Evaluation of women with clinically suspected thrombotic thrombocytopenic purpura‐hemolytic uremic syndrome during pregnancy

Abstract

Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is more common in women, and commonly occurs during pregnancy and the immediate postpartum period. An important clinical issue is the distinction of TTP-HUS from the more common obstetric complications, preeclampsia and HELLP syndrome (hemolysis, elevated liver function tests, low platelets). Clinical suspicion of TTP-HUS requires urgent intervention with plasma exchange treatment, a procedure with substantial risk, while preeclampsia and HELLP syndrome typically resolve spontaneously following delivery. Since clinical features of these syndromes can be similar, especially if preeclampsia becomes severe or if seizures (defining eclampsia) occur, the differential diagnosis may be arbitrary. This review addresses the evaluation and management of these syndromes and describes a clinical approach for determining when plasma exchange is appropriate.

Thrombotic thrombocytopenic purpura associated with a prolactin‐producing pituitary adenoma

“…Several studies show that 10-31% of TTP occurs in pregnant women [3,4], suggesting that pregnancy may have a triggering effect in the pathogenesis of TTP. Pregnancy may sensitize the endothelial cells to cytotoxic agents, or it may interfere with the release and/or degradation of von Willebrand factor multimers.…”

“…The disease occurs more frequently in younger women. In 10-31% of cases it is associated with pregnancy [3,4] and is characterized by intraluminal platelet aggregation in the microcirculation causing vascular occlusion, arrest of blood flow, hypoxia, and tissue damage [4,10].…”

Thrombotic thrombocytopenic purpura in the first trimester and successful pregnancy

“…Few cases of TTP (10%) occur during the first trimester [1,2], the majority occurring at the time of delivery or during post-partum period [4,6]. Until the effectiveness of plasma infusion and plasma exchange was recognized, TTP was associated with a mortality rate of 95% and, in case of pregnancy-related TTP, the maternal survival was rare and the fetal mortality rate approached 80% [9].…”

“…Plasma exchange should be continued for several days after normalization of platelets count and resolution of hemolysis is documented [3][4][5]9]. It has now been demonstrated that pregnancy does not impair the response to plasmapheresis and that plasmapheresis does not impair the outcome of pregnancy [3,4].…”

“…There is considerable evidence that TTP is an immune-mediated disease, due to the presence of both anti-platelet and antiendothelial cell antibodies and inhibitors to the ADAMTS-13 [10]. Therefore, it is appropriate to include intravenous immunoglobulin G therapy and glucocorticoids, as prednisone and methylprednisolone, because they do not cross the placenta [3,4] in the management of TTP [10,11]. An issue specific for young women is the risk for future pregnancies.…”