A Case of Hemophagocytic Syndrome.

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  • 咽頭痛，発熱を主訴とした血球貧食症候群例

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Abstract

A 22-year-old male with a persistant sore throat and fever was admitted to our department of otolaryngology. Physical examination revealed an erythematous pharynx, swollen tonsils with exudate and multiple swollen lymphnodes with tenderness in the neck. Although he underwent medical treatment under a diagnosis of infectious mononucleosis, his fever persisted and the level of transaminases in his blood increased.<br>Futhermore he developed pancytopenia and a pre-DIC state with a bleeding tendency. A bone marrow puncture revealed the proliferation of monocytes and histiocytes and phagocytosis. These pathologic findings as well as a high serum ferrit in level, multiple organ failure and hepatosplenomegaly suggested the diagnosis of hemophagocytic syndrome (HPS).<br>Malignant histiocytosis (MH) and virus-associated hemophagocytic syndrome (VAHS) are classified as define HPS, a type of hemopathy. Abnormal poliferation and infiltration of monocytes and histiocytes with active phagocytosis are noted in various organs such as bone marrow, liver, spleen, lung and lymph nodes. Hemophagocytosis is induced by the excess production of cytokines by activated T-lymphocytes and macrophages. Although the patient underwent chemotherapy (CHOP, MEP) as well as treatment for DIC, hedied of gastrointestinal homorrhage and sepsis caused by methicillin-resistant Staphyjococcus aereus (MRSA) and Pseudomonas aeruginosa.

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