Sakakibara T, Ida T, Mannouji E, Kikuchi T, Tatsuno K, Kawase M, Imai M
Department of Surgery, Sakakibara Heart Institute, Tokyo, Japan.
J Cardiovasc Surg (Torino). 1989 Jul-Aug;30(4):687-91.
Six cases of post-operative erythroderma after open heart surgery are described. About 10 days after seemingly uneventful recovery, all patients developed fever, erythroderma, liver enzyme elevation, pancytopenia, and an aplastic bone marrow. Their condition rapidly deteriorated, and they died within 20 days of the onset of symptoms. Skin biopsy specimens from two patients showed mild leukocytic infiltration in the epidermal basal layer and upper dermis. Immunostaining by the ABC method showed that most of these infiltrating cells were suppressor/cytotoxic T cells. HLA study of peripheral lymphocytes from two patients and their families revealed that the patients' HLA phenotypes were incompatible from their children's HLA findings. Y chromatin was present in the lymphocytes of the skin biopsy specimen of a female patient. Based on the clinical picture, skin biopsy, HLA study, and Y chromatin study, the authors strongly suspect post-transfusion GVHD as the etiology of postoperative erythroderma, although these patients lacked any known immunodeficiency.
本文描述了6例心脏直视手术后发生术后红皮病的病例。在看似恢复顺利约10天后，所有患者均出现发热、红皮病、肝酶升高、全血细胞减少及再生障碍性骨髓。他们的病情迅速恶化，在症状出现后20天内死亡。两名患者的皮肤活检标本显示表皮基底层和真皮上层有轻度白细胞浸润。采用ABC法进行免疫染色显示，这些浸润细胞大多为抑制性/细胞毒性T细胞。对两名患者及其家属外周淋巴细胞进行的HLA研究表明，患者的HLA表型与其子女的HLA结果不相容。一名女性患者皮肤活检标本的淋巴细胞中存在Y染色质。基于临床表现、皮肤活检、HLA研究和Y染色质研究，作者强烈怀疑输血后移植物抗宿主病是术后红皮病的病因，尽管这些患者没有任何已知的免疫缺陷。
