Jl. Ivanyi et al., PROGNOSTIC-SIGNIFICANCE OF BONE-MARROW RETICULIN FIBERS IN IDIOPATHICMYELOFIBROSIS - EVALUATION OF CLINICOPATHOLOGICAL PARAMETERS IN A SCORING SYSTEM, Haematologia, 26(2), 1994, pp. 75-86
A clinicopathological scoring system was performed for obtaining a bet
ter estimate of prognosis in 50 patients with idiopathic myelofibrosis
(IMF). Laboratory parameters including Hb-level, leukocyte and platel
et counts, percentage of blast cells in peripheral blood, spleen and l
iver size, and a semiquantitative histological grading of reticulin fi
bre content of bone marrow biopsies taken at the time of initial diagn
osis were analysed. Based upon these haematological and histological p
arameters three prognostic groups could be categorized with a signific
antly different survival (low-risk group with 21 patients = 75 months;
medium-risk group with 18 patients = 51 months, and 11 patients in a
high-risk group = 18 months). In an univariate (log rank test) and in
a multivariate regression analysis the Hb-concentration, mild splenome
galy (less than 5 cm) and a higher grade of bone marrow reticulin cont
ent proved to be important prognostic parameters, whilst leukopenia, t
hrombocytopenia and the presence of peripheral blast cells were only o
f prognostic significance within the first 6 months from initial diagn
osis. It was concluded that the increase of reticulin fibre deposition
in bone marrow together with anaemia and mild splenomegaly could be r
esponsible for a progressively worse life-expectancy of high-risk pati
ents with idiopathic myelofibrosis.