The very‐long‐term course of polycythaemia: a complement to the previously published data of the Polycythaemia Vera Study Group

Abstract

The very-long-term follow-up of patients initially included in the PVSG protocols provides useful information. The excess risk of cancer after chlorambucil appears to persist for 5 years after stopping this treatment. The risk of leukaemia induced by marrow suppression (32P or chemotherapy) was marked before the 10th year, but low thereafter. Phlebotomy is unacceptable as permanent treatment because of the poor clinical tolerance and the frequency of vascular complications. This treatment is also associated with a risk of early progression towards myelofibrosis with myeloid splenomegaly. In the very long term, 15 years or more after the diagnosis, this complication is the major clinical risk, affecting almost 50% of our patients surviving at this time. The prevention of this type of complication could constitute one of the objectives of future protocols dealing with this disease.

Polycythemia vera treated with pipobroman as single agent: low incidence of secondary leukemia in a cohort of patients observed during 20 years (1971–1991)

“…The cumulative risk of myelofibrosis in our experience is 2%, 4% and 9% at 5, 7 and 10 years, respectively: these data compare favorably with those reported by Najean et al 21 in the very-long-term course of PV patients managed with 32P or phlebotomy.…”

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

“…27 With regard to patients with post-PV myelofibrosis, although their prognosis was claimed to be more unfavourable, 28 this has not been confirmed in a recent study. 29 Prognostic factors and staging systems in PMF The wide variability in the survival of PMF patients has stimulated the interest for the identification of variables for predicting survival.…”

“…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. 7,9,[11][12][13][27][28][29][30]33 In one study, a poor prognostic influence of low reticulocyte counts was also observed. 34 Low leukocyte count was a poor prognostic indicator in the Lille and Mayo 11,30 series, whereas high leukocyte counts had poor prognostic weight in the latter and other studies.…”

“…34 Low leukocyte count was a poor prognostic indicator in the Lille and Mayo 11,30 series, whereas high leukocyte counts had poor prognostic weight in the latter and other studies. 11,13,14,28,30,33 According to Hb level and leukocyte count, Dupriez et al, 11 based on the analysis of the Lille series, proposed a score (the so-called Lille scoring system or Dupriez's score), which was able to identify three distinct prognostic groups. In the low-risk group (Hb 410 g/100 ml and white blood cell count o4 Â 10 9 /l or 430 Â 10 9 /l), patients had a median survival of 93 months, whereas those in the …”

STAT signaling in the pathogenesis and treatment of leukemias

“…Myelodysplastic syndrome (MDS) includes a spectrum of bone marrow disorders characterized by ine ective hematopoiesis and progression from anemia to eventual leukemic transformation (Heaney and Golde, 1999;Verwilghen and Boogaerts, 1987). Likewise, standard therapy has achieved only limited success against chronic myelomonocytic leukemia (CMML) and myeloproliferative disorders such as polycythemia vera and essential thrombocythemia (Iland et al, 1995;Najean et al, 1994). While the overproduction of erythrocytes or platelets can be controlled with marrow suppressive chemotherapy, the underlying disorder is not cured and often becomes refractory to treatment.…”