Preprint Review Version 1 Preserved in Portico This version is not peer-reviewed

Refractory Immune Thrombocytopenia or Myelodysplastic Syndrome Presenting with Isolated Thrombocytopenia? A Review of Literature and a Case Report

Riccardo Tomasello
ORCID logo ,
Claudia Cammarata
,
Marta Mattana
,
Manuela Giuseppa Ingrascì
,
Giulio Giordano
ORCID logo ,
Alessandro Lucchesi
ORCID logo ,
Waleed Ghanima
,
Sergio Mario Siragusa
,
Mariasanta Napolitano
*
Version 1 : Received: 29 September 2023 / Approved: 30 September 2023 / Online: 30 September 2023 (07:14:13 CEST)

How to cite: Tomasello, R.; Cammarata, C.; Mattana, M.; Ingrascì, M.G.; Giordano, G.; Lucchesi, A.; Ghanima, W.; Siragusa, S.M.; Napolitano, M. Refractory Immune Thrombocytopenia or Myelodysplastic Syndrome Presenting with Isolated Thrombocytopenia? A Review of Literature and a Case Report. Preprints 2023, 2023092129. https://doi.org/10.20944/preprints202309.2129.v1 Tomasello, R.; Cammarata, C.; Mattana, M.; Ingrascì, M.G.; Giordano, G.; Lucchesi, A.; Ghanima, W.; Siragusa, S.M.; Napolitano, M. Refractory Immune Thrombocytopenia or Myelodysplastic Syndrome Presenting with Isolated Thrombocytopenia? A Review of Literature and a Case Report. Preprints 2023, 2023092129. https://doi.org/10.20944/preprints202309.2129.v1

Abstract

Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by isolated thrombocytopenia caused by both immune-mediated platelet destruction and impaired platelet production in the bone mar-row, in the absence of any other identifiable cause of low platelets count. ITP in adult patients is a disease that frequently relapses and/or becomes refractory to multiple treatments during its course, with only a small minority of adult patients attaining a sustained complete remission off-therapy. Treatment of refrac-tory ITP can be challenging, due to the unlikeliness of attaining complete response over time. On the other hand, isolated thrombocytopenia may be due to a misdiagnosed clonal myeloid disorder, such as myelodys-plastic syndrome (MDS) or chronic myelomonocytic leukemia (CMML), thus mimicking ITP. We here per-form a narrative review on the association between ITP and MDS/CMML and the possibility of overlap and misdiagnosis between these conditions, while we report also the case a patient treated for a severe re-fractory thrombocytopenia which bone marrow examination led to suspect an overlap of MDS and ITP. Our clinical case describes ITP which is refractory to glucocorticoids, high-dose immunoglobulins, Rituxi-mab, splenectomy, thrombopoietin receptor agonists and Fostamatinib, that ultimately attained a short-lived response to a hypometylating agent that was initiated due to bone marrow examination that aroused the suspect of MDS.

Keywords

Immune thrombocytopenia; chronic ITP; myelodysplastic syndrome; chronic myelomonocytic leukemia; refractory thrombocytopenia

Subject

Medicine and Pharmacology, Hematology

Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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