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CLASSIFICATION. Monoclonal gammopathy of uncertain significance (MGUS) Idiopathic Associated with other diseases (autoimmune, infectious, non-heme cancer, etc) Plasma cell or lymphoid malignancy Waldenstrom's macroglobulinemia Other lymphoproliferative disorders Smoldering multiple myeloma

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plasma cell dyscrasias

CLASSIFICATION

Monoclonal gammopathy of uncertain significance (MGUS)

Idiopathic

Associated with other diseases (autoimmune, infectious, non-heme cancer, etc)

Plasma cell or lymphoid malignancy

Waldenstrom's macroglobulinemia

Other lymphoproliferative disorders

Smoldering multiple myeloma

Multiple myeloma

PLASMA CELL DYSCRASIAS
multiple myeloma

Definition

Increased numbers of abnormal or immature plasma cells in the bone marrow, or localized plasmacytoma

Monoclonal protein in blood or urine

Some patients are nonsecretors

Lytic bone lesions

Not all patients have lytic bone disease

MULTIPLE MYELOMA
multiple myeloma1

EPIDEMIOLOGY

Incidence in US approx 3 cases/100,000/yr

98% of cases > 40 yrs old

Risk factors:

genetic

radiation exposure

? chemical exposure

MULTIPLE MYELOMA
slide5

MULTIPLE MYELOMA

Serum and urine protein electrophoresis

Monoclonal IgG

Free light chain

Serum

Urine

multiple myeloma igg kappa
Multiple myeloma (IgG kappa)

IgG 2080

Anemia, leukopenia

Lytic bone lesions

10% marrow plasma cells

multiple myeloma iga
Multiple myeloma (IgA)

IgA 1010 (nl 70-140)

IgG 165 (nl 695-2190)

IgM 14 (nl 60-265)

Anemia, thrombocytopenia

16% marrow plasma cells

slide8
MGUS

Ifix: monoclonal IgG kappa

No proteinuria

IgA 234 (50-540)

IgG 1840 (600-1600)

IgM 85 (40-250)

Normal CBC

waldenstrom macroglobulinemia with hyperviscosity syndrome
Waldenstrom macroglobulinemia with hyperviscosity syndrome

Anemia, fatigue, retinopathy, bleeding disorder

IgA 125 (70-440)

IgG 724 (695-2190)

IgM 5130 (60-265)

Serum viscosity 3.6

Marrow: <5% plasmacytoid B-lymphocytes

waldenstrom macroglobulinemia
Waldenstrom macroglobulinemia

Mild fatigue

IgM 8460, serum viscosity 2.7

Hgb 12, WBC 3700, platelets 143,000

Marrow: 50% plasmacytoid B-lymphocytes

kappa light chain myeloma with cardiac amyloidosis
Kappa light chain myeloma with cardiac amyloidosis

Serum (hypogammaglobulinemia)

Urine (kappa light chain)

Always check the urine!

myeloma cytogenetics
MYELOMA CYTOGENETICS

t(4;14)

del (13)

Lancet 2004;363:875

multiple myeloma2

DIAGNOSIS

Monoclonal paraprotein

Intact immunoglobulin in serum (SPEP)

Light chains in serum or urine (IEP)

Marrow plasmacytosis (> 20% - may be patchy)

Lytic bone lesions

Plasmacytoma

MULTIPLE MYELOMA
multiple myeloma3

CLINICAL MANIFESTATIONS

Anemia progressing to pancytopenia

Bone pain/destruction

Hypercalcemia

Humoral immune defect

Local effects of plasmacytomas

Systemic effects of paraprotein

MULTIPLE MYELOMA
monoclonal gammopathy

COMPLICATIONS

Hyperviscosity (IgM >> IgA > IgG)

Renal dysfunction (light chains)

Glomerular

Tubular

Neuropathy

Other organ damage (rare)

Cryoglobulinemia (types I and II)

Amyloidosis

MONOCLONAL GAMMOPATHY
slide17

MULTIPLE MYELOMA

Cast nephropathy

slide18

Brit J Haematol 2004; 124:565

CRYOGLOBULINEMIA IN IgM GAMMOPATHY

multiple myeloma5
MULTIPLE MYELOMA

Presenting features (1027 pts)

Mayo Clin Proc 2003;78:21

Bone pain

58%

Fatigue

32%

Weight loss

24%

Paresthesias

5%

Fever

0.7%

ECOG PS >2

10%

multiple myeloma6
MULTIPLE MYELOMA

Preexisting conditions and risk factors

Mayo Clin Proc 2003;78:21

Cancer in 1st degree relative

42%

Myeloma in 1st degree relative

2%

Hx of plasma cell proliferative disorder

34%

multiple myeloma7

Mayo Clin Proc 2003;78:21

MULTIPLE MYELOMA

Pre-existing plasma cell disorders

multiple myeloma8
MULTIPLE MYELOMA

Physical findings

Mayo Clin Proc 2003;78:21

Palpable liver

4%

Palpable spleen

1%

Lymphadenopathy

1%

multiple myeloma9
MULTIPLE MYELOMA

Hematologic findings at presentation

Mayo Clin Proc 2003;78:21

Hgb < 12

73%

Hgb < 8

7%

ESR > 20

84%

ESR > 100

33%

WBC < 4000

20%

WBC < 2000

1%

Plts < 100K

5%

Plts > 500K

2%

multiple myeloma11
MULTIPLE MYELOMA

Serum and urine proteins

Mayo Clin Proc 2003;78:21

Monoclonal band on SPEP in 82%

3% non-secretory at presentation

multiple myeloma12
MULTIPLE MYELOMA

Types of monoclonal proteins

Mayo Clin Proc 2003;78:21

multiple myeloma13
MULTIPLE MYELOMA

Radiographic findings at presentation

Mayo Clin Proc 2003;78:21

multiple myeloma14
MULTIPLE MYELOMA

Survival

Mayo Clin Proc 2003;78:21

multiple myeloma15
MULTIPLE MYELOMA

Prognostic factors

Mayo Clin Proc 2003;78:21

*

*

*

*

*

* Most important factors in multivariate analysis

adverse cytogenetics in myeloma
Adverse cytogenetics in myeloma

del13 or del13q

t(4;14

del17p

multiple myeloma16

INDICATIONS FOR TREATMENT

Symptomatic disease

Bone destruction

Anemia

Organ dysfunction

Hypercalcemia

Increasing paraprotein or ß2-microglobulin level

MULTIPLE MYELOMA
treatment mnemonic
Calcium increased

Renal dysfunction

Anemia

Bone lesions

Treatment mnemonic
multiple myeloma17
MULTIPLE MYELOMA

TREATMENT

  • Initial treatment:
    • Thalidomide or lenalidomide + dexamethasone
    • Bortezomib + dexamethasone
    • Bortezomib + lenalidomide + dexamethasone
    • Melphalan + prednisone + lenalidomide/thalidomide
  • Autologous SCT prolongs survival
  • Refractory disease:
    • High dose cyclophosphamide
    • Platinum-based regimen
  • Bisphosphonates as adjunctive Rx
  • Allogeneic transplant role?
slide36

Overall and progression-free survival in multiple myeloma: standard chemotherapy vs high-dose chemotherapy with stem cell rescue

NEJM 2003;348:1875