waldenstrom macroglobulinemia

Waldenstrom of Macroglobulinemia



Abstract
The second international Workshop on the Waldenstrom of Macroglobulinemia (MW) has defined the criteria for the diagnosis of this hemopathy. They associate:
The presence of an IgM-type serum monoclonal protein, irrespective of its concentration;

Medullary infiltration by lymphoid and/or lymphoplasmocytaires cells;

Tumor infiltration of the intertrabéculaire type;

Tumor cells with a very variable expression profile but often: SIgM +, CD19 +,

CD20 +, CD22 +, CD25 +, CD27 +, FMC7 +, CD5 +/−, CD10 −, CD23 −, CD103 −. The variable expression of CD5, CD23, and even CD10 characterizes MW, but this diagnosis should be retained if the diagnoses of chronic lymphoid leukemia (LLC) or mantle cell lymphoma (MCL) were excluded (1.2).

Survival of cancer patients in France
Waldenstrom of Macroglobulinemia
Authors: X. Troussard, P.M. Carli, M. Maynadié, N. M.
Description of the location studied
Definition in the different classifications:
ICD-O-2: Localization C 42.0
Morphology: M9761; Tumor behaviour/3
ICD-O-1 correspondence: 169.0
Morphology: M9960; Tumor Behavior/1
Correspondence in ICD-10: C 88.0
The second international Workshop on the Waldenstrom of Macroglobulinemia (MW) defined
The criteria for the diagnosis of this hemopathy. They associate:
-The presence of an IgM-type serum monoclonal protein, irrespective of its concentration;
-Medullary infiltration by lymphoid and/or lymphoplasmocytaires cells;
-Tumor infiltration of type intertrabéculaire;
-Tumor cells with a very variable expression profile but often: SIgM +, CD19 +,
CD20 +, CD22 +, CD25 +, CD27 +, FMC7 +, CD5 +/–, CD10 –, CD23 –, CD103 –. The expression
CD5, CD23 and even the CD10 characterizes the MW, but this diagnosis must be
Retained if diagnoses of chronic lymphoid leukemia (LLC) or cell lymphoma of the
Coat (MCL) were excluded (1, 2).

Frequency
Epidemiological studies of MW are limited. Indeed, this hemopathy was
Classified either in non-Hodgkin malignant lymphomas (LMNH) or in syndromes
Lymphoproliferative chronic, especially in the LLC especially in the forms leukemic
disease, or in immunoprolifératives diseases. However, it should be
To identify this entity because of its clinical, biological and evolutionary characteristics. It
National level and the only data available are those of the
Registries specialized in hematology. In the Côte-d'or department, the incidence of
Annual between 1980 and 1997 of this disease was 0.5. As indicated in the
Tables 6 and 8 of the previous chapter, this analysis was carried out on 380 cases, of which 158 were
Deaths in the five years following diagnosis.Raw survival and relative survival
Gross survival was 87% at 1 year, 71% at 3 years and 57% at 5 years. Overall relative survival
was 94%, 84% and 75% respectively (table 1). The excess mortality rate was
Stable over time (Figure 3).

Variation by Sex and age
Relative survival was not different by sex, for this location; At 5 years of age, it was 76%
In women and 74% in men (table 1 and Figure 1).
Results: Waldenstrom de macroglobulinemia
388
Survival of cancer patients in France
On the other hand, differences appeared depending on the age of the diagnosis. Relative survival
At 5 years went from 86% for the age group 45-54 years to 60% for subjects of 75 years and
(table 2 and Figure 2).
The decrease in age-dependent survival was observed regardless of gender (table 4).
Thus, the 5-year survivals went from 88% for the 15-54 years to 66% for the more than
75 years in women and 85% to 53% for the same age groups in men. The Low
The number of cases entering this analysis did not allow for multivariate analysis.

Variation by period
Survival varied little depending on the period of diagnosis. It was 80% to 5 years, for
The period 1989-1991, 73% for the period 1992-1994 and 74% for the period 1995-
1997. This small variation was also observed at 1 year and 3 years. Given the low
Number of people, no multivariate analysis could be carried out to study this evolution.

Variation by department
The small number of cases did not allow these comparisons to be made.
Comparison with the EUROCARE study
The MW has been very rarely individualized in epidemiological studies and, because of the
Groupings operated by the authors, there is no reliable data on this
Disease. Only specific clinical series are available.

Comments
The gross survival was 57% at 5 years and the overall relative survival was 75%. Patient survival
With an MW was therefore lower than that observed in patients with an LLC (relative survival
81% and relative survival of 63%). Even if it is considered a hemopathy
of good relative prognosis, the medians of survival vary according to the studies between 5 and 10 years. These
Variations illustrate the variability of the inclusion criteria in the different studies but also
The variability of therapeutic indications. Indeed, the existence of demonstrations related to the infiltration
Tumor (adenopathies, hepatomegaly, splenomegaly, general signs or Cytopenia)
and/or signs related to the presence of circulating IgM (hyperviscosity, Agglutinins
Cold, Cryoglobulin, neuropathy or amyloidosis) should induce treatment of the patient. On the other hand,
Asymptomatic Forms (Serum IgM greater than 30g/L with medullary infiltration
Can remain stable for many years and do not justify
Treatment.

The bad prognosis factors evaluated on the clinical series are a high age, the presence
Anemia or thrombocytopenia and finally an increased beta-2-microglobulin (2).
In our study, survival relative to 5 years was 86% for the age class 45-54 years but
Only 60% for patients aged 75.
The factors involved in the progression of asymptomatic IgM peaks are also
Still pretty poorly identified. In a recent study carried out in patients with an IgM peak,
Survival was 95% at 5 years and 85% at 10 years and the probability of transformation was assessed
to 8% to 5 years and to 29% to 10 years. Multivariate analysis has shown that the transformation
was dependent on the size of the IgM peak, the presence of a blood lymphocytosis and finally
Hemoglobin (3). These parameters are also found in a study conducted by