Nemoto K, Ohnishi Y
Second Department of Pathology, Niigata University School of Medicine, Niigata, Japan.
Acta Pathol Jpn. 1987 Nov;37(11):1811-22. doi: 10.1111/j.1440-1827.1987.tb02874.x.
Clinicopathologic findings of a case of familial hemophagocytic reticulosis (FHR), for which splenectomy, and biopsy of the liver and mesenteric lymph nodes were performed, is reported. Histologically, the proliferated cells consisted of not only histiocytes showing severe erythrophagocytosis, but also lymphoid cells of various sizes. The lymphoid cells had atypical features including mitotic figures and convoluted nuclei, and were suspected to be T cells as a result of immunohistochemical studies. We therefore consider that FHR is a special type of lymphoproliferative disease accompanied by severe proliferation of erythrophagocytic histiocytes rather than being a true histiocytic disease. Cytochemical and electron microscopic findings of the involved spleen are also presented.
报告了一例家族性噬血细胞性网状细胞增生症（FHR）的临床病理结果，该病例进行了脾切除术以及肝脏和肠系膜淋巴结活检。组织学上，增殖细胞不仅包括显示严重红细胞吞噬作用的组织细胞，还包括各种大小的淋巴细胞。这些淋巴细胞具有非典型特征，包括有丝分裂象和核卷曲，免疫组织化学研究结果怀疑它们是T细胞。因此，我们认为FHR是一种特殊类型的淋巴增殖性疾病，伴有红细胞吞噬性组织细胞的严重增殖，而不是真正的组织细胞疾病。文中还呈现了受累脾脏的细胞化学和电子显微镜检查结果。
