Autoimmune- and complement-mediated hematologic condition recrudescence following SARS-CoV-2 vaccination

Abstract

A variety of autoimmune disorders have been reported after viral illnesses and specific vaccinations. Cases of de novo immune thrombocytopenia (ITP) have been reported after SARS-CoV-2 vaccination, although its effect on preexisting ITP has not been well characterized. In addition, although COVID-19 has been associated with complement dysregulation, the effect of SARS-CoV-2 vaccination on preexisting complementopathies is poorly understood. We sought to better understand SARS-CoV-2 vaccine-induced recurrence of autoimmune- and complement-mediated hematologic conditions. Three illustrative cases were identified at the University of Washington Medical Center and the Seattle Cancer Care Alliance from January through March 2021. We describe the recrudescence of 2 autoimmune conditions (ITP and acquired von Willebrand Disease [AvWD]/acquired hemophilia A) and 1 complementopathy (paroxysmal nocturnal hemoglobinuria [PNH]). We report the first known case of AvWD/acquired hemophilia A, and describe the first PNH exacerbation in the absence of complement inhibition after SARS-CoV-2 vaccination. Although SARS-CoV-2 vaccine-induced ITP is a known concern, our case clearly depicts how thrombocytopenia in the setting of preexisting ITP can sequentially worsen with each vaccine dose. Based on our experiences and these examples, we provide considerations for how to monitor and assess risk in patients with underlying autoimmune- and complement-mediated hematologic conditions.

Graphical abstract

Figure 1.

Disease parameters relative to dose 2 of the SARS-CoV-2 vaccine series. (A) The platelet count of patient 1 exhibited a mild decrease after dose 1 and a marked decrease after dose 2. The eltrombopag daily dose was adjusted to compensate for dynamic platelet destruction. (B) In patient 2, the hemolysis marker, LDH, rapidly increased after dose 2. Eculizumab was administered on day 3, with excellent response. (C) In patient 3, factor VIII and vWF activities were initially undetectable, but increased in response to methylprednisolone (MP) and prednisone (pred), Humate-P, and IVIg. aPTT improved concurrently. Red blood cell transfusion and hematocrit are noted.

Figure 2.

Ecchymoses due to acquired vWD/acquired hemophilia A. (A) In patient 3, left greater than right upper extremity ecchymoses developed on day 4 after dose 2 of the Moderna SARS-CoV-2 vaccine. (B) A similar right upper extremity ecchymosis had spontaneously developed about 1 year earlier. Although laboratory testing at this time was notable for abnormal ristocetin-induced platelet aggregation and a mildly prolonged aPTT, a definitive diagnosis of acquired vWD/acquired hemophilia A was not made until her hospital presentation, as described in case 3.