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Pulmonary involvement in patients with Thrombotic thrombocytopenic purpura

发布于：2013年11月11日    文字：【大】【中】【小】

Pulmonary involvement in patients with Thrombotic thrombocytopenic purpura

Eur J Haematol. 2013 Oct

ABSTRACT

OBJECTIVES: To determine the frequency of pulmonary involvement in patients with thrombotic thrombocytopenic purpura (TTP).

METHODS: The experience of the Oklahoma TTP-HUS (hemolytic-uremic syndrome) Registry, a population-based cohort of consecutive patients without selection or referral bias, 1995-2012, was analyzed. Evidence for pulmonary involvement in patients with TTP was also documented with a systematic review of published reports.

RESULTS: Only one of 74 Registry patients with acquired severe ADAMTS13 deficiency (activity <10%) had clinically important pulmonary involvement (transient PaO₂ , 42 mm Hg; arterial O₂ saturation, 78%; normal chest x-ray). No clinically important pulmonary involvement occurred in the remaining 73 patients. The systematic review identified 144 articles with search terms for TTP and pulmonary involvement; seven, published 1978-2002, had evaluable individual patient data. Five articles described single patients; only one patient was documented to have severe acquired ADAMTS13 deficiency; in none of the five patients was pulmonary involvement clearly related to TTP. Two articles were case series of seven patients each; no patients had ADAMTS13 activity measured. Each of the 14 patients had potential etiologies other than TTP for pulmonary involvement.

CONCLUSIONS: Only one of 74 patients in the Oklahoma Registry had clinically important pulmonary involvement. A systematic review of published reports documented no clear evidence for pulmonary involvement resulting from TTP. Clinically important pulmonary involvement may be rare in patients with TTP because: [1] pulmonary microvasculature may be inherently resistant to the formation of platelet thrombi and [2] pulmonary function can be maintained in spite of multiple microvascular thrombi.

血栓性血小板减少性紫癜（TTP）为多系统受累疾病，中枢神经系统和肾脏是最常见受累器官。肺脏也为多血管器官，但是对既往文献的综述分析发现TTP中未发现明显肺受累明确证据；本文还对Oklahoma TTP-HUS (hemolytic-uremic syndrome) Registry、从1995-2012的所有74例获得性TTP分析发现，只有1例患者有明显肺受累表现（一过性动脉氧分压42mmHg，胸片正常）。本文同时提出TTP中明显肺受累少见可能是由于：（1）肺血管可能本身就不容易形成血小板栓子；（2）即使部分肺血管床形成微血栓，肺功能仍然能维持。

http://www.ncbi.nlm.nih.gov/pubmed/24164539