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. Preprints2023, 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
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. Preprints2023, 2023092129. https://doi.org/10.20944/preprints202309.2129.v1
APA Style
Tomasello, R., Cammarata, C., Mattana, M., Ingrascì, M. G., Giordano, G., Lucchesi, A., Ghanima, W., Siragusa, S. M., & Napolitano, M. (2023). Refractory Immune Thrombocytopenia or Myelodysplastic Syndrome Presenting with Isolated Thrombocytopenia? A Review of Literature and a Case Report. Preprints. https://doi.org/10.20944/preprints202309.2129.v1
Chicago/Turabian Style
Tomasello, R., Sergio Mario Siragusa and Mariasanta Napolitano. 2023 "Refractory Immune Thrombocytopenia or Myelodysplastic Syndrome Presenting with Isolated Thrombocytopenia? A Review of Literature and a Case Report" Preprints. 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.
Copyright:
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