HELLP Syndrome, Thrombotic Thrombocytopenic Purpura or Both: Appraising the Complex Association and Proposing a Stepwise Practical Plan for Differential Diagnosis

Abstract

Both thrombocytopenia and microangiopathic hemolytic anemia (TMA) are seen in thrombotic thrombocytopenic purpura (TTP) and HELLP syndrome among other disorders during pregnancy. Although both share backgrounds of endothelial injury and microvascular thrombi and some clinical features, yet, they have different etiologies and courses. In late pregnancy, differentiating between these two pathologies can be extremely difficult due to the immense overlap in clinical and laboratory manifestations and this becomes only possible with the use of specific markers as ADAMTS-13, when available. Hereby, we describe three cases that may exemplify the complex association between PE/HELLP syndrome and TTP. The first case presented with PE/HELLP syndrome and deteriorated postpartum to improve on plasmapheresis. The second case was a known TTP patient who developed superimposed PE/HELLP at 27 weeks gestation which necessitated emergent delivery. The third was a case of preeclampsia that progressed to HELLP syndrome on day 2 postpartum but 3 days later was complicated by TTP. HELLP syndrome and TTP can co-exist, but can also complicate one another. In the absence of instantaneous results of ADAMTS-13 and when diagnosis with clinical judgement alone cannot be done with certainty, a short trial-plasmapheresis could be attempted with close observation of the immediate response. This stepwise approach might prove to be a valuable tool when integrated in the usual workup of clinical and laboratory evaluation both before and after delivery.

Molecular Advances in Preeclampsia and HELLP Syndrome

“…Moreover, HELLP syndrome or preeclampsia should also be considered in pregnant women with a personal history of hereditary or acquired TTP. It should also be noted that the coexistence of TTP and PE/HELLP syndrome should not be excluded from the beginning [ 42 , 43 , 44 , 45 , 46 ]. Otherwise, PE/HELLP are thought to be more frequent in patients who have already recovered from TTP, or even induce the development of TTP in predisposed pregnant women [ 42 , 45 , 46 ].…”

“…Table 4 presents data from several case reports, collected from our literature research process, indicating the difficulties in setting the definite diagnosis. It should be noted that there are many cases in which the final diagnosis remains vague, or the authors attribute the unsuccessful initial management to a possible coexistence of HELLP/PE and TTP from the beginning [ 43 , 44 , 45 , 46 , 47 , 48 ].…”

“…In case there is no resolution or even worse, there is a deterioration of the clinical manifestations or the laboratory parameters (persistent anemia, neurological symptoms, further thrombocytopenia) in the first days postpartum, TTP is the most probable diagnosis and plasmapheresis should be performed without any further delay. In the literature, there are several case reports in which TTP was diagnosed postpartum because of deteriorating clinical and laboratory parameters after delivery [ 42 , 43 , 44 , 45 , 46 , 47 , 48 ].…”

Pregnancy onset congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) mimicking HELLP syndrome: a case report

“…[4][5][6] When first TTP boot occurs in pregnancy, it is not always possible considered in differential diagnosis as in our case and similarly other cases. [7,8] However, pregnancy is a condition in 17 percent of women of childbearing age with TTP, and most of these women experience their first TTP episode during the second trimester of pregnancy or postpartum period. [2,4,9] HELLP syndrome is complicating pregnancies, approximately 200 times more than TTP.…”

Características clínicas y fisiológicas del síndrome de Hellp

“…Una de las teorías más aceptadas para la presentación de complicaciones hipertensivas en el embarazo, es la implantación insuficiente de las células del citotrofoblasto, que se infiltran en la porción decidual de las arterias espirales, pero no penetran en su segmento miometrial (14,15). Las arterias espirales, por lo tanto, no se transforman en canales vasculares de gran capacitancia, sino que se mantienen estrechas, lo que resulta en una disminución en el flujo placentario (16)(17)(18). y da como resultado una alta velocidad de perfusión en el espacio intervelloso, lo que genera estrés de cizallamiento en el trofoblasto (19,20).…”