Work

Thrombocytosis and megakaryocyte changes associated with PRCA

Year: 2024

Type: article

Abstract: Acquired pure red cell aplasia (PRCA) is a rare hematological disorder that results from failure of erythropoiesis 1,2 and can be distinguished from other bone marrow failure disorders by reticulocytopenia, normal granulopoiesis, and megakaryopoiesis.3,4 PRCA is often idiopathic and likely due to cytotoxic T-cell-mediated destruction of early erythroid precursors.Indeed, it can be associated with conditions such as T-cell large granular lymphocytic leukemia, B-cell dyscrasia, thymoma, immunodeficiency, and infections.[5][6][7][8] Thrombocytosis refers to an abnormal elevation in platelet count of >450 000 × 10 9 /L.This condition can be related to a primary process, usually associated with a myeloproliferative neoplasm known as essential thrombocythemia, but it more commonly presents as an epiphenomenon of other causes that include iron deficiency, chronic inflammatory conditions, and asplenia.9,10 Thrombocytosis has not been associated with PRCA.Herein, we report a cohort of patients with PRCA and thrombocytosis that have not been defined in the literature.A retrospective analysis was conducted on patient records diagnosed with PRCA at the University of Texas Southwestern and Cleveland Clinic Foundation between 2000 and 2022.This study was approved by the institutional review board of the University of Texas Southwestern Medical Center and Cleveland Clinic Foundation.The primary objective was to investigate the presence of thrombocytosis and/or megakaryocyte changes in these PRCA cases.Clinical, laboratory, and molecular data were abstracted, adhering to the guidelines established by the Declaration of Helsinki and the respective participating institutions.Among a total of 90 patients, comprehensive analysis of 27 cases were identified as having acquired PRCA with thrombocytosis and/or megakaryocyte changes noted on bone marrow biopsies (Table 1) examined.To assess treatment efficacy, hemoglobin and platelet count before and after resolution of PRCA were compared.The Shapiro-Wilk test was used to assess normality.For normally distributed data, a paired t test was performed.For nonnormally distributed data, a Wilcoxon matched-pair signedrank test was performed.For all relevant comparisons, a P value <.05 was used to set statistical significance.In a cohort of 90 patients with PRCA, we identified 27 individuals with concurrent thrombocytosis and/or megakaryocyte changes (Table 1).Of the 27 patients, the mean hemoglobin count at diagnosis was 7.6 ± 0.7 g/dL, and the mean platelet count at diagnosis was 389 000 ± 80 400 × 10 9 /L.In total, 51.9% were female.A total of 20 demonstrated megakaryocyte hyperplasia, 4 demonstrated hyperlobation, and 1 displayed megakaryocyte dysplasia.Three patients tested positive for parvovirus on polymerase chain reaction, 1 patient had chronic lymphocytic leukemia, and 10 patients had large granular lymphocytic leukemia.

Source: Blood Advances

DOI: 10.1182/bloodadvances.2023012309