Thrombocytopenia with concomitant anemia is a serious condition with a high mortality risk. Destruction of platelets, i.e., thrombocytopenia, can be secondary to either auto-antibodies (immune-mediated) or mechanical destruction (non-immune-mediated). The Coombs test is a widespread tool to differentiate between the two categories, resulting in different specific treatment approaches for each diagnosis. A peripheral blood smear can also help make the diagnosis; for instance, in cases of mechanical destruction such as thrombotic thrombocytopenic purpura (TTP), the red blood cell (RBC) shape looks fragmented, forming schistocytes. In rare instances, TTP can present with both schistocytes and a positive Coombs test, challenging the diagnosis of TTP. TTP is a hematological emergency requiring appropriate anticipation and the initiation of treatment prior to the confirmatory ADAMTS-13 test results. Mild forms of TTP can be managed with glucocorticoids and therapeutic plasma exchange. Refractory cases need more aggressive additional treatment with caplacizumab and rituximab. Caplacizumab is an expensive medication that is usually reserved for use after confirmation of a TTP diagnosis. The advantage of caplacizumab lies in its targeted mechanism of action against the A1 domain of the von Willebrand multimers that are normally destructed by the ADAMTS-13 enzyme. Here, we present a young female patient with confirmed TTP, and the initial diagnosis was challenged by the presence of antibodies with the Coombs test. Very little research has studied this rare instance and the appropriate treatment. Our case will save many future lives, as clinicians should be more aggressive in treating refractory TTP with a positive Coombs test.

Thrombotic thrombocytopenic purpura (TTP) is a rare pregnancy complication characterized by microangiopathic hemolytic anemia and consumption thrombocytopenia. We herein describe the case report of a 32-year-old woman who was six weeks pregnant with twins and developed thrombotic thrombocytic purpura (TTP). The patient had a history of sickle cell trait, migraines, and preeclampsia. She presented with complaints of nausea, fatigue, sore throat, and cough and was found to be anemic with a hemoglobin of 7 g/dl and thrombocytopenic with a platelet count of 8 x 103/μL. The patient was promptly initiated on steroids and plasmapheresis with an excellent initial response. However, after three days, she developed a sudden onset headache and shortness of breath, and repeat labs showed worsening anemia (7.3 g/dl) and thrombocytopenia (8 x 103/μL). ADAMTS13 activity was significantly low at 2%. Plasmapheresis was continued, and caplacizumab and rituximab treatment was initiated. The fetal ultrasound showed no cardiac activity in the fetal poles, and the patient had a dilation and curettage (D&C) for a missed abortion. She was discharged with a prednisone taper, daily caplacizumab, and weekly rituximab. This case report underscores the criticality of the prompt identification of TTP in its early stages, and appropriate management strategies for patients with refractory TTP (rTTP), including plasmapheresis, caplacizumab, and rituximab.

A 30-year-old woman who was 14 weeks pregnant was admitted to our hospital due to purpura, nasal bleeding, and abdominal pain. She was diagnosed with acquired thrombotic thrombocytopenic purpura (TTP) based on the presence of hemolytic anemia, thrombocytopenia, decreased ADAMTS 13 activity (<0.01 IU/ml), and high ADAMTS 13 inhibitor levels (4.8 BU/ml). Plasma exchange (PE) and steroid therapy were immediately administered. However, because she did not respond to these therapeutic approaches, rituximab was additionally administered on the sixth day of treatment. The level of ADAMTS 13 inhibitor increased to 12.5 BU/ml on the seventh day. Renal insufficiency, disturbed consciousness, and genital bleeding did not improve in spite of daily PE, steroid therapy, and second dose of rituximab. She finally died after sudden convulsions on the 14th day. Although the treatment outcomes of TTP have remarkably improved, some cases are refractory to therapy. Establishment of adequate treatment strategies for acquired TTP in pregnant women is required.

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