A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity

Author:

Yamada Risa12ORCID,Nozawa Kazuhisa12,Yoshimine Takashi3,Takasaki Yoshinari1,Ogawa Hideoki4,Takamori Kenji4,Sekigawa Iwao24

Affiliation:

1. Department of Rheumatology, School of Medicine, Juntendo University, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan

2. Department of Internal Medicine and Rheumatology, Juntendo University Urayasu Hospital, Chiba 279-0021, Japan

3. Department of Internal Medicine, Juntendo University Urayasu Hospital, Chiba 279-0021, Japan

4. Institute for Environment and Gender Specific Medicine, Juntendo University Graduate School of Medicine, Chiba 279-0021, Japan

Abstract

Thrombotic thrombocytopenia purpura (TTP) caused by a deficiency in ADAMTS-13 activity is considered to involve a subset of thrombotic microangiopathy (TMA). Although concept of TTP is included under the umbrella of TMA, discrimination of TTP from TMA is occasionally difficult in an autoimmune disorder. Herein, we report a case with TTP associated with systemic lupus erythematosus (SLE). In this case, it was difficult to discriminate TTP from TMA and the measurement of ADAMTS-13 activity was useful for obtaining an accurate diagnosis. SLE patients having thrombocytopenia in complication with anemia should be considered a monitoring of ADAMTS-13 activity even though the patients lacked symptoms of TTP related to the microvascular coagulation.

Publisher

Hindawi Limited

Subject

Immunology and Microbiology (miscellaneous),Immunology,Immunology and Allergy

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