HIV-related thrombotic thrombocytopenic purpura: report of 2 cases and a review of the literature

A M de Man, Y M Smulders, K J Roozendaal, P H Frissen

Research output: Contribution to journalReview articleAcademicpeer-review

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a syndrome characterised by the clinical pentad of microangiopathic haemolytic anaemia (MAHA), thrombocytopenia, renal failure, fluctuating neurologic signs, and fever. The aetiology of TTP is unknown, but associations with various underlying diseases, infections and drugs have been identified. One of these associations is with HIV infection. We describe the clinical picture, the laboratory results and the response to plasma therapy of two cases of HIV-associated TTP. In both patients, a longitudinal semiquantitative assessment of the numbers of schistocytes in blood was made, which correlated well with the more traditional parameters of disease activity. Since 1987, at least 49 patients with HIV-associated TTP have been reported. A case-analysis of the 38 patients who were described in sufficient detail and a review of the literature in the setting of HIV infection is presented. The most important conclusions from these combined data are: (1) TTP usually seems to occur in patients with a CD4+ lymphocyte count < 250 x 10(6).l(-1); (2) more than 50% of the patients present with TTP soon after or during an infectious or malignant disease; (3) plasma exchange is the therapy of choice, still resulting in mortality of 22%; (4) higher initial platelet count and creatinine level are correlated with an adverse outcome.

LanguageEnglish
Pages103-9
Number of pages7
JournalNetherlands Journal of Medicine
Volume51
Issue number3
Publication statusPublished - Sep 1997

Cite this

@article{e91a3a39daa94103970c12f95f550ae7,
title = "HIV-related thrombotic thrombocytopenic purpura: report of 2 cases and a review of the literature",
abstract = "Thrombotic thrombocytopenic purpura (TTP) is a syndrome characterised by the clinical pentad of microangiopathic haemolytic anaemia (MAHA), thrombocytopenia, renal failure, fluctuating neurologic signs, and fever. The aetiology of TTP is unknown, but associations with various underlying diseases, infections and drugs have been identified. One of these associations is with HIV infection. We describe the clinical picture, the laboratory results and the response to plasma therapy of two cases of HIV-associated TTP. In both patients, a longitudinal semiquantitative assessment of the numbers of schistocytes in blood was made, which correlated well with the more traditional parameters of disease activity. Since 1987, at least 49 patients with HIV-associated TTP have been reported. A case-analysis of the 38 patients who were described in sufficient detail and a review of the literature in the setting of HIV infection is presented. The most important conclusions from these combined data are: (1) TTP usually seems to occur in patients with a CD4+ lymphocyte count < 250 x 10(6).l(-1); (2) more than 50{\%} of the patients present with TTP soon after or during an infectious or malignant disease; (3) plasma exchange is the therapy of choice, still resulting in mortality of 22{\%}; (4) higher initial platelet count and creatinine level are correlated with an adverse outcome.",
keywords = "Adult, HIV Infections/complications, HIV-1, Humans, Male, Plasma Exchange, Purpura, Thrombotic Thrombocytopenic/diagnosis",
author = "{de Man}, {A M} and Smulders, {Y M} and Roozendaal, {K J} and Frissen, {P H}",
year = "1997",
month = "9",
language = "English",
volume = "51",
pages = "103--9",
journal = "Netherlands Journal of Medicine",
issn = "0300-2977",
publisher = "Van Zuiden Communications BV",
number = "3",

}

HIV-related thrombotic thrombocytopenic purpura : report of 2 cases and a review of the literature. / de Man, A M; Smulders, Y M; Roozendaal, K J; Frissen, P H.

In: Netherlands Journal of Medicine, Vol. 51, No. 3, 09.1997, p. 103-9.

Research output: Contribution to journalReview articleAcademicpeer-review

TY - JOUR

T1 - HIV-related thrombotic thrombocytopenic purpura

T2 - Netherlands Journal of Medicine

AU - de Man, A M

AU - Smulders, Y M

AU - Roozendaal, K J

AU - Frissen, P H

PY - 1997/9

Y1 - 1997/9

N2 - Thrombotic thrombocytopenic purpura (TTP) is a syndrome characterised by the clinical pentad of microangiopathic haemolytic anaemia (MAHA), thrombocytopenia, renal failure, fluctuating neurologic signs, and fever. The aetiology of TTP is unknown, but associations with various underlying diseases, infections and drugs have been identified. One of these associations is with HIV infection. We describe the clinical picture, the laboratory results and the response to plasma therapy of two cases of HIV-associated TTP. In both patients, a longitudinal semiquantitative assessment of the numbers of schistocytes in blood was made, which correlated well with the more traditional parameters of disease activity. Since 1987, at least 49 patients with HIV-associated TTP have been reported. A case-analysis of the 38 patients who were described in sufficient detail and a review of the literature in the setting of HIV infection is presented. The most important conclusions from these combined data are: (1) TTP usually seems to occur in patients with a CD4+ lymphocyte count < 250 x 10(6).l(-1); (2) more than 50% of the patients present with TTP soon after or during an infectious or malignant disease; (3) plasma exchange is the therapy of choice, still resulting in mortality of 22%; (4) higher initial platelet count and creatinine level are correlated with an adverse outcome.

AB - Thrombotic thrombocytopenic purpura (TTP) is a syndrome characterised by the clinical pentad of microangiopathic haemolytic anaemia (MAHA), thrombocytopenia, renal failure, fluctuating neurologic signs, and fever. The aetiology of TTP is unknown, but associations with various underlying diseases, infections and drugs have been identified. One of these associations is with HIV infection. We describe the clinical picture, the laboratory results and the response to plasma therapy of two cases of HIV-associated TTP. In both patients, a longitudinal semiquantitative assessment of the numbers of schistocytes in blood was made, which correlated well with the more traditional parameters of disease activity. Since 1987, at least 49 patients with HIV-associated TTP have been reported. A case-analysis of the 38 patients who were described in sufficient detail and a review of the literature in the setting of HIV infection is presented. The most important conclusions from these combined data are: (1) TTP usually seems to occur in patients with a CD4+ lymphocyte count < 250 x 10(6).l(-1); (2) more than 50% of the patients present with TTP soon after or during an infectious or malignant disease; (3) plasma exchange is the therapy of choice, still resulting in mortality of 22%; (4) higher initial platelet count and creatinine level are correlated with an adverse outcome.

KW - Adult

KW - HIV Infections/complications

KW - HIV-1

KW - Humans

KW - Male

KW - Plasma Exchange

KW - Purpura, Thrombotic Thrombocytopenic/diagnosis

M3 - Review article

VL - 51

SP - 103

EP - 109

JO - Netherlands Journal of Medicine

JF - Netherlands Journal of Medicine

SN - 0300-2977

IS - 3

ER -