Plasma Cell Neoplasms

Plasma Cell Neoplasms

AGENDA
Definition
Classification
 Plasma Cell Neoplasms
Definition
- Immuno-secretory disorders resulting from
expansion of a single clone (monoclonal) of
immunoglobulin secreting terminally
differentiated,
end-stage B cells (plasma cells).
- Secretion of a monoclonal homogenous
immunoglobulin
product (M-component).
 Plasma Cell Neoplasms
Pro-T Lymphocyte Pre-T Lymphocyte

Cytotoxic T Lymphocyte

Helper T Lymphocyte

Lymphoid Stem
Cell

Pre-B Lymphocyte B Lymphocyte Plasma Cell

Plasma Cell Neoplasms
Classification
• Plasma Cell Myeloma
• Monoclonal Gammopathy of Undetermined
Significance (MGUS)
• Plasmacytoma
• Primary Amyloidosis
 Plasma Cell Neoplasms
• Plasma Cell Myeloma
- Definition
- Epidemiology
- Diagnosis
 Plasma Cell Neoplasms
• Plasma Cell Myeloma
Definition
- Bone marrow-based multifocal monoclonal
plasma
cell proliferation characterized by serum M-
component
and osteolytic lesions.
- Spectrum of severity (indolent- to-aggressive)
 Plasma Cell Neoplasms
• Plasma Cell Myeloma
Epidemiology
- ~ 15% of hematological malignancies.
- Disease of elderly (6th – 7th decade).
 Plasma Cell Neoplasms
• Plasma Cell Myeloma
Diagnosis
A) Clinical Features
B) Radiological Features
C) Laboratory Features
 Plasma Cell Neoplasms
• Plasma Cell Myeloma
Diagnosis (cont’d)
A) Clinical Features
- Extensive skeletal destruction:
bone pain, pathological fractures
- Excessive monoclonal immunoglobulin
production:
hyperviscosity syndrome
- Depressed normal immunoglobulin
production:
recurrent infections
- Bone marrow replacement:
anemia, thrombocytopenia (late)
 Plasma Cell Neoplasms
• Plasma Cell Myeloma
Diagnosis (cont’d)
B) Radiological Features
- Lytic bone lesions
Plasma Cell Neoplasms
Plasma Cell Neoplasms
Plasma Cell Neoplasms
 Plasma Cell Neoplasms
• Plasma Cell Myeloma
Diagnosis (cont’d)
C) Laboratory Features
- Hematology
CBC, PBS examination, BM examination
- Serum Protein Electrophoresis (SPE)
Monoclonal band (IgG, IgA),
hypogammaglobulinemia
- Urine Protein Electrophoresis
Bence-Jones Protein
- Others
hypercalcemia, renal impairment
PBS
BM
BM
Plasma Cell Neoplasms

Polyclonal vs. Monoclonal Ig Production

 Plasma Cell Neoplasms
• Plasma Cell Myeloma
Diagnostic Criteria
Major Criteria Minor Criteria

Marrow plasmacytosis (30%) Marrow plasmacytosis

(10-30%)
Plasmacytoma on biopsy
M-component (serum or urine)
Serum IgG > 35g/L, IgA > 20g/L
Urine > 1g/24hrs of BJ protein
M-component
Lytic bone lesions
Hypogammaglobulinemia
 Plasma Cell Neoplasms
• MGUS
- The presence of M-component in persons
without
evidence of plasma cell neoplasm.
- ~ 25% will develop plasma cell neoplasm (~
10years).
- Follow up is essential.
 Plasma Cell Neoplasms
• MGUS (cont’d)
Diagnostic Criteria
- M-component
- Marrow plasmacytosis (< 10%)
- No lytic bone lesions
- No myeloma-related symptoms
 Plasma Cell Neoplasms
• Plasmacytoma
- Clonal proliferations of plasma cells that are
identical to plasma cell myeloma but manifest a
localized osseous or extraosseous growth pattern.
 Plasma Cell Neoplasms
• Primary Amyloidosis
- Plasma cell neoplasm that secretes an abnormal
immunoglobulin, which deposits in various tissues
and forms fibrillary protein (Amyloid).
- Systemic deposition involving heart, liver,
kidneys,
GIT, tongue, nerves.
- Binds Congo red stain to produce characteristic
birefringence.
- M-component in 80% of patients.
- 20% have plasma cell myeloma.
Congo red