cne21th

2008 WHO new classification of Plasma cell neoplasm

Symptomatic plasma cell myeloma

M-protein in serum or urine*
Bone marrow clonal plasma cells or plasmacytoma#
Related organ or tissue impairment @
(CRAB: hypercalcemia, renal insufficiency, anemia, bone lesion)

* No level of serum or urine M-protein is included. M-protein in most cases is >30g/L of IgG or >25/L of IgA or >1g/24hr of urine light chain but some patients with symptomatic myeloma have levels lower than these!
#Monoclonal plasma cells usually exceed 10% of nucleated cells in the marrow but no minimal level is designated because about 5% of patients with symptomatic myeloma have <10% marrow plasma cells
@The most important criteria for symptomatic myeloma are manifestation of end organ damage included anemia, hypercalcemia, lytic obne lesion, renal insufficiency, hyperviscosity, amyloidosis or recurrent infection

Asymptomatic (smoldering) myeloma
M-protein in serum at myeloma level (>30g/L)
AND/OR
10% or more clonal plasma cells in bone marrow
No related organ or tissue impairment or myeloma-related symptoms

Plasmacytoma
Clonal proliferation of plasma cells that are cytologically and immunophenotypically
identical to those of plasma cell myeloma, but manifest a localized osseous and extraosseous growth pattern

Solitary plasmacytoma of bone
localized bone tumor consisting of plasma cells identical to those seen in plasma cell myeloma, which appears as a solitary lytic lesion of radiological examination (complete skeletal radiographs must show no other lesions).
5% of all plasma cell neoplasm
Location :
most active hematopoiesis: vertebrae (thoracic), ribs, skull, pelvis, femur, clavicle, scapula
Clinical features :
bone pain or pathologic fracture
usually no M component or low level of gammopathies; if presented, usually disappear after local excision.
NO CRAB!!
Polyclonal Ig in normal level.
Treatment :
Radiation, local control in most patient
Prognosis :
2/3 to generalized myeloma or additionaly solitary or multiple plasmacytoma
1/3 disease free survival in 10 years
higher incidence of progression

older patients
larger mass >5 cm
persistent M-protein following local radiotherapy

Maybe occult plasma cell myeloma

Osteopenia and low levels of uninvolved immunoglobulin 

Extraosseous plasmacytoma
Neoplasm of monoclonal plasma cells forming a tumor at an extraosseous and extramedullary site.
3~5% of all plasma cell neoplasm
typically adult with a 2:1 male:female ratio
Location :
Upper respiratory tract (80%), GI tract, urinary bladder, central nervous system, breast, thyroid.......
15~20% with monoclonal gammopathy (IgA)
NO CRAB!!
Morphology must D.D with GI tract, skin MALToma with plasmacytic differentiation
Expression CD20, cytoplasmic  m rather than  g  heavy chain favor lymphoma
Treatment :
Radiation therapy
Prognosis :

regional recurrence: 25%
15% develop plasma cell myeloma
70% of patients remain disease free at 10 years (MALToma)