Idiopathic myelofibrosis: A clinical study of 80 patients

Abstract

A series of 122 consecutive patients with bone marrow fibrosis initially referred or categorized as idiopathic myelofibrosis is described. After a clinical and pathological review 14 patients were classified as postpolycythaemic myelofibrosis and 7 patients as a transitional myeloproliferative disorder. In 13 patients a diagnosis of hairy cell leukaemia was made, 3 patients had malignant lymphoma, 2 had malignant histiocytosis, and 1 patient had systemic lupus erythematosus with myelofibrosis. Two patients were excluded for further analysis owing to insufficient data. In the remaining 80 patients a diagnosis of idiopathic myelofibrosis was made. The clinical and laboratory findings in this series of patients are presented and compared to those in previous series. Infectious, cardiovascular, thromboembolic, and haemorrhagic complications were frequent, being recorded in 63%, 50%, 40%, and 33% of the patients, respectively. Various autoimmune phenomena were found in a proportion of the patients, but none had clinical evidence of connective tissue disease. Fifteen patients (19%) had a syndrome of acute myelofibrosis. The diagnostic criteria for this disease entity and its place within the spectrum of myeloproliferative disorders are discussed. In the present series acute myelofibrosis was found to encompass various transitional stages toward the evolution of acute leukaemia. It is proposed that acute or malignant myelofibrosis is considered as an acute variant of idiopathic myelofibrosis. Within this syndrome the acute variant seems to be far more common than previously recognized, which may also explain the marked clinical heterogeneity of the myelofibrosis/osteomyelosclerosis syndrome in this and most previous series.

Life expectancy and prognostic factors in the classic BCR/ABL-negative myeloproliferative disorders

“…9,[11][12][13] Male sex was associated with worse prognosis at univariate study in the Lille series, but lost significance at multivariate level. 11 The prognostic value of spleen size has been noted only occasionally, 8 and the same applies to liver size. 11 The initial haemoglobin (Hb) concentration is the parameter most consistently associated with the prognosis of PMF, with the 10 g/100 ml value being the cutoff level usually determining a more unfavourable course of the disease.…”

“…Median survival ranges from 4 to 5.5 years in modern series [6][7][8][9][10][11][12][13][14] (Figure 1). When the survival of PMF patients has been compared with that of age-and sexmatched individuals from the general population, a 31% reduction in life expectancy has been observed.…”

“…The recent discovery of the mutation V617F in the JAK2 gene in a high proportion of MPD patients [1][2][3][4] has given biological support to the early notion by Dameshek 5 placing the three disorders within the same group. With regard to the survival, PMF is associated with a substantial reduction in life expectancy, [6][7][8][9][10][11][12][13][14] and ET affects more the patients' quality of life than their survival, whereas PV is associated with both a substantial morbidity and a certain reduction in the patients' life expectancy. [15][16][17][18] Because of this, prognostic studies in the MPDs have been carried out primarily in PMF.…”

Isolated sacral injury in hairy cell leukemia: A case report and analysis of literature data

“…[9,10]. Причины и патогенез поражения костей при ВКЛ пока неиз-вестны, обсуждаются разные факторы, в том числе нарушения регуляции пролиферации фибробластов, синтеза коллагена и факторов роста, ведущие как к лизису, так и к остеосклерозу [15][16][17]. Так, показано, что у пациентов с ВКЛ без признаков очагового поражения костей ускорена резорбция костной ткани [18].…”

Idiopathic myelofibrosis mimicking hemolytic anemia

“…5,10 The anaemia is usually normochromic, normocytic. Megaloblastic anaemia due to folate and/or vitamin B-12 deficiency is rare, whereas iron deficiency due to gastrointestinal blood loss is commonly seen.…”