Abstract Number: PB1319
Meeting: ISTH 2020 Congress
Background: Cyclic thrombocytopenia (CTP) is characterized by periodical fluctuations of the platelet counts. Most cases are misdiagnosed as immune thrombocytopenia (ITP). The pathogenesis is incompletely understood and response to specific ITP treatments is poor.
Aims: To study the pathogenesis, risk factors, course of disease and response to treatment.
Methods: Between 1991 and 2018 all records of patients treated for isolated thrombocytopenia were screened. Blood counts, clinical chemistry, bone marrow aspiration, comorbidities, co-medications, complications and specific therapies were collected for patients who were not responding to ITP treatment and had the typical platelet count fluctuations. Data are presented as a descriptive analysis.
Results: We identified 10 patients of whom 8 were female (Figure 1). All patients were initially diagnosed as ITP. At presentation, 4 patients had mild mucocutaneous bleeding. The time interval until CTP diagnosis ranged from 2 months to 13 years. Platelet nadirs ranged from 4 to 46 G/l and cycle duration from 14 to 35 days. 9 patients had a disease of the thyroid gland. T-cell receptor beta and/or gamma rearrangement was found in 7 patients. Until CTP diagnosis patients were unsuccessfully treated with corticosteroids (all patients), intravenous immunoglobulins (6), TPO-receptor agonists (4), rituximab (5) and splenectomy (6). Upon CTP diagnosis, 7 patients received cyclosporine. One patient each developed T-cell large granular lymphocytic leukemia and acute myeloid leukemia. One patient had pulmonary embolism during TPO agonist therapy. One patient had intracranial bleeding at a platelet count of 1 G/l. A remission occurred in 6 patients.
Conclusions: CTP has to be considered in patients with isolated thrombocytopenia who do not respond to ITP treatment. CTP predominantly occurs in women and is often associated with a disease of the thyroid gland. Transformation into malignant hematological disorders can occur. Remission rates are high.
To cite this abstract in AMA style:Steinbrecher O, Eischer L, Sinkovec H, Mitrovic M, Eichinger S, Kyrle PA. Characteristics of Patients with Cyclic Thrombocytopenia: An Observational Cohort Study [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/characteristics-of-patients-with-cyclic-thrombocytopenia-an-observational-cohort-study/. Accessed September 21, 2023.
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