The following websites contain valuable information regarding the research and treatment of Waldenstrom's Macroglobulinemia:
Please visit our main program website: Bing Center for Waldenstrom's Research. This site contains information on the latest treatment recommendations, abstracts, news and events, current and pending clinical trials and participating institutions, basic research, and publications, as well as photos and brief bios of the Bing Center staff.
Also feel free to visit our WM Workshop Website where you will find information about our recent conference October 6-10, 2010 in Venice, Italy.
WALDENSTROM’S MACROGLOBULINEMIA/LYMPHOPLASMACYTIC LYMPHOMA (Page 11)
TABLES
Table 1. Clinical and laboratory findings for 149 consecutive
newly diagnosed patients with the consensus panel diagnosis of WM presenting to
the Dana Farber Cancer Institute. NA (not applicable).
|
|
Median |
Range |
Institutional
Normal Reference Range |
|
Age (yr) |
59 |
34-84 |
NA |
|
Gender (Male/Female) |
85/64 |
|
NA |
|
Bone marrow involvement |
30% |
5-95% |
NA |
|
Adenopathy |
16% |
|
NA |
|
Splenomegaly |
10% |
|
NA |
|
IgM (mg/dL) |
2,870 |
267-12,400 |
40-230 |
|
IgG (mg/dL) |
587 |
47-2,770 |
700-1,600 |
|
IgA (mg/dL) |
47 |
8-509 |
70-400 |
|
Serum Viscosity (cp) |
2.0 |
1.4-6.6 |
1.4-1.9 |
|
Hct (%) |
35.0% |
17.2-45.4% |
34.8-43.6 |
|
Plt (x 109/L) |
253 |
24-649 |
155-410 |
|
Wbc (x 109/L) |
6.0 |
0.3-13 |
3.8-9.2 |
|
B2M (mg/dL) |
3.0 |
1.3-13.7 |
0-2.7 |
|
LDH |
395 |
122-1,131 |
313-618 |
Table 2. Physicochemical and immunological
properties of the monoclonal IgM protein in Waldenstrom’s macroglobulinemia.
|
Properties
of IgM Monoclonal Protein |
Diagnostic
Condition |
Clinical
Manifestations |
|
Pentameric Structure |
Hyperviscosity |
Headaches, blurred vision, epistaxis, retinal hemorrhages,
leg cramps, impaired mentation, intracranial hemorrhage. |
|
Precipitation on cooling |
Cryoglobulinemia (Type I) |
Raynaud’s phenomenom, acrocyanosis, ulcers, purpura, cold
urticaria. |
|
Auto-antibody activity to Myelin Associated Glycoprotein (MAG), Ganglioside M1
(GM1), Sulfatide moieties on peripheral nerve sheaths |
Peripheral neuropathies |
Sensorimotor neuropathies, painful neuropathies, ataxic
gait, bilateral foot drop. |
|
Auto-antibody activity to IgG |
Cryoglobulinemia (Type II) |
Purpura, arthralgias, renal
failure, sensorimotor neuropathies. |
|
Auto-antibody activity to red blood cell antigens |
Cold agglutinins |
Hemolytic anemia, Raynaud’s phenomenom, acrocyanosis,
livedo reticularis. |
|
Tissue deposition as amorphous aggregates |
Organ Dysfunction |
Skin: bullous skin disease, papules, Schnitzler’s
syndrome. GI: diarrhea, malabsorption, bleeding. Kidney: proteinuria, renal failure (light chain
component). |
|
Tissue deposition as amyloid fibrils (light chain component most commonly) |
Organ Dysfunction |
Fatigue, weight loss, edema, hepatomegaly, macroglossia,
organ dysfunction of involved organs: heart, kidney, liver, peripheral
sensory and autonomic nerves. |
Table 3.
Prognostic scoring systems in Waldenstrom’s macroglobulinemia.
|
Study |
Adverse
prognostic factors |
Number
of groups |
Survival |
|
Gobbi et al98 |
Hb < 9 g/dL Age >70 yr Weight loss Cryoglobulinemia |
0-1 prognostic
factors 2-4 prognostic factors |
Median: 48 mo Median: 80 mo |
|
Morel et al99 |
Age > 65 yr Albumin < 4 g/dL Number of cytopenias: Hb <12 g/dL Platelets <150 x 109/L Wbc < 4x109/L |
0-1
prognostic factors 2 prognostic factors 3-4 prognostic factors |
5 yr: 87% 5 yr: 62% 5 yr: 25% |
|
Dhodapkar et al |
β2M >3 g/dL Hb <12 g/dL IgM <4 g/dL |
β2M < 3 mg/dL + Hb > 12 g/dL β2M < 3 mg/dL + Hb < 12 g/dL β2M > 3 mg/dL + IgM > 4 g/dL β2M > 3 mg/dL + IgM < 4 g/dL |
5 yr: 87% 5 yr: 63% 5 yr: 53% 5 yr: 21% |
|
Application of International Staging System Criteria for Myeloma to WM Dimopoulos et al |
Albumin <3.5 g/dL β2M >3.5 mg/L |
Albumin > 3.5 g/dL + β2M < 3.5
mg/dL Albumin < 3.5 g/dL + β2M < 3.5 or β2M 3.5-5.5 mg/dL β2M > 5.5 mg/dL |
Median: NR Median: 116 mo Median: 54 mo |
|
International Prognostic Scoring System for WM Morel et al104 |
Age > 65 yr Hb <11.5 g/dL Platelets <100 x 109/L β2M > 3 mg/L IgM > 7 g/dL |
0-1
prognostic factors* 2 prognostic factors** 3-5 prognostic factors *excluding age ** or age >65 |
5 yr: 87% 5 yr: 68% 5 yr: 36% |
Table 4. Summary
of Updated Response Criteria from the 3rd International Workshop on
Waldenstrom’s Macroglobulinemia.186
Complete
Response
|
CR |
Disappearance
of monoclonal protein by immunofixation; no histological evidence of bone
marrow involvement, and resolution of any adenopathy / organomegaly
(confirmed by CT scan), along with no signs or symptoms attributable to WM.
Reconfirmation of the CR status is required at least 6 weeks apart with a
second immunofixation. |
|
Partial Response |
PR |
A >50%
reduction of serum monoclonal IgM concentration on protein electrophoresis
and > 50% decrease in adenopathy/organomegaly on physical
examination or on CT scan. No new symptoms or signs of active disease. |
|
Minor Response |
MR |
A >
25% but < 50% reduction of serum monoclonal IgM by protein
electrophoresis. No new symptoms or signs of active disease. |
|
Stable Disease |
SD |
A
<25% reduction and <25% increase of serum monoclonal IgM by
electrophoresis without
progression of adenopathy/organomegaly, cytopenias or clinically significant
symptoms due to disease and/or signs of WM. |
|
Progressive Disease |
PD |
A >25%
increase in serum monoclonal IgM by protein electrophoresis confirmed by a
second measurement or progression of clinically significant findings due to
disease (i.e. anemia, thrombocytopenia, leukopenia, bulky
adenopathy/organomegaly) or symptoms (unexplained recurrent fever >
38.4°C,
drenching night sweats, > 10% body weight loss, or hyperviscosity,
neuropathy, symptomatic cryoglobulinemia or amyloidosis) attributable to WM. |