Non-Pharmacologic Management of Splenomegaly for Patients with Myelofibrosis: Is There Any Role for Splenectomy or Splenic Radiation in 2020?

Sankar, K. Nathan; Pettit, Kristen

doi:10.1007/s11899-020-00598-x

Cited by 6 publications

(7 citation statements)

References 61 publications

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“…Options outside of a clinical trial setting include hydroxyurea, interferon formulations, or JAK inhibitor re-challenge after drug holiday [63]. Surgical splenectomy or splenic irradiation may be beneficial for palliation in specific circumstances [64]. In a series of 223 patients with MF who underwent palliative splenectomy at the Mayo Clinic, relief of mechanical symptoms was reported in all patients with symptomatic splenomegaly (n = 87), and transient improvements in constitutional symptoms, portal hypertension symptoms, and red cell transfusion dependence were described in some patients [65,66].…”

Section: Refractory Splenomegalymentioning

confidence: 99%

Improving symptom burden and quality of life in patients with myelofibrosis: current strategies and future directions

Yoon

Pettit

2021

Expert Review of Hematology

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Introduction: Myelofibrosis (MF) is a complex and aggressive hematologic malignancy resulting from JAK/STAT-driven myeloproliferation and abnormal fibrogenesis. The clinical manifestations are heterogeneous and negatively impact quality of life and survival. JAK inhibitors improve symptoms and splenomegaly to a variable degree in a proportion of patients, but the effects for many patients are insufficient or short-lived.Areas covered: This review examines the constellation of symptoms that befall patients with MF, describes methods to quantify and serially monitor these symptoms, and evaluates pharmacologic and non-pharmacologic interventions for disease-related symptoms. The review also includes a discussion of areas of unmet medical need, and proposes future methods for meeting this need. Expert opinion: The treatment landscape for MF is evolving rapidly. The most effective therapies or combinations of therapies will likely simultaneously impact both the malignant hematopoietic stem cell and mechanisms of aberrant fibrogenesis that drive this disease. The goals of treatment for patients with myelofibrosis should be to improve length and quality of life. Clinical trials must be designed with these goals in mind, with endpoints focused on overall survival and symptom reduction, as opposed to surrogate endpoints such as spleen volume reduction.

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Section: Refractory Splenomegalymentioning

confidence: 99%

Improving symptom burden and quality of life in patients with myelofibrosis: current strategies and future directions

Yoon

Pettit

2021

Expert Review of Hematology

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“…Similar to splenectomy, rates of splenic irradiation have diminished; however, it remains an option for patients with bulky, symptomatic splenomegaly refractory or intolerant to medical therapies and not suitable for splenectomy. 53 Main risks include exacerbation of cytopenias, which can be severe, and temporary transfusion dependence. 54 Optimal dosing and fractionation schedules are ill-defined and often depend upon baseline haematological parameters.…”

Section: Splenic Irradiationmentioning

confidence: 99%

The management of myelofibrosis: A British Society for Haematology Guideline

McLornan,

Psaila,

Ewing

et al. 2023

Br J Haematol

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This document represents an update of the British Society for Haematology guideline on Myelofibrosis first published in 2012 and updated in 2015. 1 These guidelines aim to provide healthcare professionals with clear guidance on stratified management for primary myelofibrosis (PMF), as well as postpolycythaemia myelofibrosis (post-PV MF) and postessential thrombocythaemia myelofibrosis (post-ET MF). A separate BSH guideline covers the diagnosis and prognostic evaluation of myelofibrosis and is published alongside this guideline. I N TRODUC T IONMyelofibrosis (MF) encompasses primary myelofibrosis (PMF), postessential thrombocythaemia (ET) MF and

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“…57 The role of spleen-directed ther apy (ie, sple nec tomy or splenic irra di a tion) in MF is con tro ver sial, but the lack of dis ease-mod i fy ing poten tial and the high risk for mor bid ity and mor tal ity have gen er ally rel egated this approach to the pal li a tive set ting in those with refractory spleno meg aly-related symp toms that do not respond to any other med i cal ther apy. 58 Hypersplenism likely con trib utes to throm bo cy to pe nia in some patients, and tran sient improvements in plate let counts in throm bo cy to pe nic MF have been reported after spleen-directed ther apy, though throm bo cy to penia itself should not be an indi ca tion for this approach.…”

Section: Supportive Care For Cytopenic Mye Lo Fi Bro Sismentioning

confidence: 99%

New approaches to tackle cytopenic myelofibrosis

Reynolds

Pettit

2022

Hematology

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Myelofibrosis (MF) is a clonal hematopoietic stem cell neoplasm characterized by constitutional symptoms, splenomegaly, and risks of marrow failure or leukemic transformation and is universally driven by Jak/STAT pathway activation. Despite sharing this pathogenic feature, MF disease behavior can vary widely. MF can generally be categorized into 2 distinct subgroups based on clinical phenotype: proliferative MF and cytopenic (myelodepletive) MF. Compared to proliferative phenotypes, cytopenic MF is characterized by lower blood counts (specifically anemia and thrombocytopenia), more frequent additional somatic mutations outside the Jak/STAT pathway, and a worse prognosis. Cytopenic MF presents unique therapeutic challenges. The first approved Jak inhibitors, ruxolitinib and fedratinib, can both improve constitutional symptoms and splenomegaly but carry on-target risks of worsening anemia and thrombocytopenia, limiting their use in patients with cytopenic MF. Supportive care measures that aim to improve anemia or thrombocytopenia are often ineffective. Fortunately, new treatment strategies for cytopenic MF are on the horizon. Pacritinib, selective Jak2 inhibitor, was approved in 2022 to treat patients with symptomatic MF and a platelet count lower than 50 × 109/L. Several other Jak inhibitors are in development to extend therapeutic benefits to those with either anemia or thrombocytopenia. While many other novel non–Jak inhibitor therapies are in development for MF, most carry a risk of hematologic toxicities and often exclude patients with baseline thrombocytopenia. As a result, significant unmet needs remain for cytopenic MF. Here, we discuss clinical implications of the cytopenic MF phenotype and present existing and future strategies to tackle this challenging disease.

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Non-Pharmacologic Management of Splenomegaly for Patients with Myelofibrosis: Is There Any Role for Splenectomy or Splenic Radiation in 2020?

Cited by 6 publications

References 61 publications

Improving symptom burden and quality of life in patients with myelofibrosis: current strategies and future directions

Improving symptom burden and quality of life in patients with myelofibrosis: current strategies and future directions

The management of myelofibrosis: A British Society for Haematology Guideline

New approaches to tackle cytopenic myelofibrosis

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