Thrombotic thrombocytopenic purpura and acquired immunodeficiency syndrome diagnosed in pregnancy: Case report

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a medical emergency that demands prompt diagnosis to allow lifesaving treatment: plasmapheresis. TTP during pregnancy is rare, with estimated prevalence of 1/200 000, and even rarer in association with acquired immunodeficiency syndrome, with only two cases reported. Differential diagnosis includes HELLP syndrome (hemolysis elevated liver enzymes and low platelets), sepsis, intravascular‐disseminated coagulation, and acquired autoimmune diseases, each one with its unique treatment and prognosis. A case of a pregnant woman at 26th week with sudden onset of left hand paresthesia and purpura is reported. PLASMIC score showed high risk for ADAMTS‐13 deficiency and diagnosis of TTP was made. Human immunodeficiency virus screening test was positive on admission. Plasmapheresis and antiretroviral therapy were initiated and the delivery of a healthy newborn at full‐term gestation was achieved, unlike other cases in literature. No obstetric complications were observed and the follow‐up shows no signs of disease recurrence.

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

“…For a correct differential diagnosis, we divided the patient's tests as follows (Tables 1 and 2): During this time, a transfer to a hospital with appropriate facilities was being sought, especially as the recommended plasmapheresis could not be performed without hematological supervision. Even though PLT administration has caused controversy due to the microthrombi it can generate [9,26], Swisher showed it to be useful in very low hemoglobin levels and severe thrombocytopenia, due to high risks of bleeding [26]. On the 22 April 2021, we obtained the patient's transfer to the intensive care unit of Hematology Sibiu, a hospital accredited for the treatment of this pathology through the Acquired Thrombocytopenic Thrombotic Purpura Program, part of the National Program of Rare Genetic Diseases.…”

“…During this time, a transfer to a hospital with appropriate facilities was being sought, especially as the recommended plasmapheresis could not be performed without hematological supervision. Even though PLT administration has caused controversy due to the microthrombi it can generate [ 9 , 26 ], Swisher showed it to be useful in very low hemoglobin levels and severe thrombocytopenia, due to high risks of bleeding [ 26 ].…”

“…In terms of manifestations of the disease, it starts as an acute microangiopathy (TMA), characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and vascular microthrombi, caused by a severe deficiency of the von Willebrand factor cleavage protease ADAMTS13, a critically important enzyme that is synthesized in hepatic stellate cells [ 6 , 8 , 9 , 10 ].…”