microangiopathic hemolytic anemia

Diagnosis and Treatment of Thrombotic Thrombocytopenic Purpura Developed During Gestation: Literature Review and Case Report

RARE HEMATOLOGICAL TUMORS AND SYNDROMES , , , , , , ,

V.V. Voitsekhovskii1, L.B. Filatov2, A.V. Pivnik3, P.V. Avdonin4, T.V. Esenina5, A.G. Sudakov5

1 Amur State Medical Academy, 95 Gor’kogo str., Blagoveshchensk, Amur Oblast, Russian Federation, 675000

2 Yekaterinburg Consultative and Diagnostic Centre, 5 Suvorovskii per., Yekaterinburg, Sverdlov Oblast, Russian Federation, 620039

3 D.D. Pletnev Moscow Clinical Scientific and Practical Center under the Department of Healthcare, 86 sh. Entuziastov, Moscow, Russian Federation, 111123

4 N.K. Kol’tsov Institute of Developmental Biology, 26 Vavilova str., Moscow, Russian Federation, 119334

5 Amur Regional Clinical Hospital, 26 Voronkova str., Blagoveshchensk, Amur Oblast, Russian Federation, 675028

For correspondence: V.V. Voitsekhovskii, DSci, Associate Professor, 95 Gor’kogo str., Blagoveshchensk, Amur Oblast, Russian Federation, 675000; Tel: +7(4162)42-94-97; e-mail: voitsehovsckij@yandex.ru

For citation: Voitsekhovskii V.V., Filatov L.B., Pivnik A.V., Avdonin P.V., Esenina T.V., Sudakov A.G. Diagnosis and Treatment of Thrombotic Thrombocytopenic Purpura Developed During Gestation: Literature Review and Case Report. Klin. Onkogematol. 2014; 7(4): 587–598 (In Russ.).

ABSTRACT

This paper presents a literature review on state of art in issues of etiology, pathogenesis, clinical features and diagnosis of thrombotic thrombocytopenic purpura (ТТP or Moschcowitz’s disease). Data on a rare pathology of congenital ТТP (Upshaw—Schulman syndrome) and its manifestation during pregnancy are summarized. Various aspects of differential diagnosis for microangiopathic hemolytic anemia (МАHA), complicated issues in differential diagnosis for thrombotic microangiopathies (ТТP, hemolytic uremic syndrome, HELLP syndrome) during pregnancy are discussed. Specificity of clinical manifestations and differential diagnosis for acquired and congenital TTP in pregnant patients are considered. Issues of TTP treatment during pregnancy are exposed in detail.

Keywords: thrombotic thrombocytopenic purpura, congenital thrombotic thrombocytopenic purpura, Upshaw-Schulman syndrome, thrombotic microangiopathy, microangiopathic hemolytic anemia, ADAMTS-13, HELLP syndrome, pregnancy.

Accepted: October 3, 2014

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