A 67-year-old man was initially diagnosed for idiopathic thrombocytopenic purpura because of persistent thrombocytopenia. Due to his failure in response to prednisone and intravenous immunoglobulin treatment, the patient finally had a splenectomy. Pathological examination of the spleen showed extramedullary hematopoiesis and his platelet counts stayed at a low level. In the current admission, the patient presented with fever and hypoxemia and was suspected to have either pneumonia or pulmonary embolism (PE). Chest X-ray did not demonstrate pulmonary infiltrate. CT scan did not show any evidence of PE, but revealed generalized lymphadenopathy. Lymphadenopathy became increasingly prominent during the hospital course and a lymph node biopsy was performed (Figs. 32.1 and 32.2). The patient recalled that his fingers and lower extremities sporadically became white and icy cold with blue fingernails. Serum protein electrophoresis showed monoclonal gammopathy. Immunoglobulin quantitation revealed IgG 4690 mg/dl, IgA 614 mg/dl, IgM 339 mg/dl, kappa 3160 mg/dl, and lambda 1022 mg/dl.


Acute Myeloid Leukemia Mantle Cell Lymphoma Idiopathic Thrombocytopenic Purpura Hematologic Neoplasm Extramedullary Hematopoiesis 
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