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Transient Leukoerythroblastosis Unmasking Clonal Hematopoiesis with Myelofibrosis in Refractory Thrombocytopenia

Refractoriness to standard first-line therapy in immune thrombocytopenia (ITP) should foster additional diagnostic work-up to exclude hematological clonal disease, mostly myelodysplatic syndrome (MDS) or clonal cytopenia of unknown significance (CCUS), which may present with isolated thrombocytopeni...

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Bibliographic Details
Published in:Hemato 2024-09, Vol.5 (3), p.258-263
Main Authors: Malipiero, Giacomo, Ermacora, Anna, Pratesi, Chiara, Carbone, Antonino, Rogato, Adolfo, Prosdocimo, Simonetta, De Rosa, Rita, Doretto, Paolo
Format: Article
Language:English
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Summary:Refractoriness to standard first-line therapy in immune thrombocytopenia (ITP) should foster additional diagnostic work-up to exclude hematological clonal disease, mostly myelodysplatic syndrome (MDS) or clonal cytopenia of unknown significance (CCUS), which may present with isolated thrombocytopenia of immune or non-immune origin. We herein report on a patient who showed a transient leukoerythroblastic reaction (LEB) associated with bone marrow myelofibrosis upon rompilostim treatment, challenging a diagnosis of primary ITP and requiring additional investigations. RUNX-1-mutated myelodysplastic syndrome was eventually diagnosed. Even though LEB and marrow fibrosis have already been rarely reported during romiplostim treatment for ITP, this is the first case to our knowledge in which a background clonal hematopoiesis was diagnosed and deemed potentially involved in the abnormal response to this thrombopoietin receptor agonist (TPO-RA).
ISSN:2673-6357
2673-6357
DOI:10.3390/hemato5030021