Abstract
The co-occurrence of myeloid neoplasms and lymphoproliferative diseases (LPDs) has been epidemiologically described, particularly in myeloproliferative neoplasms (MPNs). However, the clinical features of these patients are poorly known. In this study, we evaluated a single-center cohort of 44 patients with a diagnosis of myeloid and LPD focusing on clinical features, therapy requirement, and outcome. The two diagnoses were concomitant in 32% of patients, while myeloid disease preceded LPD in 52% of cases (after a median of 37 months, 6–318), and LPD preceded myeloid neoplasm in 16% (after a median of 41 months, 5–242). The most prevalent LPD was non-Hodgkin lymphoma (50%), particularly lymphoplasmacytic lymphoma (54.5%), followed by chronic lymphocytic leukemia (27%), plasma cell dyscrasias (18.2%), and rarer associations such as Hodgkin lymphoma and Erdheim–Chester disease. Overall, 80% of BCR-ABL1-negative MPN patients required a myeloid-specific treatment and LPD received therapy in 45.5% of cases. Seven subjects experienced vascular events, 13 a grade >/= 3 infectious episode (9 pneumonias, 3 urinary tract infection, and 1 sepsis), and 9 developed a solid tumor. Finally, nine patients died due to solid tumor (four), leukemic progression (two), infectious complications (two), and brain bleeding (one). Longer survival was observed in younger patients (p = 0.001), with better performance status (p = 0.02) and in the presence of driver mutations (p = 0.003). Contrarily, a worse survival was significantly associated with the occurrence of infections (p < 0.0001). These data suggest that in subjects with co-occurrence of myeloid and lymphoid neoplasms, high medical surveillance for infectious complications is needed, along with patient education, since they may negatively impact outcome.
Highlights
The potential for myeloid neoplasms to evolve one into each other is largely known [i.e., leukemic evolution of myeloproliferative neoplasms (MPNs) and myelodysplastic syndromes (MDSs)], and the same occurs for lymphoproliferative disorders [(LPDs), i.e., chronic lymphocytic leukemia, (CLL), which may evolve to aggressive non-Hodgkin lymphomas (NHLs)]
We evaluated clinical features at first diagnosis, including demographics, performance status according to the Eastern Cooperative Oncology Group scale (ECOG), hematological parameters, cytogenetic abnormalities, and the presence of driver mutations (JAK2V617F, CALR, and MPL) for MPN cases
The two diagnoses were concomitant in 32% of patients, while myeloid disease preceded lymphoproliferative diseases (LPDs) in 52% of cases after a median time of 37 months from myeloid disease, and LPD preceded myeloid neoplasm in 16% after a median time of 41 months
Summary
The potential for myeloid neoplasms to evolve one into each other is largely known [i.e., leukemic evolution of myeloproliferative neoplasms (MPNs) and myelodysplastic syndromes (MDSs)], and the same occurs for lymphoproliferative disorders [(LPDs), i.e., chronic lymphocytic leukemia, (CLL), which may evolve to aggressive non-Hodgkin lymphomas (NHLs)]. CLL patients are known to be at higher risk for secondary neoplasms, mostly cutaneous ones [2]; hematological neoplasms are rarely observed. Irrespective of the former neoplasm (either myeloid or lymphoid), important concerns have been raised about the possible causal effect of hematological treatments on the development of second tumors, and it is still an unanswered question. Little is known about the clinical characteristics of patients with co-occurrence of myeloid and lymphoid neoplasms, and their outcome in terms of infectious and thrombotic complications, and survival. We evaluated clinical features at first diagnosis, including demographics, performance status according to the Eastern Cooperative Oncology Group scale (ECOG), hematological parameters, cytogenetic abnormalities, and the presence of driver mutations (JAK2V617F, CALR, and MPL) for MPN cases.
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