A Woman With Back, Chest Pain After Eating Wings at a Restaurant

Sunny B. Patel, MD; Richa Dhawan, MD


May 31, 2022

The patient in this case had hemolytic anemia, thrombocytopenia, hyperbilirubinemia, low C3, proteinuria, and recent discoid lupus rash. Her ADAMTS13 findings did come back slightly low, with elevated anti-ADAMTS13 autoantibody results; however, these studies often take longer than 1 week for results. The schistocytes noted on the peripheral blood smear were of greater concern. This prompted immediate initiation of daily plasma exchange. Plasma exchange is the combination of plasmapheresis, which may remove unusually large multimers of von Willebrand factor and autoantibodies against ADAMTS13, and infusion of fresh-frozen plasma or cryosupernatant, which contains additional metalloprotease. Plasma exchange has significantly improved survival rates of TTP, which was previously around 10%. Approximately 80% of patients now survive without permanent organ damage.[6]

The patient was also started on high-dose intravenous (IV) steroids (methylprednisolone, 1 g daily for 3 days) as part of treatment for TTP and exacerbation of SLE. Liver and kidney biopsies were deferred because the risks outweighed the benefits of performing each procedure. The management of this patient was discussed with an infectious disease specialist, given the newly diagnosed viral hepatitis C infection. Acute therapy for hepatitis C was postponed until after successful treatment of TTP due to the potential for worsening the condition.

This patient initially did well, with a platelet count nearing normal after 7 days of plasma exchange and IV steroids; however, she then had an exacerbation without any inciting factors, during which laboratory results again demonstrated thrombocytopenia, hemolytic anemia, a positive Coombs test result, and severe ADAMTS13 deficiency (activity level, 16%), as well as hypocomplementemia. Some data support the idea that higher titers of anti-ADAMTS13 autoantibodies (as in this patient) place patients at greater risk for complications and multiple exacerbations. Given the severity of illness and inadequate response, the patient was then given rituximab as adjuvant therapy.

The patient had a complicated hospitalization, with development of neurologic abnormalities, including confusion, headache, and left foot drop. She also developed renal failure that required hemodialysis, and multiple infections, including methicillin-sensitive Staphylococcus aureus and methicillin-resistant S aureus (MRSA) bacteremia, E coli pneumonia, and urinary tract infection (UTI) secondary to Klebsiella pneumoniae . Patients with TTP requiring ICU stay have a high mortality rate; this patient survived, however, and plasma exchange was subsequently tapered off after 32 sessions. Hemodialysis was no longer required and steroids were tapered. Given persistent the nonnephrotic range proteinuria and mild renal dysfunction, this patient will probably require a renal biopsy to help further guide long-term management, particularly if lupus nephritis or membranous proliferative glomerulonephritis is found. The patient was physically deconditioned, as is expected after a prolonged hospitalization, but her confusion and headache resolved and her hemoglobin level and platelet counts have remained stable, near baseline.


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