Thrombotic thrombocytopenia purpura in a patient with systemic sclerosis.
Thrombotic thrombocytopenia purpura in a patient with systemic sclerosis.
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系统性硬化症患者的血栓性血小板减少性紫癜。
DOI：
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发表时间：
2001
期刊：
影响因子：
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通讯作者：
P. Clements
中科院分区：
文献类型：
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作者：
Joe Yusin;K. Lewin;P. Clements
过去，血栓性血小板减少性紫癜（TTP）与硬皮病肾危象（SRC）有关。然而，在开始使用 ACE 抑制剂后，此类报道明显减少。最近，有报道再次出现，描述硬皮病患者出现 TTP 的临床证据。我们描述了一位患有长期局限性皮肤硬皮病的 50 岁女性，她出现便血和血小板减少以及其他提示 TTP 的表现。结肠活检显示腔内有血栓。她的病程因肾衰竭和高血压而复杂化，单独使用血管紧张素转换酶抑制剂治疗没有反应。经过一个血浆置换疗程后，她的情况有所好转。她出院回家，两个月后又因癫痫大发作和高血压而返回。在她的治疗过程中，她患上了成人呼吸窘迫综合症。她再次对血浆交换产生反应，并出院回家。两年来她一直保持稳定。本报告强调了对患有长期局限性皮肤硬皮病、患有肾功能衰竭、高血压和血小板减少症并伴有多器官并发症的患者进行全面评估的重要性。应考虑所有可能的病因，包括 SRC、TTP、血管炎和败血症。组织活检（在这种情况下，结肠活检显示血管腔内有血栓）可能有助于诊断。对于ACE抑制剂治疗失败的患者，可以考虑血浆置换。
Thrombotic thrombocytopenic purpura (TTP) has been associated with scleroderma renal crises (SRC) in the past. However such reports markedly diminished after the onset of ACE inhibitor use. Recently, reports again have surfaced that describe scleroderma patients presenting with clinical evidence of TTP. We describe a 50-year-old female with longstanding limited cutaneous scleroderma who presented with hematochezia and thrombocytopenia along with other findings suggesting TTP. A colon biopsy revealed thrombi within the lumen. Her course was complicated by renal failure and hypertension that did not respond to ACE inhibitor therapy alone. She improved after a course of plasma exchange. She was discharged home only to return 2 months later with grand mal seizures and hypertension. During her course she developed adult respiratory distress syndrome. She again responded to plasma exchange and she was discharged home. She has remained stable for 2 years. This report emphasizes the importance of fully evaluating patients with longstanding limited cutaneous scleroderma who present with renal failure, hypertension, and thrombocytopenia in association with multiorgan complications. All possible etiologies, including SRC, TTP, vasculitis, and sepsis should be considered. Tissue biopsies (in this case, a colon biopsy revealed thrombi within the vessel lumen) may prove beneficial in assisting with the diagnosis. For such patients who fail treatment with ACE inhibitors, plasma exchange may be considered.
影响因子：
158.5
作者：
BELL, WR;BRAINE, HG;KICKLER, TS
通讯作者：
KICKLER, TS