Term
| What are the normal peaks on SPE? |
|
Definition
| alb, alpha 1, alpha 2, beta, gamma |
|
|
Term
| what is the purpose of immunofixation? |
|
Definition
| identifies/subtypes the M spike component |
|
|
Term
|
Definition
|
|
Term
| What are the symptoms of MGUS? |
|
Definition
|
|
Term
| What is the racial predominance of MGUS? |
|
Definition
|
|
Term
| What are the resulst of SPE or immunofixation of pts with MGUS? |
|
Definition
| small monoclonal spike usually IgG |
|
|
Term
| What are the bone marrow results of pts with MGUS? |
|
Definition
| less than 10% clonal plasma cells in marrow (usually less than 5%) |
|
|
Term
| What are the radiographic findings of MGUS? |
|
Definition
|
|
Term
|
Definition
| hypercalcemia, renal insufficiency, anemia, bone lesions |
|
|
Term
| What is the significance of having MGUS? |
|
Definition
| you are at increased risk for developing myeloma |
|
|
Term
| How common is multiple myeloma? |
|
Definition
| MC lymphoid malignancy in AA and second in caucasians |
|
|
Term
| What is the typical pt with MM? |
|
Definition
| AA > C by 2x; median age = 68; rare before 53, not found in children; more common in men |
|
|
Term
| What is the median survival of MM? |
|
Definition
|
|
Term
|
Definition
| need pathological, radiological, and clinical features |
|
|
Term
| What are the two subtypes of MM? |
|
Definition
| symptomatic and asymptomatic |
|
|
Term
| What are the symptoms of symptomatic MM? |
|
Definition
| bone pain (back, long bones, pelvis) very common, anemia, dehydration, urinary frequency (renal failure), headache, fever, infections |
|
|
Term
| What are the findings of asymptomatic myeloma? |
|
Definition
| M-protein in serum at myeloma levels and/or 10% or more clonal plasma cells in marrow. no related organ or tissue impairment |
|
|
Term
| What are the laboratory findings of plasma cell myeloma? |
|
Definition
| proteinuria, azotemia, increased serum calcium, anemia, pancytopenia, monoclonal protein (M component) in serum and/or urine |
|
|
Term
| What types of monoclongal proteins are made in MM? |
|
Definition
| 75-85% make Ig; 10-20% make light chains only |
|
|
Term
| What is the most common type of serum monoclonal Ig made in multiple myeloma? |
|
Definition
|
|
Term
| What does the absence/presence of paraprotein in MM mean? |
|
Definition
| serum monoclonal IG will cause paraprotein; light chains only will sometimes have absent paraprotein |
|
|
Term
| What is different about patients that only make light chains? |
|
Definition
| there is rapid renal excretion (bence jones proteins), serum paraprotein may be absent, found on urine electrophoresis |
|
|
Term
| Can you have a non-secretory myeloma? |
|
Definition
|
|
Term
| Besides MM, what are other causes of monoclonal proteins? |
|
Definition
| B-cell lymphomas, autoimmune disease, infection (HIV) |
|
|
Term
| What are the features on PBS of a pt with MM? |
|
Definition
|
|
Term
| What are the features on bone marrow aspiration of patients with MM? |
|
Definition
| atypia and multinucleation, plasma blasts, mott cell with grapelike clusters of IG protein |
|
|
Term
| What are the MC cytogenetic abnormalities of plasma cell myelomas? |
|
Definition
| MCly hyperdiploidy and complex strucutral rearrangements |
|
|
Term
| What is the 10 yr survival of MM? |
|
Definition
|
|
Term
| What is the treatment for MM? |
|
Definition
| chemotherapy, alkylating agent and corticosteroids, bone marrow transplant |
|
|
Term
| What's another name fro osteosclerotic myeloma? |
|
Definition
|
|
Term
| What does POEMS syndrome stand for? |
|
Definition
| polyneuropathy (sensorimotor demyelination) organomegaly (hepatosplenometaly), Endocrinopathy, Monoclonal gammopathy, Skin changes |
|
|
Term
| What are the endocrinopathies of POEMS syndrome? |
|
Definition
| diabetes, gynecomastia, testicular atrophy, impotence, hypothyroid, adrenal insufficiency |
|
|
Term
| What are the skin changes associated with POEMS syndrome? |
|
Definition
| hyperpigmentation, hypertrichosis |
|
|
Term
| What causes osteosclerotic myeloma? |
|
Definition
| not well understood; may be related to changes in the levels of a cytokine or growth factor (IL-1beta, IL-6, TNFalpha, VEGF). Possibly associated with KSHV/HHV8 |
|
|
Term
| T/F Hypercalcemia, renal insufficiency, and pathologic fractures, can occur with osteosclerotic myeloma. |
|
Definition
| true but it is rare to have these symptoms |
|
|
Term
| What proteins make up the M component in serum or urine of a patient with osteosclerotic myeloma? |
|
Definition
| usually either IgG or IgA |
|
|
Term
| Does osteosclerotic myeloma have an M spike? |
|
Definition
| M component is usually low |
|
|
Term
| Which has a better prognosis, mutliple myeloma or osteosclerotic myeloma? |
|
Definition
|
|
Term
| What is the morphology of osteosclerotic myeloma on a bone marrow biopsy? |
|
Definition
| marrow osteosclerosis, focally thickened trabeculae, and paratrabecular fibrosis with entrapped plasma cells |
|
|
Term
| What are the symptoms of solitary polasmacytoma of bone? |
|
Definition
| bone pain, fracture, palpable mass with soft tissue extension |
|
|
Term
| What do you call a localized plasma cell neoplasm? |
|
Definition
| solitary plasmacytoma of bone |
|
|
Term
| What're the histological characterisitcs of plasma cells of a solitary plasmacytoma of bone? |
|
Definition
| plasma cells identical to those of myeloma |
|
|
Term
| What percent of all plasma cell neoplasms are solitary plasmacytoma of bone? |
|
Definition
|
|
Term
| Are there M-proteins associated with a solitary plasmacytoma of bone? |
|
Definition
| typically not but there are some with low level gammopathies. If M protein is present, it usually disappears with treatment. If M-protein is very high or does not disappear with treatment, suspect multiple myeloma |
|
|
Term
| What is the treatment/prognosis of a solitary plasmacytoma of bone? |
|
Definition
| treated with radiation and follow up with serum and urine immunofixation. 35% cured in 10 years; 55% develop myeloma; 10% local recurrence or another solitary plasmacytoma |
|
|
Term
| What is the gender distribution of extraosseous plasmacytoma? |
|
Definition
|
|
Term
| Where do extraosseous plasmacytomas occur? |
|
Definition
| 80% occur in the upper respiratory tract (oropharynx, nasopharynx, sinuses, larynx); other sites include GI, urinary bladder, CNS, breast, thyroid, testis, parotid, nodes, skin |
|
|
Term
| Do you get monoclonal gammopathy with an extraosseous plasmacytoma? |
|
Definition
| 15-20% have monoclonal gammopathy |
|
|
Term
| What are the symptoms of extraosseous plasmacytoma? |
|
Definition
| epistaxis, nasal obstruction, rhinorrhea. No evidence of anemia, hypercalcemia, renal insufficiency |
|
|
Term
| What is the treatment/prognosis of extraosseous plasmacytoma? |
|
Definition
| radiation; regional recurrences in 25%, development of plasma cell myeloma in 15% |
|
|
Term
| What causes primary amyloidosis? |
|
Definition
| a plasma cell or rarely a lymphoplasmacytic neoplasm that secretes light chains (common) or heavy chains (rare) |
|
|
Term
| Most commonly, light chains deposit into tissue in the form of... |
|
Definition
| beta pleated sheets; called "AL" amyloid for "amyloid light chains" |
|
|
Term
| What is the typical patient population with primary amyloidosis? |
|
Definition
| adults over 40 with male predominance |
|
|
Term
| List the clinical findings of primary amyloidosis related to deposition of amyloid in organs? |
|
Definition
| heart=CHF, kidney=nephrotic syndrome, GI=malabsorption, peripheral nerves=neuropathy, carpal tunnel syndrome, marrow=cytopenias; vessels=bleeding |
|
|
Term
| What causes bleeding in primary amyloidosis? |
|
Definition
| cytopenias from deposition in marrow; abnormal vessels; factor X deficiency (due to binding of factor X to amyloid) |
|
|
Term
| What labs are helpful in diagnosing primary amyloidosis? |
|
Definition
| M-protein in serum or urine by immunofixation (usually lambda restricted), congo red stain, immunostains for amyloid (P-component, AL), mass spectroscopy for AL typing |
|
|
Term
| What type of stain produces "apple green" birefringence under polarized light? |
|
Definition
|
|
Term
| What is the WHO name for Waldenstrom's macroglobulinemia? |
|
Definition
| lymphoplasmacytoid lymphoma |
|
|
Term
| Waldenstrom's macroglobulinemia is a neoplasm of... |
|
Definition
| mixture of small B-cells, plasmacytoid lymphocytes, and plasma cells that produce an IgM monoclonal gammopathy |
|
|
Term
| What tissues are damaged by Waldenstrom's macroglobulinemia? |
|
Definition
| lymph organs (bone marrow, lymph nodes, spleen); hyperviscosity syndrome |
|
|
Term
| What is the prognosis of Waldenstrom's macroglobulinemia? |
|
Definition
| indolent clinical course with ~5 year median survival |
|
|
Term
| What causes hyperviscosity syndrome? |
|
Definition
| aggregating paraprotein (IgM) |
|
|
Term
| Describe the pathogenesis of hyperviscosity syndrome? |
|
Definition
| circulatory insufficiency and abnormal hemostasis |
|
|
Term
| What are the symptoms of hyperviscosity syndrome? |
|
Definition
| bleeding (skin and mucosal), dyspnea (congestion on CXR), encephalopathy and visual disturbances, headache, hepatosplenomegaly, lymphadenopathy |
|
|
Term
| What does the bone marrow aspiration of a person with Waldenstrom's macroglobulinemia look like? |
|
Definition
| lymphoid + lymphoplasmacytoid + plasmacytoid cells in marrow |
|
|
Term
| Does Waldenstrom's macroglobulinemia have an M-spike on SPE? |
|
Definition
|
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