Plasma Cell Dyscrasia Flashcards
Multiple myeloma
Malignancy of plasma cells
Classic triad:
-anemia
-hypercalcemia
-lytic bone lesions
increased monoclonal Igs in peripheral blood (M protein peak on serum electrophoresis)
Greater than 10% plasma cells w/ clusters of mature plasma cells/blasts
Hypercalcemia in multiple myeloma
Results from the lytic bone lesions - which result from local tumor cell-mediated activation of osteoclasts by IL-6 (along w/ other cytokines)
Increased amounts of IL-6 is also associated w/ a worse prognosis - survival of myeloma cells is dependent on IL-6
Monoclonal immunoglobulin (paraprotein) spike (M spike)
Serum electrophoresis of multiple myeloma Pt will show increased serum protein (due to the M spike) with normal serum albumin levels
Bence-Jones proteins
In multiple myeloma - monoclonal IgG light chains secreted by malignant plasma cells
Small and filtered into urine (no M spike in serum)
The lambda light chains are nephrotoxic and can precipitate in the kidney - myeloma cast nephropathy (myeloma kidney) - most common form of renal injury
Deposition in any organ w/ multiple myeloma Pt’s can produce what?
Systemic amyloidosis
Heart - infiltrative cardiomyopathy
W/ systemic - light chains deposited in the glomeruli can be seen with Congo Red stains
Peripheral blood smear of individuals w/ multiple myeloma
Often demonstrates grouped erythrocytes w/ a stacked-coin appearance (roleaux formations)
Due to increased serum paraproteins
Plasma cells are not seen in the peripheral smear with multiple myeloma
Waldenström’s macroglobulinemia (WM)
Plasma cell dyscrasia characterized by monoclonal production of IgM (M spike like multiple myeloma)
No lytic bone lesions and no hypercalcemia
Clinical cross between multiple myeloma and small lymphocytic lymphoma
Like SLL, WM is associated with infiltration of organs outside of the bone marrow by neoplastic cells - spleen and lymph nodes (does not happen in multiple myeloma b/c IgM pentamer)
Which is prone to hyperviscosity syndrome, Waldenström’s macroglobulinemia or multiple myeloma?
Waldenström’s macroglobulinemia - signs:
Visual abnormalities, neurologic signs (HA/confusion), bleeding, and cryoglobulinemia
The bone marrow biopsy of an individual with Waldenström’s macroglobulinemia would show?
Proliferation of plasma cells, lymphocytes, and plasmacytoid lymphocytes (lymphoplasmacytic lymphoma = Waldenström’s macroglobulinemia)
Intranuclear aggregates of immunoglobulin (Dutcher bodies) may be seen in some plasma cells
Like MM, peripheral smear may show rouleaux formation due to increased serum paraproteins
Plasmacytoma
Solitary tumor of abnormal plasma cells that may be found w/i bone or soft tissue
Bone lesions are associated w/ a monoclonal gammopathy and frequently evolve into a malignancy (unlike soft tissue tumors)
Monoclonal gammopathy of undertermined significance (MGUS)
Elevated serum monoclonal immunoglobulins (M spike) in an asymptomatic adult
M proteins are found in 1-3% of asymptomatic persons over the age of 40
No signs of myeloma, and bone marrow biopsy reveals less than 10% plasma cells (benign disorder)
Minority of Pt’s will progress to myeloma
Alpha heavy-chain disease
Type of plasma cell dyscrasia that is associated w/ secretion of alpha heavy chains (no light chains)
Primarily seen in the Mediterranean regions (Mediterranean lymphoma)
Numerous plasma cells infiltrate the lamina propria of the small intestines
Proceded by an abnormality called immunoproliferative small intestinal disease (IPSID) - villous atrophy of small intestines with steatorrhea - may be Tx’d w/ Abx
Light chain cast nephropathy
Seen in multiple myeloma - free immunoglobulin light chains form obstructive casts in the renal tubules
Large eosinophilic casts composed of Bence Jones proteins are seen in the tubular lumen
Renal failure with multiple myeloma may be caused by cast nephropathy due to the deposition of what substance in the renal tubules?
Immunoglobulin light chains
Light chain cast nephropathy, in which free immunoglobulin light chains form obstructive casts in the renal tubules, is the most common renal disease with multiple myeloma.
Large, waxy, eosinophilic casts composed of Bence Jones proteins are seen in the tubular lumen
An adult with multiple myeloma presents with signs of heart failure and is found to have a restrictive cardiomyopathy due to a complication of myeloma. What is most likely responsible for the cardiomyopathy in this adult with myeloma?
Amyloid deposition
With systemic amyloidosis (AL amyloidosis), which is a complication of myeloma, light chains can be deposited in the glomeruli (seen best with Congo Red stains). In the heart light chain amyloid deposition with AL amyloidosis can cause restrictive cardiomyopathy.
E. Plasma cell neoplasm
Difference in renal damage by AL amyloid deposition and deposition of lambda light chains
C. increased serum paraproteins
Paraproteins = immunoglobulins
Difference between Waldentsrom macroglobulinemia and miltiple myeloma
