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Blood, Vol. 96 No. 3 (August 1), 2000:
pp. 852-858
Prognostic factors in Waldenström macroglobulinemia: a
report on 232 patients with the description of a new scoring system
and its validation on 253 other patients
Pierre Morel,
Mathieu Monconduit,
Dominique Jacomy,
Pascal Lenain,
Bernard Grosbois,
Christine Bateli,
Thierry Facon,
Isabelle Dervite,
Francis Bauters,
Albert Najman,
Aimery de Gramont, and
Eric Wattel
From the Service d'Hématologie Clinique, Hôpital
Schaffner, Lens; the Service d'Hématologie, Centre Henri
Becquerel, Rouen; the Service de Médecine Interne, Hôpital
Sud, Centre Hospitalier Régional et Universitaire, Rennes; the
Service des Maladies du Sang, Hôpital Huriez, Centre Hospitalier
Régional et Universitaire, Lille; the Service des Maladies du
Sang, the Service de Médecine Interne Oncologie, Hôpital
Saint Antoine, Paris, and the Service d'Hématologie,
Hôpital Edouard Herriot, Lyon, France.
Using Cox models, we established a new prognostic system based on
simple clinical parameters in a training series of 232 patients whose
diagnoses were made before 1989. Adverse prognostic factors for
survival (P < .01) were age 65 years or older, male gender, albumin level lower than 40 g/L, hemoglobin level lower than 12 g/dL,
platelet count less than 150 × 109/L, white blood cell
count less than 4 × 109/L, high number of cytopenias,
and hepatomegaly. Taking age (age 65 years or older, 1 point; younger
than 65 years, 0 points), albumin (less than 40 g/L, 1 point; 40 g/L or
more, 0 points), and total number of cytopenias (no cytopenia, 0 points; 1 cytopenia, 1 point; 2 or 3 cytopenias, 2 points) into
account, we separated the 232 patients into 3 groups with low (score 0 or 1), intermediate (score 2), or high (score 3 or 4) risk, associated
with 5-year survival rates at 87%, 62%, and 25%, respectively
(P < .0001). Only the presence of 2 or 3 cytopenias was an
independent prognostic factor among patients younger than 65 years
(P < .0001). Albumin level lower than 40 g/L and the
presence of 1 or more cytopenia defined a prognostic system for
patients 65 years and older. Patients at low risk, intermediate risk,
and high risk had 5-year survival rates at 92%, 63%, and 27%,
respectively (P < .0001). The 3 prognostic systems
separated the 167 patients of a test series in groups with
significantly different survival rates. The overall scoring system
retained a significant prognostic value in 86 additional patients
treated between 1990 and 1996. We conclude that the combination of age,
albumin level, and blood cell counts might help to select patients with
Waldenström macroglobulinemia for treatment and to evaluate
therapeutic results.

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