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[复发性Moschcowitz病的多器官受累。在治疗困境中脾切除术是否为最终手段?]

[Multi-organ involvement in recurrent Moschcowitz disease. Splenectomy as ultima ratio in a therapeutic dilemma?].

作者信息

Jakob A R, Hiller E

机构信息

Medizinische Klinik III, Klinikum Grosshadern der Ludwig-Maximilians-Universität München.

出版信息

Med Klin (Munich). 1998 Oct 15;93(10):624-6. doi: 10.1007/BF03042678.

Abstract

BACKGROUND

The symptoms primarily described in Morbus Moschcowitz included thrombocytopenic purpura and hemolytic anemia. In addition it presents with a variety of clinical manifestations depending on the organs involved (i.e. neurological, renal, gastrointestinal, cardiac involvement). It is a rare disease and the pathogenesis still remains unclear. The efficacy of derived therapeutical concepts can hardly be assessed in controlled trials.

CONCLUSION

Currently the main option seems to be plasma therapy. In non-responders surgical procedures (splenectomy) may be of benefit to the patient.

摘要

背景

Moschcowitz病主要描述的症状包括血小板减少性紫癜和溶血性贫血。此外,根据受累器官(即神经、肾脏、胃肠道、心脏受累)的不同,它还会表现出多种临床表现。这是一种罕见疾病,发病机制仍不清楚。在对照试验中很难评估衍生治疗概念的疗效。

结论

目前主要的治疗选择似乎是血浆疗法。对于无反应者,手术治疗(脾切除术)可能对患者有益。

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