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41 Cards in this Set
- Front
- Back
Plasma cell disorders are often called __________.
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Plasma cell dyscrasias
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What is multiple myeloma?
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Malignant proliferation of plasma cells in marrow.
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What is the most common primary malignancy of bone?
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Multiple myeloma
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In multiple myeloma, what serum molecule is often seen in high concentrations?
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IL-6
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IL-6 is an important growth factor for ______________.
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plasma cells
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Multiple myeloma plasma cells can activate what other cell type? What is the result?
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Osteoclasts through their RANK receptor (Osteoclast Activating Factor). They eat away at bone resulting in 'punched-out' lesions seen on X-ray. Increased risk of fracture. Lesions results in bone pain and hypercalcemia.
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Where do we typically see the 'punched-out' lytic bone lesions associated with multiple myeloma?
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(1) Vertebrae
(2) Skull |
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The plasma cells of multiple myeloma can produce what?
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(1) Osteoclast activating factor
(2) Immunoglobulins. |
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A patient has increased serum protein and you can't determine the cause. What could you do?
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Serum Protein Electrophoresis (SPEP)
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How is the gamma band on SPEP in normal circumstances? How does it look in multiple myeloma?
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Wide and not as high as albumin peak.
A sharp tall spike in the gamma region. |
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What does M-spike in multiple myeloma mean?
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Monoclonal
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The M-spike on SPEP in multiple myeloma is most commonly due to what Ig?
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Monoclonal IgG or IgA
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What is an important complication of multiple myeloma?
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Monoclonal plasma cells --> Lacks antigenic diversity --> Infection is the MC cause of death
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What is a complication of multiple myeloma involving having too much protein in the blood?
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Rouleax formation on smear. Increased serum protein decreases charge between RBCs.
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Aside from infection and rouleax formation, what is another possible complication of multiple myeloma?
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Primary AL amyloidosis. Free light chains circulates in serum and deposit in tissue.
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The proteins in primary amyloidosis associated with multiple myeloma can deposit in tissues. What else can the free chains do?
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Excreted in urine as Bence-Jones protein (proteinuria). Deposition in kidney tubules leads to risk of renal failure (myeloma kidney).
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You see a patient in your clinic with a high serum protein. SPEP shows M spike. You suspect MM. You don't find any lytic lesions, hypercalcemia, amyloid or proteinuria. What is going on?
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MGUS; Monoclonal Gammopathy of Undetermined Significance
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MGUS is common in the ___________.
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elderly
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MGUS is seen in ___% of ____-year-old individuals.
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5%; 70yo
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__% of patients with MGUS develop multiple myeloma each year.
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1%
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MGUS could almost be described as being a __________.
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dysplasia
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What is Waldenströms macroglobulinemia?
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B-cell LYMPHOMA with monoclonal IgM production (macroglobulin = IgM, because it is big)
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How do patients with Waldenströms macroglobulinemia present as?
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(1) Generalized LAD (it is a lymphoma); lytic bone lesions would be absent
(2) Increased serum protein with M spike (comprised of IgM) (3) Hyperviscosity due to high IgM. Visual and neurologic deficits show (e.g., retinal hemorrhage or stroke). (4) Bleeding (defective PLT aggregation in hyperviscosity) |
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When patients have acute complications in Waldenstroms macroglobulinemia we can treat them with?
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Plasmapheresis. Removes IgM from serum.
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Langerhans cells are _______?
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Specialized dendritic cells found predominantly in the skin.
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Langerhans cells are derived from?
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Bone marrow monocytes
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Langerhans cell present antigens to?
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Naive T-cells
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A tumor of langerhans cells is called?
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Langerhans cell histiocytosis
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What is characteristically seen in Langerhans cell histiocytosis?
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Birbeck graunles on EM (tennis rackets).
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Cells in langerhans cell histiocytosis have what markers?
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CD1a+, S100+ by immunohistochemistry.
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How many subtypes of langerhans cell histiocytosis do we have?
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3
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How could you know that a langerhans cell histiocytosis disease is malignant?
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If the disease is named after someone, the proliferation is usually malignant.
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Malignant proliferations in langerhans cell histiocytosis usually involve what?
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The skin. Shows up as a rash.
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Memory help: If the langerhans cell histiocytosis has two names in it, it is usually seen in?
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It will usually be seen in children less than 2 (infants)
If there is three peoples names it is usually seen in children greater than the age of 3. |
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Letterer-Siwe classic presentation?
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Classically presents as skin rash and cystic skeletal defects in an infant (infant < 2 yrs old).
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What organs are involved in Letterer-Siwe disease? What is the prognosis?
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Multiple organs may be involved.
Letterer-Siwe is rapidly fatal. |
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Eosinophilic granuloma is a ___________ (benign/malignant) proliferation of _____________ in _________.
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benign (no name in disease name); Langerhans cells; bone
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Classic presentation of eosinophilic granuloma?
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Pathologic fractures in adolescent; skin is not involved (benign, so it does not involve skin)
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What does a biopsy of a eosinophilic granuloma show?
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Biopsy shows Langerhans cells with mixed inflammatory cells, including eosinophils.
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If you got a patient with pathologic fractures that's an adolescent (tenåring, ungdom), you would start to think about what?
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(1) Osteosarcoma
(2) Eosinophilic granuloma |
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Classic presentation of Hand-Schüller-Christian disease?
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Classic presentation is scalp rash (malignant, not benign, so it involves skin), lytic skull defects, diabetes insipidus and exopthalmos. Classically seen in people greater than the age of 3.
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