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[伴有髓样脾肿大型血液学表现的系统性肥大细胞增多症]

[Systemic mastocytosis with myeloid splenomegaly type hematological manifestations].

作者信息

Krulik M, Brivet F, Audebert A A, Curioni S, Debray J

出版信息

Ann Med Interne (Paris). 1977 Mar;128(3):245-53.

PMID:869393
Abstract

The authors report the case of a patient suffering from systemic mastocytosis with cutaneous, medullary, bone, hepatic and splenic involvement, the clinical and haematological picture of which was that of myeloid splenomegaly. Furthermore, this picture included hypersplenism, clearly demonstrated by isotopic study and the successful results of splenectomy. Blood histamine levels were normal though urinary excretion of histamine was increased. Histamine levels in the spleen were greatly increased. Serotonin levels in the blood and its urinary metabolites were normal. Heparin activity was demonstrated in the spleen, but not in the blood.

摘要

作者报告了一例患有系统性肥大细胞增多症的患者,其皮肤、骨髓、骨骼、肝脏和脾脏均受累,临床和血液学表现为髓样脾肿大。此外,该表现还包括脾功能亢进,同位素研究和脾切除的成功结果清楚地证明了这一点。血液组胺水平正常,尽管组胺的尿排泄增加。脾脏中的组胺水平大幅升高。血液中的血清素水平及其尿代谢产物正常。脾脏中显示有肝素活性,但血液中未显示。

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