Multiple Myeloma and Related Conditions
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Questions and Answers

What is a common clinical presentation in symptomatic patients with lymphadenopathy?

  • Elevated blood pressure
  • Increased appetite
  • Weight loss (correct)
  • Mood swings

    • Which factor is correlated with a better outcome in patients with CLL/SLL?

  • Deletions of 11q
  • Presence of NOTCH1 mutations
  • Somatic hypermutation (correct)
  • High expression of ZAP-70

    • What is the typical leukocyte count range in patients with CLL who have heavy tumor burdens?

  • Less than 5,000 cells/µL
  • In excess of 200,000 cells/µL (correct)
  • Between 10,000 and 25,000 cells/µL
  • Between 50,000 and 100,000 cells/µL

    • Which of the following is a common complication in patients with hypogammaglobulinemia?

    <p>Increased susceptibility to bacterial infections</p> Signup and view all the answers

    What is the overall median survival for patients diagnosed with CLL/SLL?

    <p>4 to 6 years</p> Signup and view all the answers

    What does the presence of spherocytes in an autoimmune hemolytic anemia indicate?

    <p>Hyperchromatic round erythrocytes</p> Signup and view all the answers

    Which immunophenotype markers are typically expressed in CLL/SLL?

    <p>CD19 and CD20</p> Signup and view all the answers

    What molecular anomaly is commonly associated with CLL/SLL?

    <p>Deletions of 13q14.3</p> Signup and view all the answers

    What is the typical presenting symptom in patients diagnosed with CLL/SLL?

    <p>Patients are often asymptomatic</p> Signup and view all the answers

    What role does Nuclear Factor Kappa B (NF-KB) play in the pathology of CLL/SLL?

    <p>Promotes cell survival</p> Signup and view all the answers

    What type of nucleated cells might be seen in cases of severe anemia?

    <p>Nucleated erythroid cells</p> Signup and view all the answers

    What is a distinctive feature of the tumor cells in CLL/SLL seen at higher magnification?

    <p>Small round lymphocytes</p> Signup and view all the answers

    What typically initiates the proliferation of CLL/SLL cells?

    <p>Stimuli from stromal cells</p> Signup and view all the answers

    What percentage of solitary myeloma cases typically present as a solitary lesion of bone or soft tissue?

    <p>3-5%</p> Signup and view all the answers

    Where do extraosseous lesions in solitary myeloma usually occur?

    <p>Lungs, oronasopharynx, or nasal sinuses</p> Signup and view all the answers

    What is the likely outcome for solitary osseous plasmacytoma if left untreated?

    <p>Progression to multiple myeloma</p> Signup and view all the answers

    Which condition serves as a middle ground between multiple myeloma and MGUS?

    <p>Smoldering myeloma</p> Signup and view all the answers

    What is the serum M protein level indicative of smoldering myeloma?

    <blockquote> <p>3 g/dL</p> </blockquote> Signup and view all the answers

    What percentage of patients with smoldering myeloma progress to multiple myeloma over 15 years?

    <p>75%</p> Signup and view all the answers

    What could be a characteristic of Igs produced in solitary myeloma cases?

    <p>Abnormal physicochemical properties</p> Signup and view all the answers

    What is true concerning the suppression of normal humoral immunity in solitary myeloma?

    <p>It leads to increased infections.</p> Signup and view all the answers

    What is the characteristic feature of Burkitt lymphomas regarding the MYC gene?

    <p>They are associated with translocations of the MYC gene on chromosome 8.</p> Signup and view all the answers

    Which protein is always NOT expressed in DLBCL specimens?

    <p>BCL2</p> Signup and view all the answers

    What type of immunophenotype is typically expressed in Burkitt lymphomas?

    <p>High levels of cyclin D1 and surface Ig</p> Signup and view all the answers

    What characteristic cell population is notably absent in Burkitt lymphomas?

    <p>Centroblasts and prolymphocytes</p> Signup and view all the answers

    What demographic is primarily affected by Burkitt lymphomas?

    <p>Children and young adults</p> Signup and view all the answers

    What response to chemotherapy is typical for Burkitt lymphomas?

    <p>Good response with cure rates in most cases</p> Signup and view all the answers

    Which immunophenotype marker is NOT associated with Burkitt lymphomas?

    <p>CD5</p> Signup and view all the answers

    What is the typical surface immunoglobulin expression in Burkitt lymphoma?

    <p>IgM and IgD</p> Signup and view all the answers

    Which characteristic is associated with the cytological features of Burkitt lymphoma?

    <p>Homogeneous populations of small lymphoid cells</p> Signup and view all the answers

    Which chromosome contains the c-MYC gene that is often translocated in Burkitt lymphoma?

    <p>Chromosome 8</p> Signup and view all the answers

    What type of cells are primarily involved in Hairy Cell Leukemia?

    <p>B lymphocytes</p> Signup and view all the answers

    Which immunophenotype marker is commonly expressed in Hairy Cell Leukemia?

    <p>CD19</p> Signup and view all the answers

    What is a significant clinical feature of Multiple Myeloma related to bone health?

    <p>Lytic bone lesions</p> Signup and view all the answers

    A critical cytokine for the proliferation and survival of myeloma cells is?

    <p>IL-6</p> Signup and view all the answers

    The genetic mutations associated with most cases of Hairy Cell Leukemia occur in which gene?

    <p>BRAF</p> Signup and view all the answers

    What common physical finding is associated with Pancytopenia in Multiple Myeloma?

    <p>Hypersplenism</p> Signup and view all the answers

    What is the peak age of incidence for Multiple Myeloma?

    <p>65-70 years</p> Signup and view all the answers

    Which feature of Multiple Myeloma contributes to the pathogenesis of bone destruction?

    <p>Upregulation of RANKL</p> Signup and view all the answers

    Which of the following is NOT commonly associated with Multiple Myeloma?

    <p>Increased lymphadenopathy</p> Signup and view all the answers

    The radiographic presentation of bone lesions in Multiple Myeloma is best described as:

    <p>Punched-out defects</p> Signup and view all the answers

    Study Notes

    Solitary Myeloma (Plasmacytoma)

    • 3-5% of cases present as a solitary lesion of bone or soft tissue
    • Bone lesions occur in the same locations as multiple myeloma
    • Extraosseous lesions occur in the lungs, oronasopharynx, or nasal sinuses
    • Modest elevations of M proteins in the blood or urine may be found in some patients
    • Solitary osseous plasmacytoma almost inevitably progresses to multiple myeloma, which can take 10-20 years or longer
    • Extraosseous plasmacytomas involving the upper respiratory tract are frequently cured by local resection

    Smoldering Myeloma

    • A middle ground between multiple myeloma and monoclonal gammopathy of uncertain significance (MGUS)
    • Plasma cells make up 10-30% of the marrow cellularity
    • Serum M protein level is >3 g/dL but patients are asymptomatic
    • About 75% of patients progress to multiple myeloma over a 15-year periods

    Multiple Myeloma

    • Clonal proliferation of neoplastic plasma cells in the bone marrow
    • A plasma cell neoplasm commonly associated with lytic bone lesions, hypercalcemia, renal failure, and acquired immune abnormalities
    • Incidence is higher in men and people of African descent
    • Peak age of incidence: 65-70 years old
    • Strictly a disease of older adults
    • Genetically heterogeneous
    • Associated with frequent rearrangements involving IGH locus on chromosome 14q32 and various proto-oncogenes
    • Proliferation and survival of myeloma cells are dependent on several cytokines, most notably IL-6
      • IL-6 is an important growth factor for plasma cells
      • IL-6 is produced by the tumor cells themselves and resident marrow stromal cells
      • High serum levels of IL-6 are seen in patients with active disease
      • High serum levels of IL-6 are associated with poor prognosis
    • Major pathological feature: bone destruction, which is mediated by factors produced by neoplastic plasma cells
      • Myeloma-derived MIP1α, chemokine that augments osteoclast formation
      • Myeloma-derived MIP1α upregulates the expression of the receptor activator of NF-kB ligand (RANKL) by bone marrow stromal cells
      • Net effect: marked increase in bone resorption leading to hypercalcemia and pathologic fractures
    • Modulators of the Wnt pathway are potent inhibitors of osteoblast function resulting in a net effect of marked increase in bone resorption leading to hypercalcemia and pathologic fractures

    Radiographic Presentation

    • Bone lesions appear radiographically as punched-out defects
      • Usually about 1-4 cm in diameter

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    Description

    This quiz covers the key topics of solitary myeloma, smoldering myeloma, and multiple myeloma. Learn about the clinical presentations, progression patterns, and pathological characteristics of these plasma cell disorders. Test your understanding of their differences and implications for patient management.

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