Plasma cell dyscrasias Flashcards
What are the reactive plasma cell conditions?
Chronic infections (H. pylori gastritis, osteomyelitis, endometris) Autoimmune processes (lupus, hepatitis)
What are the plasma cell neoplastic conditions?
Multiple myeloma
Monoclonal gammopathy of undetermined significance (MGUS)
Plasmacytoma (Extramedullary or solitary of bone)
Lymphoplasmatic lymphoma
Amyloidosis
How do we assess clonality of plasma cell?
Monoclonal antibodies in the serum or urine Light chain restriction in cell cytoplasm (kappa v lambda) by flow cytometry or immunohistochemistry Immunophenotypic aberrancy (e.g. CD56 on plasma cells)
What are M proteins?
How do they appear in serum?
How do they appear in urine?
What else can they be assocciated with?
Monoclonal antibodies
Intact immunoglobulins and free light chains
Full antibodies are filitered with kidney damage, free light chains may pass through glomerulus (Bence-Jones proteins
B-cell lymphomas (monoclonal gammopathy), rarely reactive states
How are M proteins IDed?
Most important spikes in electrophoresis?
What does a gamma-globulin spike indicate?
What does the immunofixation do?
Identification with electrophoresis and immunofixation
Most important spikes in electrophoresis are the albumin fraction (positive lead) and gamma fraction (negative lead)
Spike means that there is one dominant Ab
Allows specific ID of the gammaglobulin
What is multiple myeloma?
Benign/malignant?
Male or female dominant?
Race dominant?
Age range?
Most common plasma cell neoplasm
Malignant
Males greater than female
AAs greater than caucasians
50-70 y/o
What are the diagnostic criteria for MM?
Clonal plasma cells - Bone marrow and plasmacytoma
M protein - Serum and/or Urine
Need to check for Bence Jones protein (kidney disease results in complete excretion, 20% have no serum findings)
End organ damage: hyperCalcemia, Renal insufficiency, Anemia, Bone Disease (CRAB
Which bone lesions are associated with MM?
Why is there an association with hypercalcemia?
Bone pain
Pathologic fractures in Vertebrae, Ribs, Skull, Pelvis, Femur
Osteopenia
Hypercalcemia is secondary to the bone resorption
What is this?
Clonal plasma cells in myeloma
What effect does M protein have on immune system?
Immunosupression
Elevated risk for recurrent infections due to abnormal Ig production
Most common cause of death
What is the prognosis of multiple myeloma?
How is it treated?
Incurable, median survival of 4-6 years treateed (chemo and tandem autologous bone marrow transplants)
What is a plasmacytoma?
How is it treated when distinct (non-systemic)?
Are there clonal plasma cells in marrow? M protein?
Localized growth of monoclonal plasma cell
Radiation therapy
No clonal plasma cells in marrow.
+/- M protein
What is lymphoplasmacytic lymphoma?
What protein is associated with it?
What syndrome is associated with it?
Lymphoma with plasmacytic differentiation (B cells and plasma cells are neoplastic)
IgM paraprotein
Waldenstrom’s macroglobulinemia
What are the symptoms of Waldenstrom’s macroglobulinemia?
Visual/neurologic impairment
Cryoglobulinemia - Raynaud phenomenon, Bleeding
Monoclonal Gammopathy of Undetermined Significance
What is it?
What is the diagnostic criteria?
Most common form of monoclonal gammopathy (2% in >50y/o; 3% in >70y/o)
Diagnostic criteria:
<3 g/dL serum M protein
<10% clonal plasma cells in bone marrow
No myeloma-related end organ damage
