 論文
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