Early diagnosis, tailored care key to managing TTP in pregnancy
Case series from Qatar details experiences of three women
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Timely diagnosis and treatment of thrombotic thrombocytopenic purpura (TTP) during pregnancy — along with coordinated multidisciplinary care — can lead to favorable outcomes for both mother and baby, according to a case series detailing the experiences of three pregnant women with the disease.
Diagnosis was not straightforward in all cases, due to overlapping symptoms with other pregnancy-related conditions. However, the three women were successfully managed through prompt recognition, tailored care, and close collaboration between hematologists and obstetricians, resulting in maternal recovery and successful deliveries despite severe complications in some.
“These cases underscore the importance of early diagnosis, individualized treatment, multidisciplinary care, and proactive management to optimize maternal and neonatal outcomes,” the researchers wrote.
The study, “Thrombotic thrombocytopenic purpura in pregnancy: Lessons from a case series of three patients,” was published in the Journal of International Medical Research.
TTP is a blood disorder marked by a deficiency of ADAMTS13, an enzyme that helps regulate blood clotting. When the enzyme is missing or isn’t working properly, blood clots form in small blood vessels. As a result, platelets are used up, and red blood cells are destroyed as they pass through narrowed vessels, leading to low platelet levels (thrombocytopenia) and low red blood cell levels (anemia).
TTP triggered by pregnancy
Pregnancy is a well-established trigger of TTP, occurring in about one in 25,000 pregnancies and usually appearing in the third trimester. Therapeutic plasma exchange (TPE), the mainstay treatment for TTP, has dramatically reduced maternal mortality. In TPE, a patient’s plasma (the liquid part of blood) is replaced with donor plasma to remove harmful antibodies and help restore ADAMTS13 activity.
In severe or recurrent cases, immunosuppressive therapies such as corticosteroids and rituximab may be added. Cablivi (caplacizumab-yhdp), when combined with TPE and immunosuppressive treatments, can also provide rapid disease control. Early diagnosis and treatment are therefore essential to prevent complications for both the mother and fetus.
Researchers in Qatar described the clinical features and treatment approaches of three women with pregnancy-related TTP, highlighting how early diagnosis and coordinated care helped achieve favorable outcomes.
The first two women had inherited TTP, which is caused by mutations in the gene responsible for making ADAMTS13, leading to little or no functional enzyme. The third had acquired TTP, in which the immune system produces antibodies that block the enzyme’s activity.
Lab findings in all three women showed severe thrombocytopenia along with signs of hemolytic anemia — specifically microangiopathic hemolytic anemia (MAHA), in which red blood cells are destroyed as they pass through small blood vessels blocked by clots. Although the diagnosis was not immediately clear in all cases, these findings ultimately raised suspicion of TTP and prompted treatment initiation.
In one case, a 34-year-old woman at 33 weeks of pregnancy, had a headache, dysuria (pain or a burning sensation when urinating), dark-colored urine, and lower-leg swelling. Doctors suspected TTP early based on lab findings, and prescribed standard treatment with TPE along with immunosuppressive therapy with corticosteroids. Although the woman’s platelet levels initially improved, they later declined, requiring escalation of care. Following a multidisciplinary team decision prompted by worsening platelet counts, labor was induced, and she delivered a healthy baby by cesarean section.
The second woman, 24 and at 31 weeks of pregnancy, showed stroke-like symptoms, including difficulty speaking and weakness on one side of the body. TTP was not initially suspected, but worsening blood markers and MAHA led to the diagnosis. She was promptly started on TPE and corticosteroids, which stabilized her condition. The woman ultimately delivered her baby without complications by cesarean section and remained in remission during follow-up.
The third woman, 36 and at full term, was initially suspected to have a pregnancy-related condition called HELLP syndrome, which is marked by liver problems, hemolysis, and low platelet counts. However, her platelet count continued to decline after delivery, confirming the TTP diagnosis. She was treated with TPE and required additional therapies, including rituximab and Cablivi, due to disease relapse. Her condition stabilized with treatment, and she did not experience further relapses during four years of follow-up.
“The cases highlight essential diagnostic challenges, including overlaps with other pregnancy-related [complications] such as HELLP syndrome … as well as the importance of early recognition and immediate initiation of TPE to prevent life-threatening complications,” the researchers wrote. “In practice, the presence of MAHA and thrombocytopenia warrants immediate evaluation for TTP.”
The researchers stressed that timely ADAMTS13 testing is essential to confirm the diagnosis, distinguish between acquired and inherited TTP, and guide treatment.
