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

Abstract

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.

Pathological Mechanisms and Novel Testing Methods in Thrombotic Thrombocytopenic Purpura

“…The widely accepted mechanism of TTP begins with a disruption in normal regulation of the coagulation process due to decreases in ADAMTS13. A major function of ADAMTS13 is as a negative regulator of early coagulation, namely cleavage of UL-vWF multimers, thereby disallowing the formation of platelet-vWF thrombi in the small vessels thus maintaining homeostasis [ 1 , 2 , 3 , 4 , 5 , 7 ]. The congenital lack of, or autoantibodies against, ADAMTS13 depletes its levels in circulation and leads to the formation of platelet-multimer thrombi.…”

“…The nervous system is the most common system affected, with neurological symptoms occurring in 40–80% of TTP patients; major sequelae, such as stroke, can be fatal [ 4 ]. Additional systems may be affected, including but not limited to the kidneys, skin, and gastrointestinal tract [ 4 , 5 ]. However, these symptoms overlap extensively with various TMAs in addition to any number of medication reactions, infections, autoimmune diseases, and/or neoplasias [ 3 , 5 , 6 , 7 ].…”

“…Additional systems may be affected, including but not limited to the kidneys, skin, and gastrointestinal tract [ 4 , 5 ]. However, these symptoms overlap extensively with various TMAs in addition to any number of medication reactions, infections, autoimmune diseases, and/or neoplasias [ 3 , 5 , 6 , 7 ]. Complicating these differential diagnoses is the 90% mortality rate of untreated TTP requiring a high level of suspicion and testing which at times could be delayed [ 3 ].…”

New‐onset and relapsed thrombotic microangiopathy post‐COVID‐19 vaccination

“…A total of 84 cases of TMA associated with COVID‐19 vaccination were found in 58 articles, 2,9–65 including 64 patients diagnosed with TTP (Table 1), 17 cases manifested as aHUS (Table 2, cases 1–17), and three patients presented as unclassified TMA (Table 2, cases 18–20). Subsequently, relevant clinical records were extracted for analysis, including baseline characteristics, laboratory tests, treatment options, and outcomes.…”

“…There were 64 affected individuals presented as TTP (Table 1), 2,9–49 including 44 newly diagnosed (68.8%) and 20 relapsed (31.3%). The median age was 49.5 (14–84) years.…”