Malignant histiocytosis と思われる症例について

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  • A Case of Presumed Malignant Histiocytosis
  • Malignant histiocytosis ト オモワレル ショウレイ ニ

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There is a disease entity the main symptoms of which include fever, emaciation, anemia, hepatosplenomegaly, icterus and lymphadenopathy and which, in most instances, proves fatal within 6 months after onset. The disease is histologically characterized by systemic proliferation of histiocytes and prohistiocytes. Scott & Robb-Smith, in 1939, and Rappaport, later in 1963, proposed to designate this pathologic condition as histiocytic medullary reticulosis and malignant histiocytosis, respectively. The nomenclature has been since a matter of controversy, particularly on pathological grounds. The disease is rare and sporadic. In a case we present herein, we termed the condition “malignant histiocytosis”.<br>The patient was a 68-year-old male farmer who presented symptoms of an enlarged left palatine tonsil and bilateral cervical lymphadenopathy. The following observation led to a diagnosis of “malignant histiocytosis”, although the diagnosis was not confirmed at biopsy: 1) there was no accleration of erythrocyte sedimentation rate, 2) thrombocytopenia was present, 3) there was progressive anemia, 4) a mild degree of leukocytopenia, 5) hepatosplenomegaly with associated liver function impairment and jaundice, 6) enlargement of cervical, axillary and inguinal nodes, 7) numerous atypical (histiocyte-like) cells in the peripheral blood and bone marrow aspirates, 8) a temperature over 38°C persisted for a considerable period of time, and 9) the disease ran an acute course, ending with death of the patient after 3 mohths. VEMP therapy proved to be effective in controlling swelling of palatine tonsil and cervical lymphadenopathy but the systemic symptoms were not alleviated. Autopsy was not performed. The main pathological finding at biopsy were non specific inflammation. The palatine tonsil and the cervical lymph node showed infitration of abnormal histiocytic cells, intermingling with other kinds of cells such as, lymphoid cells, plasma cells, eosinophils. Death was attributed to acute cardio-vascular insufficiency.

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