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Click here to download a biography of Dr. Jan Gosta Waldenstrom
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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 most recent conference in August, 2014 in London, UK.

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 al100

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 al102

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.

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