Microangiopathies in pregnancy

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a potentially reversible, life-threatening medical emergency. We present a case of a 21-year-old female with evidence of haemolytic anaemia based on the presence of positive markers of haemolysis. Negative Coomb's test, thrombocytopenia and placental infarcts raised suspicion for a thrombotic microangiopathy. She was diagnosed with TTP and managed with emergency plasma exchange. Her recovery was immediate.A presumptive diagnosis of TTP should be based on the presence of microangiopathic haemolytic anaemia with thrombocytopenia and plasma exchange should be initiated while complete work up is pending. Using the regular pentad solely for diagnosis of TTP will lead to underdiagnosis of many cases and should be avoided.Several microangiopathies can be seen during pregnancy including TTP/atypical haemolytic uraemic syndrome, HELLP syndrome, pre-eclampsia, disseminated intravascular coagulopathy and antiphospholipid antibody syndrome. Distinction between each type will be the focus of our discussion as treatment decisions differ accordingly.

Atypical hemolytic uremic syndrome: when pregnancy leads to lifelong dialysis: a case report and literature review

“…HELLP and preeclampsia in pregnancy are also common microangiopathies [ 33 ]. HELLP is a syndrome of MAHA, elevated liver enzymes and low platelet counts during the third trimester of pregnancy [ 33 , 34 ].…”

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

“…It is responsible for the cleavage of the unusually large von Willebrand factor (vWF) multimers. 4 Decreased activity of ADAMTS13 leads to persistence of those multimers in the blood, which in turn leads to platelet aggregation and formation of platelet thrombi and hence thrombocytopenia from platelet consumption. Hemolytic anemia and schistocytes result from shearing of RBCs, as they pass through the microthrombi.…”

“…In certain conditions, where there is overlap of clinical manifestations between different microangiopathies ,a decision could be made to proceed with plasma exchange based on high index of suspicion. 4 Plasma exchange serves two purposes, first removal of the plasma along with all the preformed microthrombi and autoantibodies and second is repletion of the deficient ADAMTS13 activity by administration of plasma rich in ADAMTS13. Hence, it is superior over plasma infusion which can serve only the latter purpose and is accompanied by a risk of volume overload.…”

Atypical Hemolytic Uremic Syndrome During Pregnancy: Short Review and Case Report with a Favorable Outcome

“…There are several variants of TMA that complicate the course of pregnancy: pregnancy-associated hypertension, preeclampsia (PE), DIC and HELLP syndrome, thrombotic thrombocytopenic purpura (TTP), catastrophic antiphospholipid syndrome (CAS), sepsis and, as the main manifestation of TMA, -atypical hemolytic uremic syndrome (aHUS) [1,2]. Life-threatening complications of TMA include acute renal failure (ARF), brain injury (coma), gastrointestinal tract injury (hemorrhage), and heart failure (acute heart failure).…”