TY - JOUR
T1 - How I treat symptomatic splenomegaly in patients with myelofibrosis
AU - Mesa, Ruben A.
PY - 2009
Y1 - 2009
N2 - Managing patients with myelofibrosis (MF), either those with primary MF or those whose MF has evolved from antecedent polycythemia vera or essential thrombocythemia, presents many challenges to the hematologist. MF patients have a range of debilitating disease manifestations (eg, massive splenomegaly, cytopenias, constitutional symptoms, and transformation to a treatment-refractory blast phase). Cure is potentially achievable through allogeneic stem cell transplantation; however, this therapy is either inappropriate or not feasible for the majority of patients. Therefore, remaining therapies are palliative but can be of significant value to some MF patients. In particular, management of symptomatic splenomegaly remains one of the most perplexing aspects of MF clinical care. Using medications is the simplest approach for reducing splenomegaly, yet achieving symptomatic response without undue myelosuppression is challenging. Splenectomy or radiotherapy offers benefit, but careful patient selection and close monitoring are required because both have the potential for dangerous adverse effects. Experimental medical therapies, such as JAK2 inhibitors, show promise and may soon play an important role in the management of symptomatic splenomegaly in MF patients. Future care of MF patients, including splenomegaly management, will continue to require the hematologist to select therapeutic options carefully in the context of realistic, achievable goals.
AB - Managing patients with myelofibrosis (MF), either those with primary MF or those whose MF has evolved from antecedent polycythemia vera or essential thrombocythemia, presents many challenges to the hematologist. MF patients have a range of debilitating disease manifestations (eg, massive splenomegaly, cytopenias, constitutional symptoms, and transformation to a treatment-refractory blast phase). Cure is potentially achievable through allogeneic stem cell transplantation; however, this therapy is either inappropriate or not feasible for the majority of patients. Therefore, remaining therapies are palliative but can be of significant value to some MF patients. In particular, management of symptomatic splenomegaly remains one of the most perplexing aspects of MF clinical care. Using medications is the simplest approach for reducing splenomegaly, yet achieving symptomatic response without undue myelosuppression is challenging. Splenectomy or radiotherapy offers benefit, but careful patient selection and close monitoring are required because both have the potential for dangerous adverse effects. Experimental medical therapies, such as JAK2 inhibitors, show promise and may soon play an important role in the management of symptomatic splenomegaly in MF patients. Future care of MF patients, including splenomegaly management, will continue to require the hematologist to select therapeutic options carefully in the context of realistic, achievable goals.
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U2 - 10.1182/blood-2009-02-195974
DO - 10.1182/blood-2009-02-195974
M3 - Article
C2 - 19332765
AN - SCOPUS:67049109249
SN - 0006-4971
VL - 113
SP - 5394
EP - 5400
JO - Blood
JF - Blood
IS - 22
ER -