Paraneoplastic acral vascular syndrome: first report linked to chronic myelomonocytic leukemia and summary of existing literature

This report presents an unprecedented case of paraneoplastic acral vascular syndrome manifesting as a clinical presentation of chronic myelomonocytic leukemia (CMML). The case study examines the specific clinical features observed, the diagnostic challenges encountered, and the treatment considerations. A comprehensive review of recent literature highlights the mechanisms underlying this syndromic association. It reviews previously documented cases, providing context and deepening understanding of the relationship between CMML and paraneoplastic vascular phenomena. A 60-year-old woman was admitted with symptoms including new-onset digital ischemia, anemia, splenomegaly, and several constitutional symptoms that had persisted for 3 months. After evaluating all available data, the patient was diagnosed with CMML-1 (proliferative type) complicated by acral vascular cyanosis up to digital gangrene, and chemotherapy was initiated. The treatment plan involved hydroxyurea, Vidaza (azacitidine), and oral Vespid. Fortunately, the patient showed improvement. To our knowledge, this case is the first documented association between paraneoplastic acral vascular syndrome and CMML.