Plasma cell myeloma

ICD-O-1 Morphology
Effective 1978 - 1991
ICD-O-2 Morphology
Effective 1992 - 2000
ICD-O-3 Morphology
Effective 2001 and later
for cases diagnosed 1978 and later
Primary Site(s)
Primary site must be bone marrow (C421)

Not Applicable
Module Rule
Plasma cell myeloma is a bone marrow-based multifocal plasma cell neoplasm. The disease spans a clinical spectrum from asymptomatic to aggressive forms, plus disorders caused by the deposition of abnormal immunoglobulin chains in tissue.

Plasma cell myeloma is a type of cancer of the plasma cells which are immune cells in bone marrow that produce antibodies.

A criterion for diagnosing plasma cell myeloma is usually equal to or greater than 10% of plasma cells in the bone marrow, but some symptomatic patients have a lower percentage. The registrar does not code plasma cell myeloma based on the percentage of plasma cells. There must be a diagnosis PCM.

A clinical diagnosis (no bone marrow biopsy done or unknown if bone marrow biopsy done) of PCM may be based on amyloidosis with associated renal impairment, anemia, and/or hypercalcemia supported by radiologic evidence of multiple lytic bone lesions.

Any case, including DCO's, listed as myeloma, multiple myeloma or PCM may be coded to 9732/3.

Multiple bone marrow biopsies may be done. Only one of the biopsies needs to be positive.
Abstractor Notes
Plasma cell myeloma (PCM) usually has generalized bone marrow involvement. Lytic bone lesions and bone tumor masses of plasma cells also occur.

There are three clinical variants of plasma cell myeloma, all of which are coded to 9732/3.

1. Asymptomatic (smoldering or inactive) PCM: Bone marrow involvement, but no related organ or tissue impairment. Similar to MGUS in its lack of symptoms, but more likely to develop to symptomatic PCM. About 8% of patients are initially asymptomatic.

2. Non-secretory myeloma occurs when there is absence of an M protein on immunofixation electrophoresis; there is impaired (or not) secretion of immunoglobulin into the blood or urine. A bout 3% of PCM cases are non-secretory.

3. Plasma cell leukemia (PCL) occurs when the number of plasma cells in the peripheral blood is 20% of the leukocyte differential count. Other areas of involvement include spleen, liver, pleural effusions, ascites, and cerebrospinal fluid. PCL may be present at diagnosis or occur as a late feature of PCM (secondary PCL); 2-5% of myeloma cases are primary PCL. Clinical features overall are similar to PCM. Lymphadenopathy, organomegaly and renal failure are often present in PCL. PCL is an aggressive disease with short survival.

The International Staging System for Multiple Myeloma Staging for Multiple Myeloma is based on:
1. Amount of monoclonal (or myeloma) protein (M protein) in the serum and/or urine
2. Various clinical parameters such as: hemoglobin and serum calcium concentrations, number of lytic bone lesions
3. Presence or absence of renal failure.
Stage I
Stage II
Stage III

Watchful waiting: Asymptomatic patients with no lytic lesions and normal renal function.

For patients with symptoms and advanced disease
1. Induction therapy
2. Consolidation therapy
3. Maintenance therapy
4. Supportive care
Definitive Diagnostic Methods
Bence-Jones protein
Bone marrow biopsy
Genetic testing
Peripheral blood smear
Genetics Data
Five major oncogenes involved in 14q32 translocation: cyclin D1, C-MAF, FGFR3/MMSET, cyclin D3, and MAFB
High load of IGHV gene somatic hypermutation
Immunoglobulin heavy and light chain genes are clonally rearranged
Whole or partial chromosome deletions or translocations
CD56 aberrantly expressed (except PCL)
CD56- (PCL)
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Transformations to
Corresponding ICD-9 Codes
203.0 Multiple myeloma
203.1 Plasma cell leukemia
Corresponding ICD-10 Codes
C90.0 Multiple myeloma
C90.1 Plasma cell leukemia
Corresponding ICD-10-CM Codes (U.S. only)
C90.0 Multiple Myeloma (effective October 01, 2015)
C90.1 Plasma cell leukemia (effective October 01, 2015)
Signs and Symptoms
Bence-Jones protein accumulation in the renal tubules causing renal damage
Bone pain
End-organ damage
Pathological fractures
Skeletal destruction with osteolytic lesions
Progression and Transformation
Extramedullary involvement usually indicates advanced disease
Epidemiology and Mortality
Age: 70 years median age (rare in children and adults less than 30)
Incidence: 10-15% of hematopoietic malignancies
Mortality: 20% of deaths from hematopoietic malignancies
Race: Occurs in african americans twice as much as caucasians
Sex: male predominance
Survival: Stage I: 62 months median survival; Stage II: 44 months median survival; Stage III: 29 months median survival