An example of chocked disk of 3-4 diopters (right eye) and 4-5 diopters (left eye) in a patient with cerebral alteration (encephalomalacia respectively bleeding) suffering from polycythemia vera is discussed in the light of own observation, literature, differential diagnosis, pathogenesis, and therapy. An internal treatment by radioactive phosphorus and blood-letting gave restitutio fere ad integrum.
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Blood Res
January 2025
Department of Rheumatology of Lucania - UOSD of Rheumatology, "Madonna Delle Grazie" Hospital, Matera, Italy.
This case report describes the clinical course of a 78-year-old patient diagnosed with polycythemia vera (PV), who presented with pronounced acrocyanosis of the hands in 2021. The patient was treated with hydroxyurea (oncocarbide), and nailfold capillaroscopy revealed an "abnormal pattern" characterized by pronounced architectural disarray and capillary tortuosity, which is uncommon in patients with myeloproliferative neoplasms (MPNs). In 2023, owing to suboptimal symptom management and hematological side effects, the treatment was switched to ruxolitinib, which led to significant clinical improvements by 2024, including near-complete resolution of acrocyanosis and substantial improvement in capillaroscopic abnormalities, with only residual capillary tortuosity noted.
View Article and Find Full Text PDFCurr Med Res Opin
January 2025
Section of Hematology, Department of Radiological and Hematological Sciences, Catholic University, Fondazione Policlinico Gemelli IRCCS, Rome, Italy.
Introduction: The management of patients with Polycythemia Vera (PV) traditionally includes low-dose aspirin, phlebotomy, and cytoreductive therapy for high-risk individuals. Recent evidence suggests that cytoreductive treatment may be warranted for patients with additional risk factors beyond the traditional criteria of a history of thrombosis and age over 60 years. Introducing new therapeutic agents, including ropeginterferon alfa-2b and ruxolitinib, enables a more personalized treatment approach tailored to individual patient characteristics.
View Article and Find Full Text PDFCureus
January 2025
Neurology, St. Luke's Medical Center, Quezon City, PHL.
[This corrects the article DOI: 10.7759/cureus.76294.
View Article and Find Full Text PDFBlood Adv
January 2025
Department of Hematology, Oncology, Hemostaseology, and Stem Cell Transplantation, Medical Faculty, RWTH Aachen University, Aachen, Germany.
Interferon alpha (IFNa) is approved for the therapy of patients (pts) with polycythemia vera (PV), a subtype of myeloproliferative neoplasms (MPN). Some pts achieve molecular responses (MR), but clonal factors sensitizing for MR remain elusive. We integrated colony formation and differentiation assays with single-cell RNA seq and genotyping in PV-derived cells vs.
View Article and Find Full Text PDFAm J Hematol
January 2025
CRIMM, Center Research and Innovation of Myeloproliferative Neoplasms, University of Florence, AOU Careggi, Florence, Italy.
The clinical relevance of TP53 mutations (TP53) in myeloproliferative neoplasms (MPN) and their prognostic interaction with MPN subtype designation has not been systematically studied. In the current study, 114 patients with MPN harboring TP53 (VAF ≥ 2%) were evaluated for overall survival (OS), calculated from the time of TP53 detection: chronic phase myelofibrosis (MF-CP; N = 61); blast-phase (MPN-BP; N = 31) or accelerated-phase (MPN-AP; N = 16) MPN, and polycythemia vera/essential thrombocythemia (PV/ET; N = 6). Sixty-five (57%) patients harbored International Consensus Classification (ICC)-defined multihit TP53 and 56 (49%) monosomal/complex karyotype (MK/CK).
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