Primary myelofibrosis: current therapeutic options

Campos, Paula de Melo

doi:10.1016/j.bjhh.2016.04.003

Cited by 11 publications

(9 citation statements)

References 76 publications

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“…The patient has no JAK2 (Janus kinase 2, V617 F), MPL (thrombopoietin receptor, W515 K/L) and CALR (calreticulin, exon 9 indel) mutation which are identified in approximately 90% of patients to help with the diagnosis and the prognostic stratification of Primary myelofibrosis (PMF) patients [ 9 , 10 ]. This means that the cases had no basis of genetic mutations for PMF.…”

Section: Discussionmentioning

confidence: 99%

Could cytokine release syndrome induce acute myelofibrosis in CD19 chimeric antigen receptor T cells therapy?

et al. 2020

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CART cells therapy can give rise to most common and concerning two side effects-cytokine release syndrome (CRS) and neurotoxicity. But in our CD19 CART cells therapy clinical trial, we observed 1 out of 17 patients with B-cell acute lymphoblastic leukemia (B-ALL) developed acute myelofibrosis(AMF) after grade IV CRS post to the CD19 CART cells therapy. This finding suggests that the CART cells therapy may have rare and serious AMF, which we should pay important attention to. Trial registration:NCT02968472. Registered 18 November 2016-Retrospectively registered, https:

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Section: Discussionmentioning

confidence: 99%

Could cytokine release syndrome induce acute myelofibrosis in CD19 chimeric antigen receptor T cells therapy?

et al. 2020

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“…[56] In patients with transfusiondependent anaemia and iron overload, iron chelation therapy is the main approach to prevent iron-induced organ damage. [35,54] Splenectomy may exceptionally be indicated for palliative control of persistent anaemia. [54] It improves anaemia and thrombocytopaenia by allowing for sequestered blood cells to enter the peripheral circulation.…”

Section: Treatment Of Pmf-associated Anaemiamentioning

confidence: 99%

“…[35,54] Splenectomy may exceptionally be indicated for palliative control of persistent anaemia. [54] It improves anaemia and thrombocytopaenia by allowing for sequestered blood cells to enter the peripheral circulation. [40] However, splenectomy is associated with significant morbidity and mortality, with the most common complications being bleeding, infection and thrombosis.…”

Section: Treatment Of Pmf-associated Anaemiamentioning

confidence: 99%

“…Splenic irradiation is a well‐recognised alternative to splenectomy in patients with PMF who have symptomatic splenomegaly and are ineligible for surgical procedures. [54] Although radiation dosages vary widely, the most commonly administered dose for splenomegaly is reportedly 10 Gy in 10 fractions over 2 weeks. [55] Splenic irradiation is thought to have a role in reducing the number of neoplastic cells in the spleen, leading to improvements in both splenic size and discomfort.…”

Section: Treatment Of Pmfmentioning

confidence: 99%

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Advances in management of primary myelofibrosis and polycythaemia vera: Implications in clinical practice

Amé

Barraco

Ianotto

et al. 2023

eJHaem

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Primary myelofibrosis (PMF) and polycythaemia vera (PV) are rare BCR‐ABL1‐negative myeloproliferative neoplasms, associated with an increased risk of thrombosis, haemorrhagic complications and progression to fibrosis or leukaemia or fibrosis for PV. Both diseases are characterised by biological and clinical heterogeneity, leading to great variability in their management in routine clinical practice. In this review, we present an updated overview of the diagnosis, prognosis and treatment of PMF and PV, and we discuss how our multidisciplinary expert group based across France translates this evidence‐based knowledge into routine clinical practice.

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“…Also, a 10% WL after allogenic HSCT (alloHSCT) was associated with worse clinical outcome [5]. This evidence demonstrates the importance of a comprehensive nutritional assessment, including measurement of dry weight, which is necessary for monitoring disease progression and to allow effective planning of nutritional interventions [6].…”

Section: Introductionmentioning

confidence: 99%

Phase Angle and Dry Weight Benefit Nutritional Management of a Patient with Primary Myelofibrosis Undergoing Allogeneic Hematopoietic Stem Cell Transplantation

Novelli

Fatobene²,

Silva

et al. 2019

Case Rep Clin Nutr

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Background: Progressive, involuntary weight loss (WL) is common in patients with primary myelofibrosis (PMF), and the etiology of this manifestation is multifactorial. A triad of ascites, edema, and splenomegaly is common; therefore, the body weight may be overestimated due to fluid overload. A comprehensive nutritional assessment, including measurement of dry weight, is necessary for monitoring disease progression and to allow the effective planning of nutritional interventions. Also, the phase angle (PA) has been suggested as an important prognostic and muscle mass marker in patients with cancer. To date, there have been no reports of bioimpedance analysis (BIA) of patients with PMF. Case Presentation: A 56-year-old man with PMF underwent allogeneic hematopoietic stem cell transplantation (HSCT) with fatigue and postprandial abdominal fullness, exhibiting splenomegaly (> 10 cm) and bilateral lower-extremity edema. On day –13, a BIA was performed. The patient had a body weight of 73.5 kg, with a fluid balance of +9.2 L and a PA at 50 kHz of 3.15°. A nutritional intervention was administered for 26 days, and on day +13 of HSCT, a repeat BIA revealed an estimated body weight of 69.7 kg with a fluid balance of +5.8 L and a PA at 50 kHz of 3.17°, as well as nonsignificant WL (3.1% in 1 month). Conclusions: This case report is an important addition to the nutritional assessment of PFM patients, since we could provide a better nutritional intervention due to our understanding of the estimated dry weight and assess the PA to investigate a new element of prognosis. Additional studies are needed to demonstrate the effectiveness of BIA in these patients.

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Primary myelofibrosis: current therapeutic options

Cited by 11 publications

References 76 publications

Could cytokine release syndrome induce acute myelofibrosis in CD19 chimeric antigen receptor T cells therapy?

Could cytokine release syndrome induce acute myelofibrosis in CD19 chimeric antigen receptor T cells therapy?

Advances in management of primary myelofibrosis and polycythaemia vera: Implications in clinical practice

Phase Angle and Dry Weight Benefit Nutritional Management of a Patient with Primary Myelofibrosis Undergoing Allogeneic Hematopoietic Stem Cell Transplantation

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