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Familial hemophagocytic lymphohistiocytosis in Israel: II. Pathologic findings
Author(s) -
Soffer Dov,
Okon Elimelech,
Rosen Nechama,
Stark Batia,
Hershko Chaim
Publication year - 1984
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(19841201)54:11<2423::aid-cncr2820541119>3.0.co;2-4
Subject(s) - hemophagocytosis , histiocyte , medicine , malignant histiocytosis , hemophagocytic lymphohistiocytosis , pathology , histiocytosis , differential diagnosis , bone marrow , lymph , disease , pancytopenia
Presented are the pathologic findings in familial hemophagocytic lymphohistiocytosis (FHLH), based on observation of 11 cases from four affected families. The outstanding morphologic feature was a multiorgan involvement with lymphohistiocytic cellular infiltrates. The organs most frequently affected were the bone marrow and lymph nodes and, less frequently, the liver and the brain. The histiocytes in the infiltrates were usually bland and displayed prominent hemophagocytosis. The differential diagnosis of FHLH and the difficulty in distinguishing it from malignant histiocytosis is discussed. In view of the focal distribution of lesions, it is suggested that histopathologic diagnosis of FHLH be made only after evaluation of combined findings in several organs and consideration of the patient's family history as well as the result of the clinical and laboratory investigations.