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恶性组织细胞增多症。组织细胞性髓性网状细胞增生症。

Malignant histiocytosis. Histiocytic medullary reticulosis.

作者信息

Ruuskanen O, Kero P, Rajamäki A, Ekfors T, Vilkki P, Nordman E

出版信息

Acta Paediatr Scand. 1978 Mar;67(2):249-54. doi: 10.1111/j.1651-2227.1978.tb16313.x.

Abstract

Three cases of malignant histiocytosis occurring in children aged 2 months, 10 months and 14 years, are described. In all children the diagnosis was based on anaemia, granulocytopenia or thrombocytopenia, splenomegaly and marked erythrophagocytosis by bone marrow and lymph node atypical histiocytes. Two children aged 10 months and 14 years, underwent splenectomy after which combined chemotherapy with cyclophosphamide, vincristine and prednisone (COP) was started. In the older child a complete remission was achieved. The younger child died soon after the onset of the treatment. The youngest child was treated with bleomycin, adriamycin, cyclophosphamide, vincristine and prednisone (BACOP). He died of pneumonia and sepsis two months after the start of the treatment.

摘要

本文描述了3例分别发生于2个月、10个月和14岁儿童的恶性组织细胞增多症。所有患儿的诊断依据均为贫血、粒细胞减少或血小板减少、脾肿大以及骨髓和淋巴结中不典型组织细胞显著的红细胞吞噬现象。两名分别为10个月和14岁的儿童接受了脾切除术,术后开始使用环磷酰胺、长春新碱和泼尼松(COP)联合化疗。年龄较大的患儿实现了完全缓解。年龄较小的患儿在治疗开始后不久死亡。最小的患儿接受了博来霉素、阿霉素、环磷酰胺、长春新碱和泼尼松(BACOP)治疗。治疗开始两个月后,他死于肺炎和败血症。

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