Myeloma Flashcards
Renal failure from precipitation of light chains in the tubules (1) results in renal atrophy
- Bence Jones proteins; refers to myeloma nephrosis
M‐protein is most frequently IgM
Lymphoplasmacytic Lymphoma
These malignant cells can infiltrate many different tissues (lung, liver, spleen, lymph nodes, kidney etc.).
myeloma - plasma cells
lesions are found in the GI tract or respiratory tract
Heavy chain disease
cold urticaria from precipitation of the IgM in cold temperatures
Raynaud’s phenomenon in Lymphoplasmacytic Lymphoma
can evolve to full blown myeloma
Plasmacytoma
solitary lesion of neoplastic plasma cells in either the skeleton or soft tissue; frequently found in the nasopharynx
Plasmacytoma
proliferation of a clone of plasma cells in the bone marrow which results in multiple lytic skeletal lesions
Multiple myeloma
The M‐ components of multiple myeloma are most frequently ?
IgG (60%), IgA (25%), rarely IgM and quite rare IgD
M‐protein can also be demonstrated in the serum and in the tumor cells
Plasmacytoma
To summarize patients may have: (1) from the multiple lytic bone lesions.
- bone pain, fractures, hypercalcemia and anemia; refers to myeloma
The pathology of the bone lysis is related to secretion of (1) by the malignant clone of plasma cells, which in turn promotes secretion of (2) which then promotes osteoclast activity and bone lysis.
- IL‐6 2. RANK‐ligand; refers to myeloma
proliferation of neoplastic plasma cells in the GI tract or respiratory tract
Heavy chain disease
Κ or λ light chain of immunglobin
Bence Jones proteins; multiple myeloma
Translocation IgG locus chromosome 14 fusion partner fibroblast growth factor 3 on chromosome 4
Multiple myeloma
lymphocytes produce exclusively IgA
Heavy chain disease
lymph node or spleen lesions like lymphoma
heavy chain disease
The bone marrow of patients will contain at least 10% plasma cells some with obvious abnormal morphology.
myeloma
plasma cells secrete free light chains that deposit in the tissues as amyloid deposits
Primary or immune associated amyloidosis
Rouleaux formation of RBCs on blood smear
myeloma
develop full blown myeloma at a rate of about 1%/year
Monoclonal Gammopathies of Undetermined Significance (MGUS)
presence of <3gm/dL of a monoclonal protein in an otherwise asymptomatic patient
Monoclonal Gammopathies of Undetermined Significance (MGUS)
when produced in excess can markedly increase the blood viscosity resulting in (1) impairment, (2) symptoms, excess (3), cryoglobulenemia and Raynaud’s phenomenon
- visual 2. neurologic 3. bleeding Waldenstrom macroglobulenemia in Lymphoplasmacytic Lymphoma
Variant of Malt lymphoma
heavy chain disease
regulates B-cell differentiation
PAX5; Lymphoplasmacytic Lymphoma Chromosomal translocation 9:14
multiple lytic skeletal bony lesions frequently involving vertebrae, skull, ribs, pelvis
myeloma
proliferation of neoplastic monoclonal plasma cells and small lymphocytes
Lymphoplasmacytic Lymphoma
frequently found in the nasopharynx
Plasmacytoma
excess amounts of monoclonal protein (called M‐protein )
Multiple myeloma
Chromosomal translocations IgH locus on chromosome 14 and PAX5 gene on chromosome 9
Lymphoplasmacytic Lymphoma
mostly affects kidney and heart
Primary or immune associated amyloidosis
Upper respiratory tract(sinuses ,nasopharynx ,larynx)
Plasmacytoma
recurrent bacterial infections and even sepsis from suppression of normal immunoglobulins by the neoplastic clone of plasma cells
myeloma
mostly nodal lesions
Lymphoplasmacytic lymphoma
amyloid deposits in kidney, heart, GI tract and blood vessels causing?
renal failure, cardiac failure, and malabsorption in GI tract; refers to Primary or immune associated amyloidosis
Renal failure may be seen in up to half of all cases.
myeloma
mostly bony lesions
myeloma
Diagnosis of myeloma
Detecting the M‐protein using electrophoresis and by bone marrow biopsy demonstrating >10% plasma cells.
In extreme cases the malignant plasma cells spill over into the peripheral blood and this is then termed (1).
- plasma cell leukemia; refers to myeloma