Concurrence of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura: a case report and review of the literature.

BACKGROUND: Immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura are both causes of thrombocytopenia. Recognizing thrombotic thrombocytopenic purpura is crucial for subsequent treatment and prognosis. In clinical practice, corticosteroids and rituximab can be used to treat both immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura; plasma exchange therapy is the first-line treatment in thrombotic thrombocytopenic purpura, while corticosteroids are strongly recommended as first-line treatment in immune thrombocytopenic purpura. The differential diagnosis of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura is essential in clinical practice. However, case reports have suggested that immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura can occur concurrently. CASE PRESENTATION: We report the case of a 32-year-old Asian female without previous disease who presented with pancytopenia, concurrent with immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura. The morphology of the megakaryocytes in the bone marrow indicated immune-mediated thrombocytopenia. The patient received glucocorticoid treatment, and her platelet count increased; however, schistocytes remained high during the course of the therapy. Further investigations revealed ADAMTS13 activity deficiency and positive ADAMTS13 antibodies. The high titer of antinuclear antibody and positive anti-U1-ribonucleoprotein/Smith antibody indicated a potential autoimmune disease. However, the patient did not fulfill the current criteria for systemic lupus erythematosus or mixed connective tissue disease. The patient responded well to plasma exchange therapy, and her platelet count remained normal on further follow-up. CONCLUSIONS: Concurrence of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura is rare, but clinicians should be aware of this entity to ensure prompt medical intervention. Most of the reported cases involve young women. Human immunodeficiency virus infection, pregnancy, and autoimmune disease are the most common underlying conditions.

Clinical relapse of immune-mediated thrombotic thrombocytopenic purpura following COVID-19 vaccination.

De novo and relapsed immune-mediated thrombotic thrombocytopenic purpura (iTTP) have been documented to have occurred following severe acute respiratory syndrome coronavirus 2 (COVID-19) vaccination. Here, we present a case of a 28-year-old woman who received the tozinameran (BNT162b2, Pfizer-BioNtech) vaccine for COVID-19 and experienced an iTTP relapse during longitudinal follow-up. She received the vaccine 30 months after her initial diagnosis, while she was in clinical remission. She was not in complete ADAMTS-13 remission, as she had undetectable ADAMTS-13 activity during follow-up except for one isolated measurement of 48%. Shortly after vaccination, she developed complaints of bruising, petechiae, ataxia, and an episode of slurred speech. Laboratory testing demonstrated thrombocytopenia, schistocytes, and eventually undetectable ADAMTS-13 activity. She was successfully treated with caplacizumab, rituximab, and corticosteroids without plasma exchange. She achieved complete clinical and ADAMTS-13 remission after treatment. We recommend caution in the administration of COVID-19 vaccines for survivors of iTTP in remission with severely deficient ADAMTS-13 activity.

Thrombotic Thrombocytopenic Purpura after Ad26.COV2-S Vaccination.

The U.S. Food and Drug Administration (FDA) recently issued an Emergency Use Authorization (EUA) for two highly effective Sars-CoV-2 (COVID-19) vaccines from Pfizer-BioNTech and Moderna. More recently, EUA was granted for the Johnson and Johnson COVID-19 vaccine which uses traditional virus-based technology. In this vaccine, researchers added the gene for the coronavirus spike protein to modified Adenovirus 26 and named it Ad26.COV2-S. Nearly 7 million doses of the Ad26.COV2-S have been administered as of mid-April 2021. Recently the Federal Drug Administration and Center for Disease Control and Prevention reviewed data involving six reported cases in the United States of cerebral venous sinus thrombosis in combination with thrombocytopenia in people who received the vaccination. All cases were in women between 18 and 48, with symptoms developing six to 13 days after vaccination. A recent study in the United Kingdom reported similar events in 23 patients age 21 to 77, 61% of which were female, with cases of presumed vaccine induced thrombosis and thrombocytopenia occurring six to 24 days after vaccination. We report a 62-year-old female who presented to the emergency department (ED) with acute onset of altered mental status. She had received the Ad26.COV2-S vaccine 37 days prior to ED presentation. She developed thrombotic thrombocytopenic purpura (TTP) and no other cause was found. To our knowledge this is the first case in the United States of thrombotic thrombocytopenic purpura after receiving the Ad26.COV2-S COVID-19 vaccine.