How I treat thrombotic thrombocytopenic purpura in pregnancy

Abstract

Thrombotic thrombocytopenic purpura (TTP) is an acute, life-threatening thrombotic microangiopathy (TMA) caused by acquired or congenital severe deficiency of ADAMTS13. Pregnancy is a recognized risk factor for precipitating acute (first or recurrent) episodes of TTP. Differential diagnosis with other TMAs is particularly difficult when the first TTP event occurs during pregnancy: a high index of suspicion and prompt recognition of TTP are essential for achieving a good maternal and fetal outcome. An accurate distinction between congenital and acquired cases of pregnancy-related TTP is mandatory for safe subsequent pregnancy planning. In this article, we summarize the current knowledge on pregnancy-associated TTP, and describe how we manage TTP during pregnancy in our clinical practice.

A Race against the Clock: A Case Report and Literature Review Concerning the Importance of ADAMTS13 Testing in Diagnosis and Management of Thrombotic Thrombocytopenic Purpura during Pregnancy

“…Including our patient in the National TTP Program was essential, as she was the first patient who benefitted from the initiation of the caplacizumab treatment in Romania. Maternal–fetal complications (IUGR, premature birth, fetal death, maternal stroke) have been common and can be difficult for the patient [ 14 , 27 ], and the relapse rate is 100% [ 8 , 11 , 18 , 23 , 24 ]. It has been postulated that proteins found in the placental circulation may serve as maternal triggers for the development of anti-ADAMTS13 autoantibodies and may be associated with pregnancy complications [ 13 , 14 , 26 , 27 ].…”

“…Maternal–fetal complications (IUGR, premature birth, fetal death, maternal stroke) have been common and can be difficult for the patient [ 14 , 27 ], and the relapse rate is 100% [ 8 , 11 , 18 , 23 , 24 ]. It has been postulated that proteins found in the placental circulation may serve as maternal triggers for the development of anti-ADAMTS13 autoantibodies and may be associated with pregnancy complications [ 13 , 14 , 26 , 27 ]. Histopathological analysis of the placenta can provide valuable data on how the vascular thrombosis and the characteristic microangiopathy of TTP occurs.…”

“…Until her transfer, the only symptoms of the patient were mild fatigue and blurred vision, but no bleeding, neurological symptoms, or renal manifestations, which, once again, emphasized the heterogeneity of this disease that makes it difficult for a clinician to diagnose. In the literature, the diagnosis of TTP is still based on the clinical picture and laboratory tests, and the treatment should be initiated as quickly as possible before determining ADAMTS13, as TTP underdiagnosed and undertreated can quickly turn lethal [ 11 , 13 , 14 ]. The patient’s relatives were instructed to request a specific, semi-quantitative ADAMTS13 analysis, which was subsequently performed, for which we would like to thank the representative of the National Program for Rare Genetic Diseases, who supported our efforts for a definite diagnosis.…”

“…As a result, the ischemic lesions that occur can affect any organ, including the placenta [ 11 ]. Acquired TTP with autoantibodies and severe ADAMTS13 deficiency (<10%) is the most common form of this disease [ 12 , 13 , 14 ].…”

TTP: From empiricism for an enigmatic disease to targeted molecular therapies

“…Patients who experience a severe drop in ADAMTS13 activity during pregnancy require pre‐emptive measures including PEX and steroids. Rituximab is ideally reserved for the post‐partum period owing to lack of safety evidence 113 …”