悪性組織球症およびその類縁疾患の臨床病理

Abstract

Clinicopathologic findings of 3 cases of malignant histiocytosis (MH), 8 cases of histiocytic medullary reticulosis (HMR), a case of familial hemophagocytic reticulosis (FHR), 8 cases of lymphoproliferative disorders terminating in HMR-like features (symptomatic HMR) and allied disorders are examined. Although it is problem whether MH is identical with HMR, there are some cases overlapping with each other. Pathologically, HMR has a broad spectrum condition containing etiology unknown, reactive, cataplastic, and neoplastic histiocytosis. Symptomatic HMR, especially the case of mosquitoe allergy is a very important on considering the pathogenesis of HMR. Because, as a result of prolonged mosquitoe allergy, immunodifficiency and abnormal T cell proliferation presented and eventually HMR-like feature progressed. It would consider that FHR is a special case of lymphoproliferative disorders rather than true histiocytosis. So called leukemic reticulosis or malignant reticulosis in Japan were heterogenous disorder including not only histiocytosis, but also T-cell, B-cell malignancy and monocytic leukemia.