Blood reviews · 2025
Myelofibrosis-associated extramedullary hematopoiesis: Insights into hepatic, pulmonary, and thrombotic complications.
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Amanam Idoroenyi, Otoukesh Salman, Pullarkat Vinod, Ghoda Lucy, Forman Stephen, Marcucci Guido, Al Malki Monzr, Ali Haris
Аннотация
BACKGROUND: Primary myelofibrosis (PMF), the most aggressive of the Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), is characterized by progressive marrow fibrosis, ineffective hematopoiesis, and a pro-inflammatory milieu. These pathobiologic features drive extramedullary hematopoiesis (EMH) and confer heightened risks for hepatic, pulmonary, and thrombotic complications, particularly in patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HCT).
OBJECTIVE: Drawing on our institutional transplant experience in PMF, we review the pathophysiology, clinical manifestations, and management of EMH-related complications to optimize outcomes in this complex patient population.
METHODS: We synthesized current literature on EMH in MPNs and integrated contemporary data on hepatic portal hypertension, pulmonary hypertension, splanchnic vein thrombosis, and transplant-related complications. Key insights from recent European Society for Blood and Marrow Transplantation (EBMT) registry data are highlighted.
RESULTS: Portal hypertension affects 3-18 % of patients with myeloproliferative neoplasms (MPNs), driven by intrahepatic sinusoidal infiltration, splenic hyperdynamic circulation, and splanchnic thrombosis. Splanchnic vein thrombosis often precedes overt MPN diagnosis, with JAK2V617F detected in a substantial proportion of affected patients. Pulmonary complications-including extramedullary hematopoiesis (EMH), pulmonary hypertension, and peri-engraftment respiratory failure-contribute meaningfully to non-relapse mortality in the transplant setting. Ruxolitinib, reduced-intensity conditioning, and spleen-directed strategies have improved engraftment kinetics and mitigated graft-related complications in myelofibrosis patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HCT). Institutional observations are incorporated throughout to contextualize hepatic, pulmonary, and vascular manifestations of EMH in contemporary practice.
CONCLUSION: These institutional observations complement published data and emphasize that EMH-related hepatic, pulmonary, and vascular complications are biologically active yet potentially reversible when clonal disease control is achieved. EMH-associated complications in PMF require a multidisciplinary management approach tailored to disease biology and transplant considerations. Advances in cytokine modulation, conditioning strategies, and emerging antifibrotic agents hold promise for improving patient outcomes.
OBJECTIVE: Drawing on our institutional transplant experience in PMF, we review the pathophysiology, clinical manifestations, and management of EMH-related complications to optimize outcomes in this complex patient population.
METHODS: We synthesized current literature on EMH in MPNs and integrated contemporary data on hepatic portal hypertension, pulmonary hypertension, splanchnic vein thrombosis, and transplant-related complications. Key insights from recent European Society for Blood and Marrow Transplantation (EBMT) registry data are highlighted.
RESULTS: Portal hypertension affects 3-18 % of patients with myeloproliferative neoplasms (MPNs), driven by intrahepatic sinusoidal infiltration, splenic hyperdynamic circulation, and splanchnic thrombosis. Splanchnic vein thrombosis often precedes overt MPN diagnosis, with JAK2V617F detected in a substantial proportion of affected patients. Pulmonary complications-including extramedullary hematopoiesis (EMH), pulmonary hypertension, and peri-engraftment respiratory failure-contribute meaningfully to non-relapse mortality in the transplant setting. Ruxolitinib, reduced-intensity conditioning, and spleen-directed strategies have improved engraftment kinetics and mitigated graft-related complications in myelofibrosis patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HCT). Institutional observations are incorporated throughout to contextualize hepatic, pulmonary, and vascular manifestations of EMH in contemporary practice.
CONCLUSION: These institutional observations complement published data and emphasize that EMH-related hepatic, pulmonary, and vascular complications are biologically active yet potentially reversible when clonal disease control is achieved. EMH-associated complications in PMF require a multidisciplinary management approach tailored to disease biology and transplant considerations. Advances in cytokine modulation, conditioning strategies, and emerging antifibrotic agents hold promise for improving patient outcomes.