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B‐cell lymphoma associated with haemophagocytic syndrome (HPS) is extremely rare in Western countries but has recently been increasingly reported in Asian countries. We describe seven patients with B‐cell lymphoma associated with HPS, six males and one female, age range 41–82 years (median 63 years). All patients had fever and splenomegaly, and six of the seven patients had hepatomegaly with no associated lymphadenopathy. The bone marrow showed haemophagocytosis and an infiltration of lymphoma cells. All patients showed increased levels of lactate dehydrogenase, C‐reactive protein, ferritin and soluble interleukin‐2 receptor. Lymphoma cells were positive for CD19, CD20 and surface immunoglobulin in all patients examined, and positive for CD5 in four of seven patients. Cytogenetic analyses of bone marrow cells showed a complex structural abnormality including chromosome 14q32 in two patients, 19q13 in three patients and deletion of the terminal part of 8p21 in six patients. The prognosis was poor; only two of the seven patients have survived in complete remission with a median survival of 11 months. These data suggested that B‐cell lymphoma associated with HPS might constitute a distinct biological and clinical disease entity. Abnormality of chromosome 19q13 and loss of 8p21 might be involved in the pathogenesis of this disease.

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A 38-year-old man, diagnosed as having multicentric Castleman's disease (plasma cell type) in 1995, had been treated with melphalan and prednisolone or prednisolone alone, but there was no remarkable response. In 2002, he was admitted to our hospital with a chief complaint of increasing dyspnea on effort. Laboratory data showed high serum IgG (10050 mg/dl), interleukin-6 (37.9 ng/ml), and vascular endothelial growth factor (VEGF 1920 pg/ml) levels. In addition, serum viscosity was very high (6.0 cp). Electrocardiogram, echocardiogram, and cardiac catheterization demonstrated pulmonary hypertension (PH). There were no other demonstrable causes of PH suggesting that PH was due to hyperviscosity syndrome and high VEGF level. He was treated with plasmapheresis, resulting in a transient improvement of dyspnea. Then, he was given humanized anti-interleukin-6 receptor antibody (tocilizumab), which resulted in the dramatic improvement of dyspnea and PH a few weeks later. PH is a rare complication of MCD, and could be successfully treated with tocilizumab.