An Autopsy Case of Mixed Connective Tissue Disease Complicated by Thrombotic Thrombocytopenic Purpura

被引：1

作者：

Furuya, Makiko Yashiro ^{[1
]}

Watanabe, Hiroshi ^{[1
]}

Sato, Shuzo ^{[1
]}

Fujita, Yuya ^{[1
]}

Tenmoku, Jumpei ^{[1
]}

Matsuoka, Naoki ^{[1
]}

Asano, Tomoyuki ^{[1
]}

Suzuki, Eiji ^{[2
]}

Kobayashi, Hiroko ^{[1
]}

Migita, Kiyoshi ^{[1
]}

机构：

[1] Fukushima Med Univ, Dept Rheumatol, Sch Med, Fukushima, Japan

[2] Ohta Nishinouchi Hosp, Div Rheumatol, Koriyama, Fukushima, Japan

来源：

INTERNAL MEDICINE | 2020年 / 59卷 / 10期

关键词：

mixed connective tissue disease (MCTD); thrombotic thrombocytopenic purpura (TTP); a disintegrin-like metalloproteinase with thrombospondin type 1 motif; member 13 (ADAMTS 13); autopsy; MICROANGIOPATHIC HEMOLYTIC-ANEMIA; SYSTEMIC-LUPUS-ERYTHEMATOSUS; FACTOR-CLEAVING PROTEASE; UREMIC SYNDROME; PATIENT; FEATURES; COHORT;

D O I：

10.2169/internalmedicine.3939-19

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

We herein report a patient with mixed connective tissue disease (MCTD) who had been stable for years but suddenly developed thrombotic thrombocytopenic purpura (TTP). The patient showed a clinical pentad of signs of TTP, low activity of ADAMTS13, and positivity of anti-ADAMTS13 antibodies. She did not respond to plasma exchange or steroid therapy and died five days after admission. An autopsy revealed microthrombi in the brain, heart, kidney, adrenal glands, esophageal submucosa, and bone marrow as well as diffuse alveolar hemorrhaging. Physicians should bear in mind that TTP can occur in MCTD patients regardless of disease activity.

引用

页码：1315 / 1321

页数：7

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