Relapsed/refractory thrombotic thrombocytopenic purpura treated with N-acetylcysteine: a case report

被引：4

作者：

Demircioglu, Sinan ^{[1
]}

Ekinci, Omer ^{[1
]}

Dogan, Ali ^{[1
]}

Demir, Cengiz ^{[1
]}

机构：

[1] Van Yuzuncu Yil Univ, Dept Hematol, TR-65040 Van, Turkey

来源：

SCOTTISH MEDICAL JOURNAL | 2018年 / 63卷 / 04期

关键词：

Thrombotic thrombocytopenic purpura; relapsed; refractory; N-acetylcysteine; MANAGEMENT; DIAGNOSIS;

D O I：

10.1177/0036933018775240

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Thrombotic thrombocytopenic purpura is a rare condition that presents with microangiopathic haemolytic anaemia, thrombocytopaenia, fever, renal impairment and neurological symptoms. Plasma exchange is a lifesaving treatment for this condition. However, some cases may be non-responsive to plasma exchange, or loss of response may occur. Treatment options for refractory cases include high-dose corticosteroids, rituximab, vincristine, cyclophosphamide, splenectomy, bortezomib and N-acetylcysteine. We present a refractory case of thrombotic thrombocytopenic purpura responding to the last of these therapies.

引用

页码：122 / 124

页数：3

共 14 条

[1]

Acedillo RR, 2016, BMJ CASE REP, V2016, P1

[2] N-acetylcysteine reduces the size and activity of von Willebrand factor in human plasma and mice [J].

Chen, Junmei ;

Reheman, Adili ;

Gushiken, Francisca C. ;

Nolasco, Leticia ;

Fu, Xiaoyun ;

Moake, Joel L. ;

Ni, Heyu ;

Lopez, Jose A. .

JOURNAL OF CLINICAL INVESTIGATION, 2011, 121 (02) :593-603

[3] N-Acetylcysteine: an old drug, a new insight, a potentially effective treatment for thrombotic thrombocytopenic purpura [J].

George, James N. ;

Lopez, Jose A. ;

Konkle, Barbara A. .

TRANSFUSION, 2014, 54 (05) :1205-1207

[4] Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura [J].

Levy, GG ;

Nichols, WC ;

Lian, EC ;

Foroud, T ;

McClintick, JN ;

McGee, BM ;

Yang, AY ;

Siemieniak, DR ;

Stark, KR ;

Gruppo, R ;

Sarode, R ;

Shurin, SB ;

Chandrasekaran, V ;

Stabler, SP ;

Sabio, H ;

Bouhassira, EE ;

Upshaw, JD ;

Ginsburg, D ;

Tsai, HM .

NATURE, 2001, 413 (6855) :488-494

[5] Treatment of refractory thrombotic thrombocytopenic purpura with N-acetylcysteine: a case report [J].

Li, Gloria W. ;

Rambally, Siayareh ;

Kamboj, Jasmine ;

Reilly, Sean ;

Moake, Joel L. ;

Udden, Mark M. ;

Mims, Martha P. .

TRANSFUSION, 2014, 54 (05) :1221-1224

[6] The role of rituximab in the management of patients with acquired thrombotic thrombocytopenic purpura [J].

Lim, Wendy ;

Vesely, Sara K. ;

George, James N. .

BLOOD, 2015, 125 (10) :1526-1531

[7]

Roberts A W, 1991, J Clin Apher, V6, P150, DOI 10.1002/jca.2920060305

[8] The role of N-acetylcysteine in the treatment of thrombotic thrombocytopenic purpura [J].

Rottenstreich, Amihai ;

Hochberg-Klein, Sarit ;

Rund, Deborah ;

Kalish, Yosef .

JOURNAL OF THROMBOSIS AND THROMBOLYSIS, 2016, 41 (04) :678-683

[9] ADAMTS-13 in the Diagnosis and Management of Thrombotic Microangiopathies [J].

Sarig, Galit .

RAMBAM MAIMONIDES MEDICAL JOURNAL, 2014, 5 (04)

[10] Thrombotic Thrombocytopenic Purpura: 2012 American Society for Apheresis (ASFA) Consensus Conference on Classification, Diagnosis, Management, and Future Research [J].

Sarode, Ravi ;

Bandarenko, Nick ;

Brecher, Mark E. ;

Kiss, Joseph E. ;

Marques, Marisa B. ;

Szczepiorkowski, Zbigniew M. ;

Winters, Jeffrey L. .

JOURNAL OF CLINICAL APHERESIS, 2014, 29 (03) :148-167

← 1 2 →