Immunoproliferative Disorders Flashcards

Vocabulary flashcards covering immunoproliferative disorders, including plasma cell phenotypes, Multiple Myeloma, Waldenstrom’s Macroglobulinemia, and Monoclonal Gammopathy of Undetermined Significance.

Long-lived plasma cells

Cells in the bone marrow that produce most $IgA$ and $IgG$ in normal adults, characterized by the phenotype $CD38^{bright}$, $syndecan-1^{bright}$, $CD19+$ and $CD56^{weak/-}$.

Plasmablasts

Slowly proliferating cells that migrate to the bone marrow after T helper cell stimulation and typically die via apoptosis after several weeks or months.

Monoclonal gammopathy

The production of elevated levels of a single class and type of immunoglobulin by a single clone of plasma cells.

M protein

A monoclonal protein, also known as a paraprotein, produced in plasma cell dyscrasias.

Polyclonal gammopathy

A secondary disease characterized by the elevation of two or more immunoglobulins produced by several clones of plasma cells.

Bence-Jones Proteins

Free light chains often associated with renal damage and Light Chain Disease.

Multiple Myeloma (MM)

A plasma cell neoplasm characterized by the accumulation of plasma cells in the bone marrow ($>90\%$) and monoclonal protein in blood or urine.

14q32 translocation

A genomic instability event found in $50\%$ of Multiple Myeloma cases.

Chromosome 13 deletion

A karyotype abnormality involving the loss of genetic material from chromosome 13 found in $50\%$ of Multiple Myeloma cases.

MM Epidemiology

Typical onset occurs between ages $40-70$ years, with peak incidence in the seventh decade and affects males in approximately $61\%$ of cases.

Beta-2-microglobulin

A laboratory level used to predict the prognosis of patients with Multiple Myeloma.

Lytic Lesions

Bone destruction caused by increased bone resorption in Multiple Myeloma, often resulting in bone pain in the back or chest.

Waldenstrom’s Macroglobulinemia (WM)

A disorder involving abnormal proliferation of plasmacytoid lymphocytes and production of $19S-IgM$.

Hyperviscosity syndrome

A condition more prevalent in WM than MM, caused by high levels of $IgM$ leading to blurred vision and cardiopulmonary abnormalities.

Cryoglobulinemia

A condition where proteins (often $IgG/IgA$ binding to $IgM$) precipitate at $4\,^{\circ}C$ and reverse at $37\,^{\circ}C$, common in Waldenstrom’s Macroglobulinemia.

Periodic Acid Schiff (PAS) stain

A staining method used on bone marrow aspirates during the laboratory evaluation of Waldenstrom's Macroglobulinemia.

Monoclonal Gammopathy of Undetermined Significance (MGUS)

A condition where serum monoclonal protein is $<3\,g/dL$, bone marrow plasma cells are $<10\%$, and there is an absence of lytic bone lesions or other clinical signs.

Light Chain Disease

A condition where only Bence Jones Proteins are produced, often showing a very unusual urine pattern on electrophoresis.

Franklin’s Disease

A synonym for gamma Heavy Chain Disease (HCD).

Alpha Heavy Chain Disease

The most common form of Heavy Chain Disease (HCD).