A 71-year-old woman was admitted because of persistent fever for 2 weeks. A diagnosis of haemophagocytic lymphohistiocytosis (HLH) was made on the basis of persistent high fever, pancytopoenia, hyperferritinaemia, increased soluble interleukin-2 receptor (sIL-2R) levels and histiocytosis and hemophagocytosis in the bone marrow. CT showed neither infection nor lymphadenopathy. After administration of prednisolone, haematological findings improved and the fever resolved; however, the patient developed persistent fever after 6 months. In addition, levels of lactate dehydrogenase and sIL-2R increased again. CT scans revealed diffuse lymphadenopathy, and Hodgkin's lymphoma was diagnosed by lymph node biopsy. Progression of pancytopenia was observed, and bone marrow examination showed a relapse of HLH. After six courses of chemotherapy were given for Hodgkin's lymphoma, complete remission was achieved with no evidence of pancytopenia. Hodgkin's lymphoma may be the underlying cause in HLH cases of unknown aetiology. Hence, the clinical course should be carefully monitored even in the absence of lymphadenopathy.
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