二次性骨髄線維症,血球貪食症候群およびstage I,非Hodgkinリンパ腫を合併した肝のInflammatory Pseudotumor (IPT)の一例

A 73-year-old male was admitted to our hospital with pyrexia, weight loss, anorexia and liver tumor found by ultrasonography on April 6, 1992. Physical examination revealed hepatomegaly 5cm below the costal margin. Laboratory findings on admission were as follows: a hemoglobin of 7.3g/dl, a platelet count of 2.8×104/μl, elevated levels of lactic dehydrogenase and alkaline phosphatase as well as leucine aminopeptidase and γ-glutamyltranspeptidase. Bone marrow examinations revealed hemophagocytosis and myelofibrosis. Antibiotics therapy was then initiated, and symptoms and abnormal laboratory findings including hemophagocytosis improved. He had hepatectomy of the left lobe with the diagnosis of malignant hepatic tumor by imagings. Inflammatory pseudotumor (IPT) without evidence of malignancy was diagnosed microscopically. Biopsy of a enlarged paraesophageal lymph node in operation showed stage I, non-Hodgkin's lymphoma (NHL). The postoperative course was excellent, and the disappearance of myelofibrosis was found by the second bone marrow biopsy after lobectomy for the IPT. Clinical course with those analytical results suggest that myelofibrosis and hemophagocytosis are closely associated with IPT. And an etiologically important role of chronic inflammatory stimulation of IPT for developing of NHL is suggested.