The Diagnostic Challenge of Acquired Thrombotic Thrombocytopenic Purpura in Children: Case Report and Review of the Literature

被引：3
作者：
Schapkaitz, Elise [1 ,3 ]
Schickerling, Tanya Marie [2 ,3 ]
机构：
[1] Charlotte Maxeke Johannesburg Acad Hosp, Dept Hematol & Mol Med, Johannesburg, South Africa
[2] Charlotte Maxeke Johannesburg Acad Hosp, Dept Pediat & Child Hlth, Div Pediat Hematol & Oncol, Johannesburg, South Africa
[3] Univ Witwatersrand, Fac Hlth Sci, Johannesburg, South Africa
来源：
LABORATORY MEDICINE | 2018年 / 49卷 / 03期
关键词：
thrombotic thrombocytopenic purpura; HIV; plasma exchange; children; ADAMTS-13; HAART; HEMOLYTIC-UREMIC SYNDROME; ACTIVE ANTIRETROVIRAL THERAPY; PLASMA-EXCHANGE; ADAMTS13; ACTIVITY; MICROANGIOPATHY; INFECTION; INFUSION; FEATURES;
D O I：
10.1093/labmed/lmy015
中图分类号：
R446 [实验室诊断]; R-33 [实验医学、医学实验];
学科分类号：
1001 ;
摘要：
Acquired thrombotic thrombocytopenic purpura (TTP) is a rare disease in children. Despite advances in diagnosis and treatment, acquired TTP remains a challenging disease due to the lack of a simple diagnostic test and the variable response to plasma exchange. Herein, we describe a case of a 5-year-old of black ethnicity boy who presented with a sudden onset of fatigue and body aches. Laboratory investigations revealed a thrombotic microangiopathic manifestation. TTP was diagnosed, and plasma exchange and corticosteroids were initiated, with an excellent response. Subsequently, reduced disintegrin and metalloprotease with thrombospodin-2-like repeats (ADAMTS-13) activity and human immunodeficiency virus (HIV) were confirmed. Antiretroviral treatment was started as long-term management. At last follow-up, he continues in stable remission.
引用
收藏
页码：268 / 271
页数：4
相关论文
共 16 条
[1]   THROMBOTIC THROMBOCYTOPENIC PURPURA - REPORT OF 16 CASES AND REVIEW OF LITERATURE [J].
AMOROSI, EL ;
ULTMANN, JE .
MEDICINE, 1966, 45 (02) :139-+
[2]   Frequency and Significance of HIV Infection among Patients Diagnosed with Thrombotic Thrombocytopenic Purpura [J].
Benjamin, Melody ;
Terrell, Deirdra R. ;
Vesely, Sara K. ;
Voskuhl, Gene W. ;
Dezube, Bruce J. ;
Hovinga, Johanna A. Kremer ;
Laemmle, Bernhard ;
George, James N. .
CLINICAL INFECTIOUS DISEASES, 2009, 48 (08) :1129-1137
[3]   Treatment of thrombotic thrombocytopenic purpura beyond therapeutic plasma exchange [J].
Coppo, Paul ;
Froissart, Antoine .
HEMATOLOGY-AMERICAN SOCIETY OF HEMATOLOGY EDUCATION PROGRAM, 2015, :637-643
[4]   Lessons learned from the Oklahoma thrombotic thrombocytopenic purpura-hemolytic uremic syndrome registry [J].
George, James N. ;
Terrell, Deirdra R. ;
Swisher, Karen K. ;
Vesely, Sara K. .
JOURNAL OF CLINICAL APHERESIS, 2008, 23 (04) :129-137
[5]   Thrombotic microangiopathy in patients with acquired immunodeficiency syndrome before and during the era of introduction of highly active antiretroviral therapy [J].
Gervasoni, C ;
Ridolfo, AL ;
Vaccarezza, M ;
Parravicini, C ;
Vago, L ;
Adorni, F ;
Cappelletti, A ;
Monforte, AD ;
Galli, M .
CLINICAL INFECTIOUS DISEASES, 2002, 35 (12) :1534-1540
[6]   ADAMTS13 activity and the presence of acquired inhibitors in human immunodeficiency virus-related thrombotic thrombocytopenic purpura [J].
Gunther, Karen ;
Garizio, Dominique ;
Nesara, Paul .
TRANSFUSION, 2007, 47 (09) :1710-1716
[7]   Human immunodeficiency virus associated thrombotic thrombocytopenic purpura - favourable outcome with plasma exchange and prompt initiation of highly active antiretroviral therapy [J].
Hart, Daniel ;
Sayer, Ruth ;
Miller, Robert ;
Edwards, Simon ;
Kelly, Anne ;
Baglin, Trevor ;
Hunt, Beverley ;
Benjamin, Sylvia ;
Patel, Raj ;
Machin, Samuel ;
Scully, Marie .
BRITISH JOURNAL OF HAEMATOLOGY, 2011, 153 (04) :515-519
[8]  
Hovinga JAK, 2007, J THROMB HAEMOST, V5, P2284
[9]  
Moake JL, 2002, ARCH PATHOL LAB MED, V126, P1430
[10]   Thrombotic thrombocytopenic purpura in patients with retroviral infection is highly responsive to plasma infusion therapy [J].
Novitzky, N ;
Thomson, J ;
Abrahams, L ;
du Toit, C ;
McDonald, A .
BRITISH JOURNAL OF HAEMATOLOGY, 2005, 128 (03) :373-379
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