TY - JOUR
T1 - Modern management of splenomegaly in patients with myelofibrosis
AU - Tremblay, Douglas
AU - Schwartz, Myron
AU - Bakst, Richard
AU - Patel, Rahul
AU - Schiano, Thomas
AU - Kremyanskaya, Marina
AU - Hoffman, Ronald
AU - Mascarenhas, John
N1 - Publisher Copyright:
© 2020, Springer-Verlag GmbH Germany, part of Springer Nature.
PY - 2020/7/1
Y1 - 2020/7/1
N2 - Myelofibrosis (MF) is a chronic myeloproliferative neoplasm which can lead to massive splenomegaly secondary to extramedullary hematopoiesis. Patients frequently exhibit debilitating symptoms including pain and early satiety, in addition to cellular sequestration causing severe cytopenias. JAK 1/2 inhibitors, such as ruxolitinib and fedratinib, are the mainstay of therapy and produce significant and durable reductions in spleen volume. However, many patients are not eligible for JAK 2 inhibitor therapy or become refractory to treatment over time. Novel therapies are in development that can reduce the degree of splenomegaly for some of these patients. However, splenectomy, splenic irradiation, and partial splenic artery embolization remain valuable therapeutic options in select patients. In this review, we will discuss currently available pharmacologic therapies and describe promising drugs currently in development. We will also delve into the efficacy and safety concerns of splenectomy, splenic irradiation, and partial splenic artery embolization. Finally, we will propose a treatment algorithm to help guide clinicians in the management of symptomatic splenomegaly in patients with MF.
AB - Myelofibrosis (MF) is a chronic myeloproliferative neoplasm which can lead to massive splenomegaly secondary to extramedullary hematopoiesis. Patients frequently exhibit debilitating symptoms including pain and early satiety, in addition to cellular sequestration causing severe cytopenias. JAK 1/2 inhibitors, such as ruxolitinib and fedratinib, are the mainstay of therapy and produce significant and durable reductions in spleen volume. However, many patients are not eligible for JAK 2 inhibitor therapy or become refractory to treatment over time. Novel therapies are in development that can reduce the degree of splenomegaly for some of these patients. However, splenectomy, splenic irradiation, and partial splenic artery embolization remain valuable therapeutic options in select patients. In this review, we will discuss currently available pharmacologic therapies and describe promising drugs currently in development. We will also delve into the efficacy and safety concerns of splenectomy, splenic irradiation, and partial splenic artery embolization. Finally, we will propose a treatment algorithm to help guide clinicians in the management of symptomatic splenomegaly in patients with MF.
KW - JAK inhibitor
KW - Myelofibrosis
KW - Splenectomy
KW - Splenic artery embolization
KW - Splenic irradiation
KW - Splenomegaly
UR - http://www.scopus.com/inward/record.url?scp=85084739553&partnerID=8YFLogxK
U2 - 10.1007/s00277-020-04069-4
DO - 10.1007/s00277-020-04069-4
M3 - Review article
C2 - 32417942
AN - SCOPUS:85084739553
SN - 0939-5555
VL - 99
SP - 1441
EP - 1451
JO - Annals of Hematology
JF - Annals of Hematology
IS - 7
ER -