Young women appear to be more susceptible to the concurrence of immune thrombocytopenic purpura (ITP) and thrombotic thrombocytopenic purpura (TTP), a new study published in the Journal of Medical Case Reports revealed. In addition, human immunodeficiency virus infection, pregnancy, and autoimmune disease are the most common underlying conditions linked to ITP and TTP.
The study reported the case of a 32‑year‑old Asian female without any prior health issues. She was presented with pancytopenia, concurrent with immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura. The megakaryocyte morphology in the bone marrow showed immune‑mediated thrombocytopenia. Although glucocorticoid treatment increased her platelet count, schistocytes remained elevated throughout therapy.
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She was also presented with anemia, with slightly increased mean corpuscular volume (MCV), increased reticulocyte level, decreased haptoglobin, and increased lactate dehydrogenase (LDH) level, suggesting hemolysis. Further analysis revealed a deficiency of ADAMTS13 activity and positive ADAMTS13 antibodies along with a high titer of antinuclear antibodies and a positive result for anti‑U1‑ribonucleoprotein/Smith antibody, suggesting the possibility of a potential autoimmune disease (TTP in this case). However, the patient did not meet the criteria for systemic lupus erythematosus (SLE) or mixed connective tissue disease. The patient responded well to the plasma exchange therapy, and her platelet count remained normal throughout the follow‑up.
The patient was initially treated with intravenous methylprednisolone 40 mg for one day, but the schistocyte level in the peripheral blood increased to over 6%. The dose was then increased to 240 mg for five days, increasing the patient’s platelet count to 216× 109/L. However, the schistocytes remained at 5% while the PLASMIC score was high.
Corticosteroids and rituximab can treat both ITP and TTP; however, plasma exchange therapy (PEX) is the first treatment for TTP, while corticosteroids are recommended as the primary treatment for ITP. Therefore, it is crucial to differentiate between ITP and TTP in clinical practice accurately.
Caplacizumab is the newest TTP therapy, which is a humanized, single-variable domain, anti-vWF immunoglobulin targeting the A1 domain of vWF , hence preventing the interaction with the platelet glycoprotein Ib-IX-V receptor. This therapy showed effectiveness when added to the standard treatment for acquired TTP as it shortened the time to normalize platelets and decreased the recurrence rate.
“This is a rare case of concurrent ITP and TTP in a previously healthy woman. This case highlights the importance of considering all possible causes of thrombocytopenia, especially when specific treatments (i.e., PEX, glucocorticoids) should be given in the first line,” the authors added.
Lin HC, Huang J, Huang J et al. Concurrence of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura: a case report and review of the literature. J Med Case Reports. Published online February 8, 2023. doi: 10.1186/s13256-023-03762-y