Myelofibrosis in Young Adults

Shalev, Oded; Goldfarb, Ada; Ariel, Ilana; Rachmilewitz, Eliezer A.

doi:10.1159/000206800

Acta Haematol

1983

DOI: 10.1159/000206800

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Myelofibrosis in Young Adults

Oded Shalev¹,

Ada Goldfarb²,

Ilana Ariel

et al.

Abstract: 3 young adults with myelofibrosis, a rare disease in this age-group, are presented. Their clinical course was distinguished by a virtually asymptomatic state at time of diagnosis and a stable, or very slowly progressive course over a 4- to 5-year follow-up. Myelofibrosis in young adults may pursue a more benign course than that usually noted in older poulations.

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Cited by 2 publications

References 8 publications

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Idiopathic myelofibrosis — an update with particular reference to clinical aspects and prognosis

Hasselbalch

1993

Int J Clin Lab Res

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Idiopathic myelofibrosis (IMF) is characterized by excessive accumulation of connective tissue in the bone marrow as part of a clinical syndrome which in its classical form is featured by leukoerythroblastic anemia and huge splenomegaly at the time of diagnosis. An acute variant of the disease exists being featured by pancytopenia, nor or minimal splenomegaly and a rapidly fatal clinical course. This review describes the relationship of IMF to other chronic myeloproliferative disorders and highlights current concepts of the pathogenesis of bone marrow fibrosis, implicating the intramedullary release of various growth factors, including platelet-derived growth factor beta. In a subgroup of patients bone marrow fibrosis may develop consequent to autoimmune bone marrow damage. The clinical and laboratory findings in some of the larger series of patients are presented and the reasons for the highly variable clinical presentation and prognosis are critically discussed. It is proposed that studies on prognosis in IMF are based upon simple prognostic staging systems, which should include the Hb-concentration, platelet count, spleen size and the presence/absence of osteomyelosclerosis on X-ray. Using these parameters the patients are easily categorized into three prognostic groups with highly different survival times.

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Idiopathic myelofibrosis — an update with particular reference to clinical aspects and prognosis

Hasselbalch

1993

Int J Clin Lab Res

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Young patients with myelofibrosis have distinct clinicomolecular features, favorable prognosis, and commonly exhibit inflammatory comorbidities

Horvat,

Abdallah,

Xie

et al. 2023

Ann Hematol

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Myelofibrosis in Young Adults

Cited by 2 publications

References 8 publications

Idiopathic myelofibrosis — an update with particular reference to clinical aspects and prognosis

Idiopathic myelofibrosis — an update with particular reference to clinical aspects and prognosis

Young patients with myelofibrosis have distinct clinicomolecular features, favorable prognosis, and commonly exhibit inflammatory comorbidities

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