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Plasma Cell Disorders
196-200
10
Biology
Professional
12/27/2012

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Cards

Term
What are the classic clinical manifestations of Multiple Myeloma?
Definition
Neoplastic clonal proliferation of plasma cells with monoclonal Ig present in blood/urine.

Patients usually present with some combination of bone pain, fatique, parasthesia and weight loss.

1) Bone (80%)- spine most commonly
2) Anemia (60%)
3) Infections (20%)
4) Hypercalcemia (10%)
5) Renal involvement (20%)- Bence Jones protein
Term
57 year old male presents with severe back pain that is exacerbated by movement and improves at night. He also complains of dry mouth, nausea and some confusion.

What other questions would you ask this patient and how could you ultimately diagnose him?
Definition
Sounds like bone pain and dry mouth/nausea/confusion are characteristic of Hypercalcemia.

These are characteristic of MM, but not specific.

1) look for renal involvement (20%) of proximal or distal tubule, thoracic/lumbosacaral radiculopathy and anemia

2) Classic triad (need 2)
- > 10% abnormal plasma cells in bone marrow (Order CONVENTIONAL radiography and MRI for other lesions)

- Either osteolytic bone lesions or elevated serum and/or urine M protein to >3g/dl or >1g/dl, respectively
Term
What treatment options are available for multiple myeloma?
Definition
1) Immunomodulators (Thalidomide and lenalinomide)

2) Proteosome antagonist (Bortezomib)

3) Autologous stem cell transplant after high dose melphalan (NOT Allogeneic)
Term
Multiple myeloma is responsible for 1% of malignant disorders and makes up 10% of hematological malignancies. Which of the following is not a classic clinical feature seen in these patients?

1) Hypercalcemia from bone involvement
2) Bone pain
3) Hypercalcemia from increased parathyroid hormone related peptide.
4) Anemia
5) Acute renal impairment
6) Bleeding complications in IgA myeloma patient
Definition
3: PTHrP (parathyroid hormone related peptide) is a protein similar to parathyroid hormone which is produced at an ectopic site associated with a paraneoplastic hypercalcemia and malignancy. It is not the cause of the hypercalcemina seen in patients with multiple myeloma. Multiple myeloma, a cancer of plasma cells, is associated with excessive tumor-induced, osteoclast-mediated bone destruction.

Key mediators of the osteoclastic bone resorption in myeloma include receptor activator of nuclear factor-κB ligand (RANKL) and macrophage inflammatory protein-1α. Available data suggest that RANKL is the final common mediator of osteoclastic bone resorption.
Term
Diagnosis of Myeloma includes all of the following apart from:

1) 10% plasma cell involvement in bone marrow
2) Osteolytic bony lesions
3) Elevation in serum M protein >3g/dL
4) Elevation in urine M protein >3g/dL
Definition
4) > 1g/dl in urine
Term
T-99 Bone Scan can be used to visualize osteolytic bone lesions.

True or False
Definition
False: The radionuclide bone scan detects areas of the bone that has increased osteoblastic activity. This is often seen in areas of involvement with malignancy or healing fractures. A characteristic of multiple myeloma is that cortical bone involvement causes either osteoporosis or purely lytic lesions which cannot be detected by a radionuclide bone scan.
Term
How can one distinguish most easily between MM and MGUS?
Definition
**Smoldering can be more complicated, with M spike OR >10 % PC, but will not have bone symptoms

MGUS will have <3 g M spike, <10% PC in bone marrow and no anemia/lytic lesions/renal involvement

MM has to have >10% PC, >3g M spike (serum) or >1g M spike (urine), usually with bone involvement.
Term
What is the international staging system for symptomatic MM?
Definition
Based on serum B2 microglobin and albumin

1) B2M <3.5 and ALB >3.5
2) Not 1 or 3
3) B2M >5.5
Term
What is the most powerful adverse prognostic indicator for MM?
Definition
Deletion of chromosome 13 is the single most powerful adverse prognostic factor for all times to events in patients referred for high-dose therapy

Elevated B2M (especially above 5.5) is also useful
Term
Distinguish Waldenstrom’s Macroglobulinemia from MM.
Definition
Waldenstrom's

1) Bone marrow infiltration by plasmacytoid lymphocytes
2) High levels of IgM
3) No signs of myeloma
4) No bone disease
5) Lymphadenopathy or splenomegaly present in 40%
6) Serum viscosity elevated in 30% patients
7) Median survival 5 years
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