Moschcowitz Syndrome or Thrombotic Thrombocytopenic Purpura and Antiphospholipid Antibody Syndrome as a Rare Cause of Thrombocytopenia in Pregnancy Mimicking Hemolysis, Elevated Liver Enzymes, and Low Platelets Syndrome in a Patient with Bad Obstetric History: A Diagnostic Dilemma

Abstract

Thrombotic microangiopathy (TMA) is a group of disorders characterized by microangiopathic hemolytic anemia, moderate to severe thrombocytopenia, and end-organ damage. In pregnancy, TMA is most commonly due to preeclampsia and hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome. Very rarely it occurs due to Moschowitz syndrome also known as thrombotic thrombocytopenic purpura (TTP), antiphospholipid antibody (APLA) syndrome, or atypical hemolytic uremic syndrome (aHUS). All of these share common clinical features; yet their etiologies are different. Although both HELLP and TTP share common features of endothelial injury and microvascular thrombi, yet, they have different courses and line of management. In term pregnancy, differentiating between these two pathologies can be extremely difficult due to the extreme overlap in clinical and laboratory manifestations, and this becomes possible only with the use of specific markers as ADAMTS-13, whenever available. Unfortunately, delay in timely diagnosis and treatment can be life-threatening. We describe case of 25-year-old pregnant woman who came with HELLP syndrome which did not resolve postpartum only to improve after plasmapheresis.

Molecular Advances in Preeclampsia and HELLP Syndrome

“…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 ]. Although delivery is not mandatory in TTP, it is required when TTP is combined with preeclampsia, even at a gestational age before 34 weeks [ 47 ].…”

“…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 ].…”

“…Those results can be evaluated at a second time and are likely to contribute to the confirmation of the diagnosis or the differential diagnosis between the hereditary and the acquired form of the disease [ 42 ]. Identifying the specific form of the disease can help us decide which is the optimal therapeutic option in each case—corticosteroids or rituximab in the acquired form and plasma administration or plasmapheresis in the hereditary [ 47 ]. Corticosteroids should also be combined with plasmapheresis in the beginning, until the acquired form of the disease is excluded with the negativity of anti-ADAMTS13 autoantibodies [ 45 ].…”