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Disseminated histiocytosis associated with atypical lymphoid cells (lymphohistiocytosis)
Author(s) -
Berard Costan W.,
Cooper Richard A.,
Freireich Emil J,
Rabson Alan S.
Publication year - 1966
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(196610)19:10<1429::aid-cncr2820191016>3.0.co;2-m
Subject(s) - medicine , pancytopenia , hepatosplenomegaly , fever of unknown origin , malignant histiocytosis , pathology , prednisone , hemophagocytosis , lymphoma , lymph node biopsy , histiocytosis , bone marrow , sinus histiocytosis with massive lymphadenopathy , histiocyte , surgery , disease
Biopsy of a cervical lymph node from a 3 1/2‐year‐old Caucasian boy originally was diagnosed as malignant lymphoma, lymphocytic type. Following therapy with radiation and prednisone, the patient had transient pancytopenia but was free of apparent disease for the subsequent 15 months. At age 5 he presented with fever, induration and congestion of the lower abdomen and scrotum, splenomegaly, abnormal liver function, hepatomegaly, lymphadenopathy and progressive pancytopenia. Biopsies of axillary and abdominal adipose tissue, cervical and axillary lymph nodes, liver and bone marrow were suggestive of, but not diagnostic of, malignant lymphoma. Despite therapy with prednisone, cyclophosphamide, and vincristine, the patient had continued fever and progressive hepatosplenomegaly and expired on the sixty‐first hospital day. At autopsy there was no evidence of lymphocytic lymphoma but there was disseminated histiocytosis involving liver, spleen, bone marrow, lymph nodes, diaphragm and abdominal panniculus. In retrospect, occasional similar histiocytes were noted in lymph node sections from 2 years previously. The authors interpret this case as representative of disseminated lymphohistiocytosis, an apparently familial disease with distinctive clinicopathologic features.