Plasma cell myeloma

Name
Plasma cell myeloma
ICD-O-1 Morphology
9730/3
Effective 1978 - 1991
ICD-O-2 Morphology
9732/3
Effective 1992 - 2000
ICD-O-3 Morphology
9732/3
Effective 2001 and later
Reportable
for cases diagnosed 1978 and later
Primary Site(s)
C421

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Grade
6 - B-cell
Module Rule
None
Alternate Names
Alpha PCM
Alpha plasma cell myeloma
Early multiple myeloma
Early myeloma
Evolving multiple myeloma
Evolving myeloma
Evolving plasma cell myeloma
Gamma PCM
Gamma plasma cell myeloma
Indolent myeloma
Indolent PCM
Indolent plasma cell myeloma
Kahler's disease
Multiple myeloma
Myeloma, NOS
Medullary plasmacytoma
Myelomatosis
Non-secretory myeloma
PCM
Plasma cell leukemia
Plasmacytic leukemia
Primary PCL
Smoldering myeloma
Secondary plasma cell leukemia
Smoldering plasma cell myeloma
Definition
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.

The 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.

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 progress to symptomatic PCM. About 8% of patients are initially asymptomatic.

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.

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 cerrebrospinal 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.
Definitive Diagnostic Methods
Bence-Jones protein
Bone marrow aspiration
Bone marrow biopsy
FISH
Genetics testing
Immunophenotyping
Peripheral blood
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
Trisomies
Whole or partial chromosome deletions or translocations
Immunophenotyping
CD19-
CD38
CD56 aberrantly expressed (except PCL)
CD56- (PCL)
CD79a
CD138
VS38c
Treatments
Chemotherapy
Hormone therapy
Immunotherapy
Stem cell transplant
Transformations to
None
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 (effective October 1, 2015 U.S. only)
C90.0 Multiple Myeloma
C90.1 Plasma cell leukemia
Signs and Symptoms
Anemia
Bence-Jones protein accumulation in the renal tubules causing renal damage
Bone pain
End organ damage
Hypercalcemia
Pathological fractures
Serum monoclonal protein
Skeletal destruction with osteolytic lesions
Diagnostic Exams
None
Recurrence and Metastases
Extramedullary involvement usually indicates advanced disease
Epidemiology and Mortality
None