Low‐dose, single‐fraction, whole‐lung radiotherapy for pulmonary hypertension associated with myelofibrosis with myeloid metaplasia

Steensma, David P.; Hook, C. Christopher; Stafford, Scott L.; Tefferi, Ayalew

doi:10.1046/j.1365-2141.2002.03695.x

Cited by 85 publications

(54 citation statements)

References 10 publications

(20 reference statements)

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“…Platelets and megakaryocytes contain serotonin in their granules and its release in the pulmonary circulation might contribute to the origin of this serious complication in these patients. Pulmonary hypertension resulting from diffuse pulmonary EMH responds to whole lung irradiation with a single dose of 100 cGy (78).…”

Section: Treatment For Extramedullary Hematopoiesismentioning

confidence: 99%

Myelofibrosis with Myeloid Metaplasia: New Developments in Pathogenesis and Treatment

2004

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Myeloid metaplasia with myelofibrosis (MMM) is a chronic myeloproliferative disorder (CMPD) characterized by progressive anemia, massive splenomegaly, both hepatosplenic and non-hepatosplenic extramedullary hematopoiesis (EMH), a leukoerythroblastic blood smear, circulating progenitor cells, and marked bone marrow stromal reaction including collagen fibrosis, osteosclerosis and angiogenesis. The overall median survival is 5 years although it might range from 2 to 15 years depending on the presence or absence of clinically defined prognostic factors. Death is often due to leukemic transformation, portal hypertension or infection. In addition to shortened survival, quality of life is often affected by frequent red blood cell transfusions, profound constitutional symptoms, and cachexia. Drug therapy and autologous hematopoietic stem cell transplantation (HSCT) are of only palliative value and have not been shown to improve survival. The role of allogeneic HSCT, both myeloablative and non-myeloablative, is actively being investigated. Both splenectomy and radiation therapy have defined therapeutic roles to control EMH-associated symptoms. Analysis of the molecular biology of the disease is underway with the aid of animal models leading to the identification of novel therapeutic targets. Among the novel agents tested, thalidomide seems the most promising although newer agents are on the horizon.

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Section: Treatment For Extramedullary Hematopoiesismentioning

confidence: 99%

Myelofibrosis with Myeloid Metaplasia: New Developments in Pathogenesis and Treatment

2004

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“…External beam radiotherapy is also effective for the involvement of vital organs, including the lung, central nervous system and liver (Price & Bell, 1985;Landolfi et al, 1988;Tefferi et al, 2001b;Steensma et al, 2002). Recently, low-dose radiation has also been shown to be an effective therapy for severe extremity pain (Neben-Wittich et al, 2010).…”

Section: Radiotherapymentioning

confidence: 99%

Guideline for the diagnosis and management of myelofibrosis

et al. 2012

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SummaryThe guideline group regarding the diagnosis and management of myelofibrosis was selected to be representative of UK-based medical experts, together with a contribution from a single expert from the USA. MEDLINE and EMBASE were searched systematically for publications in English from 1966 until August 2011 using a variety of key words. The writing group produced the draft guideline, which was subsequently revised by consensus of the members of the General Haematology and Haemato-oncology Task Forces of the British Committee for Standards in Haematology (BCSH). The guideline was then reviewed by a sounding board of UK haematologists, the BCSH and the British Society for Haematology Committee and comments incorporated where appropriate. The criteria used to state levels and grades of evidence are as outlined in the Procedure for Guidelines commissioned by the BCSH; the 'GRADE' system was used to score strength and quality of evidence. The objective of this guideline is to provide healthcare professionals with clear guidance on the investigation and management of primary myelofibrosis, as well as post-polycythaemic myelofibrosis (post-PV MF) and post-thrombocythemic myelofibrosis (post-ET MF) in both adult and paediatric patients.

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“…Low-dose radiation therapy is the therapy of choice for symptomatic foci of extramedullary haemopoiesis in places such as the spinal cord, peritoneum or pleura, granulocytic sarcomas of the bone causing local pain, and pulmonary hypertension secondary to diffuse myeloid metaplasia of the lung (Price & Bell, 1985;Leinweber et al, 1991;Barlett et al, 1995;Steensma et al, 2002a). Low-dose radiation has also been given as treatment for symptomatic hepatomegaly ).…”

Section: Radiation Therapymentioning

confidence: 99%

Modern management of myelofibrosis

Cervantes

2005

Br J Haematol

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SummaryThe conventional treatment of myelofibrosis involves a wait‐and‐see approach for asymptomatic patients, oral chemotherapy for the hyperproliferative forms of the disease, androgens or erythropoietin for the anaemia, and splenectomy in selected patients. Low‐dose thalidomide plus prednisone is a well‐tolerated therapy for the anaemia and the thrombocytopenia of myelofibrosis, whereas imatinib has shown little efficacy. Allogeneic stem cell transplantation (allo‐SCT) is the only curative therapy for myelofibrosis. Its standard modality has an associated mortality of about 30% and can be applied to younger patients with high‐risk disease or resistant to conventional treatment. Reduced‐intensity conditioning allo‐SCT involves a low mortality and is a promising therapy for patients aged 45–70 years old with the above characteristics. Autologous SCT is a palliative therapy for patients resistant to conventional treatment who lack a suitable donor. The next candidates for the treatment of myelofibrosis are the thalidomide derivatives, the proteasome inhibitors, and vascular endothelial growth factor neutralizing antibodies.

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Low‐dose, single‐fraction, whole‐lung radiotherapy for pulmonary hypertension associated with myelofibrosis with myeloid metaplasia

Cited by 85 publications

References 10 publications

Myelofibrosis with Myeloid Metaplasia: New Developments in Pathogenesis and Treatment

Myelofibrosis with Myeloid Metaplasia: New Developments in Pathogenesis and Treatment

Guideline for the diagnosis and management of myelofibrosis

Modern management of myelofibrosis

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