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1746 Part XI: Malignant Lymphoid Diseases Chapter 107: Myeloma 1747

chemotherapy to avoid permanent deficits. Specifically, local radiother- osteopenia, and impending fractures of long bones and pelvis. MRI and
apy using less than 30 Gy is potentially curative, in the presence of a PET-CT are more sensitive than the bone survey, and better capture
solitary plasmacytoma; in patients with systemic disease, the DT-PACE early bone disease, the extent of bone disease, and EMD. Both MRI and
regimen, which combines high-dose dexamethasone pulsing, as part PET-CT findings have important prognostic implications. 209,364 These
of the combination with oral dexamethasone, daily thalidomide, and 4 tests are now being used more frequently specifically in smoldering
days of continuous-infusion cisplatin, doxorubicin, cyclophosphamide, multiple myeloma (SMM) patients. Assessment of the heart by echocar-
and etoposide, can provide effective treatment and should be followed diogram and electrocardiogram is useful in the right clinical context to
by local radiation in the absence of symptom relief and lack of tumor detect cardiac amyloidosis and/or LCDD, and, in selected cases, cardiac
shrinkage. If a singular vertebral collapse is evident without plasmacy- MRI may be helpful to demonstrate myocardial infiltration. Measure-
toma, decompressive laminectomy is the treatment of choice. ment of brain natriuretic peptide and N-terminal prohormone B-type
natriuretic peptide are useful screening tests to detect cardiac dysfunc-
INITIAL EVALUATION OF THE PATIENT tion caused by amyloidosis or LCDD.
WITH MYELOMA STAGING
Multiple attempts have been made to define clinical and laboratory
Minimal evaluation requirements include a complete blood count parameters that have prognostic significance in myeloma. 231,377,378 Of the
with differential white cell count; examination of a blood film for the many staging systems, the Salmon-Durie staging system was histori-
presence of rouleaux and circulating myeloma cells; a comprehensive cally the most commonly used, however, it has been replaced by newer
serum metabolic panel for the detection of hypercalcemia, renal failure, staging systems, which reflect better myeloma biology. The Salmon-
379
serum β M, C-reactive protein, and elevation of LDH (Table 107–4). Durie system relates myeloma cell mass to the extent of bony disease,
2
Myeloma protein studies should include serum protein electrophoresis hemoglobin, and calcium levels, and the monoclonal immunoglobulin
to quantitate the serum protein electrophoretic pattern in combination levels in serum and urine (Table 107–5). However, measurement of
with nephelometric quantitation of immunoglobulin levels, serum-free
light-chain assay, and a 24-hour urine collection to quantitate 24-hour
total urinary protein and determine specific urinary proteins, such as
light chains or Bence Jones protein, using urine electrophoresis. TABLE 107–5. Assessment of Myeloma Tumor Mass
Immunofixation of serum and urine is needed for the immuno- (Salmon-Durie)
globulin heavy- and light-chain isotype determination. Serum-free
12
2
light-chain assay is of particular use in the monitoring of patients with I. High tumor mass (stage III) (>1.2 × 10 myeloma cells/m )*
a plasma monoclonal immunoglobulin, the diagnosis and monitoring One of the following abnormalities must be present:
of patients who would otherwise be considered to have nonsecretory A. Hemoglobin <8.5 g/dL, hematocrit <25%
myeloma, and patients who only have light-chain proteinuria. Marrow B. Serum calcium >12 mg/dL
aspiration and biopsy should include genetic studies (FISH and cyto- C. Very high serum or urine myeloma protein production
genetics) and flow cytometry. Newer modalities such as mutational rates:
profiling and gene expression studies are being undertaken but are not
yet standard of care tests. Radiographic examination usually comprises 1. IgG peak >7 g/dL
a metastatic bone survey to detect vertebral compression fractures, 2. IgA peak >5 g/dL
3. Urine light chains >12 g/24 h
D. >3 lytic bone lesions on bone survey (bone scan not
TABLE 107–4. Assessment of Myeloma acceptable)
2
12
Complete blood count and differential count; examination of II. Low tumor mass (stage I) (<0.6 × 10 myeloma cells/m )*
blood film All of the following must be present:
Chemistry screen, including calcium, creatinine, lactate dehydro- A. Hemoglobin >10.5 g/dL or hematocrit >32%
genase, BNP, proBNP B. Serum calcium normal
β -Microglobulin, C-reactive protein C. Low serum myeloma protein production rates:
2
Serum protein electrophoresis, immunofixation, quantification of 1. IgG peak <5 g/dL
immunoglobulins, serum-free light chains 2. IgA peak <3 g/dL
24-Hour urine collection for protein electrophoresis, immunofixa- 3. Urine light chains <4 g/24 h
tion, quantification of immunoglobulins, including light chains D. No bone lesions or osteoporosis
12
Marrow aspirate and trephine biopsy with metaphase cytogenet- III. Intermediate tumor mass (stage II) (0.6 to 1.2 × 10 myeloma
ics, FISH, immunophenotyping; gene array, and plasma cell label- cells/m )*
2
ing index (if available) All patients who do not qualify for high or low tumor mass cat-
Bone survey and MRI; PET-CT (if available) egories are considered to have intermediate tumor mass
Echocardiogram with assessment of diastolic function and mea- A. No renal failure (creatinine ≤2 mg/dL)
surement of interventricular septal thickness; EKG (if amyloidosis B. Renal failure (creatinine >2 mg/dL)
suspected)
*Estimated number of neoplastic plasma cells.
BNP, brain natriuretic peptide; CT, computed tomography; EKG, elec- Reproduced with permission from Durie BG, Salmon SE: A clinical stag-
trocardiogram; FISH, fluorescence in situ hybridization; MRI, magnetic ing system for multiple myeloma. Correlation of measured myeloma
resonance imaging; PET, positron emission tomography; proBNP, cell mass with presenting clinical features, response to treatment, and
prohormone B-type natriuretic peptide. survival. Cancer 1975 Sep;36(3):842–854.

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