Soffer D, Okon E, Rosen N, Stark B, Hershko C
Cancer. 1984 Dec 1;54(11):2423-31. doi: 10.1002/1097-0142(19841201)54:11<2423::aid-cncr2820541119>3.0.co;2-4.
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.
本文基于对来自四个患病家族的11例病例的观察，呈现了家族性噬血细胞性淋巴组织细胞增生症（FHLH）的病理表现。突出的形态学特征是多器官受累伴淋巴细胞和组织细胞浸润。最常受累的器官是骨髓和淋巴结，较少受累的是肝脏和脑。浸润中的组织细胞通常形态温和，呈现显著的噬血现象。文中讨论了FHLH的鉴别诊断以及将其与恶性组织细胞增多症区分开来的困难。鉴于病变的局灶性分布，建议仅在评估多个器官的综合表现、考虑患者家族史以及临床和实验室检查结果后，再做出FHLH的组织病理学诊断。
