Precautions ensure safe C-section for woman with PNH, anemia
Case report shows importance of managing bleeding, clotting risks
A case report highlights the steps necessary to prevent dangerous clots and minimize bleeding when performing a cesarean section on someone who has co-occurring paroxysmal nocturnal hemoglobinuria (PNH) and aplastic anemia, a condition in which the bone marrow fails to produce enough blood cells.
Researchers in Greece described the case of a woman with both conditions who, with appropriate precautions, underwent a successful cesarean section to deliver a baby boy. The study, “Rotational Thromboelastometry (ROTEM)-Assisted Anaesthetic Management of a Parturient With Paroxysmal Nocturnal Haemoglobinuria and Aplastic Anaemia for Caesarean Section: A Case Report,” was published in Cureus.
“Reports of anaesthetic management in pregnant patients with concurrent PNH and [aplastic anemia] are exceedingly rare, making this case a valuable contribution to the literature,” the researchers wrote.
In PNH, part of the immune system becomes abnormally activated, leading to the destruction of blood cells and resulting in low blood cell counts and potentially dangerous blood clots. Aplastic anemia is a disorder in which the body stops making enough new blood cells, resulting in low counts of blood cells and of platelets (cell fragments that help with blood clotting).
PNH and aplastic anemia are closely related, and people with either disorder are at increased risk of developing the other. Yet, the co-occurrence of these two diseases can pose a conundrum for clinicians: on the one hand, PNH can cause dangerous clots, but on the other, aplastic anemia can lead to low platelet levels, making it harder for blood to clot correctly. Clinicians have to carefully balance treatments to prevent bleeds and treatments to prevent clots to minimize the risk of either. This balancing act can be especially tricky in pregnant patients, where a dangerous clot or bleed can be life-threatening for both the pregnant person and the developing fetus.
Diagnosis comes during pregnancy
The case report centers on a 25-year-old woman who was pregnant for the first time. Shortly after she became pregnant, her lab tests revealed low blood cell counts. Further testing led to a diagnosis of co-occurring PNH and aplastic anemia.
The woman began treatment with the approved PNH treatment Soliris (eculizumab). She also received the immunosuppressant cyclosporine, as well as iron and folic acid supplements. But her diseases remained difficult to manage, and she required regular transfusions of red blood cells and platelets throughout her pregnancy. By the time she reached the third trimester, she needed transfusions on a near-daily basis.
The woman underwent a planned cesarean section in her 33rd week of pregnancy. C-sections are often performed while a patient is awake, but given the complexity of her condition, the woman and her clinicians agreed that the surgery would be done under general anesthesia.
During the surgery, clinicians used a technique called rotational thromboelastometry (ROTEM) to monitor the extent to which the woman’s blood was clotting. This allowed them to fine-tune treatment: For example, when her blood was in a more pro-clotting state, they could delay administration of therapies to promote clotting.
“ROTEM may offer useful, real-time information to guide transfusion and coagulation management and could potentially assist in balancing the dual risks of bleeding and thrombosis [clotting],” the researchers wrote, noting a need for additional studies on the use of this technique in people with PNH and/or aplastic anemia.
The surgery was completed in just over an hour, and no notable complications were reported after delivery.
The woman’s experience highlights the importance of careful management during pregnancy and birth for people with co-occurring PNH and aplastic anemia, the scientists said.
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