Plasma Cell Disorders

What are plasma cells?

-what happens during plasma cell disorders

B cells in the bone marrow that secrete antibodies

-in plasma cell disorders, the antibodies are not functional & are unable to fight infections

What immunoglobulin is abundant in Multiple Myeloma (MM)?

IgG Protein M

-AKA myeloma proteins, or paraproteins

What are the clinical manifestations of Multiple Myeloma (MM)? (6)

-Bone pain (esp. in low back or ribs)

-Bone fractures & lytic changes

-Anemia (overproduction of plasma cells decreased RBC count)

-Hypercalcemia

-Renal failure

-Hypercoagulablity (increased plasma cells increases viscosity of blood)

What is the function of Serum Protein Electrophoresis (SPEP) and Immunofixation (IFE) in the evaluation of Multiple Myeloma (MM)?

SPEP: separates proteins by size (detects abnormality)

IFE: detects WHICH immunoglobulin is abnormal (IgG M in MM)

What lab findings are common in patients with Multiple Myeloma (MM)?

-52% IgG (mostly protein M)

-21% IgA

-16% Light chain only (Bence-Jones) in urine

-Increased total protein

-Hypercalcemia

-Anemia

What is present on a Multiple Myeloma (MM) peripheral smear?

RBC Rouleaux formation

True or False: Bone scans are useful in assessing Multiple Myeloma (MM).

-explain

False

-bone scans measure osteoBlastic components, but MM does NOT have osteoBlastic components

-osteoClasts are in control in MM

What is the diagnostic criteria for Multiple Myeloma (MM)? (3)

1. Presence of plasma cells ≥ 10% in bone marrow

2. Monoclonal protein in serum or urine (Bence Jones)

3. Evidence of end-organ damage (CRAB)

-calcium elevation

-renal insufficiency

-anemia

-bone lytic lesions

In Multiple Myeloma (MM), as levels of Beta-2 microglobulin _________________, prognosis ________________.

increase

decreases

high levels associated with poorer prognosis

What is the role of Bisphosphonates in Multiple Myeloma (MM) treatment?

-What is a risk of using bisphosphonates?

prevent bone reabsorption

-plasma cells reabsorb bone, causing further growth of myeloma

-Bisphosphonates get incorporated into the bone & prevent this

-stop osteoclasts from taking over

can cause osteonecrosis of the jaw

-make sure pt sees dentist before starting therapy

What is the difference between osteoblasts & osteoclasts?

blasts: build bone

clasts: destroy bone

What is the role of thalidomide in the treatment of Multiple Myeloma (MM)?

-what risks does it pose?

-what are its derivatives? (2)

inhibits blood vessel formation & can starve tumor of blood supply

-congenital limb abnormalities

-Lenalidomide & Pomalidomide

What is the risk of using Bortezomib in treatment of Multiple Myeloma (MM)?

can cause peripheral neuropathy

-stop tx if occurs

What is the process for an Autologous bone marrow transplant in the treatment of Multiple Myeloma (MM)?

-what kind of patients are not eligible for this?

"self" BMT

-take patient's own BM & treat with high dose chemo

-then give patient back the healthy stem cell

patients > 80 are not eligible

What is the diagnostic criteria for Monoclonal Gammopathy of Undetermined Significance (MGUS)? (3)

all 3 must be met:

1. Serum monoclonal protein < 3 g/dL

2. Clonal bone marrow plasma cells < 10%

3. Absence of end-organ damage (no CRAB)

Monoclonal Gammopathy of Undetermined Significance (MGUS) is often a precursor to...

Multiple myeloma

What is the treatment for Monoclonal Gammopathy of Undetermined Significance (MGUS)?

-what tests should be done to monitor it?

observation only

Monitoring: repeat every 6 months; if stable, can be done yearly

-electrophoresis

-CBC

-creatinine

-calcium

Which immunoglobulin is secreted in Waldenstrom Macroglobulinemia (WM)

IgM M

-only disorder that secretes IgM!!!

Waldenstrom Macroglobulinemia (WM) commonly presents similarly to...

-give examples of presentations

platelet problems

-bleeding

-bruising

-fatigue

-weight loss

-anemia

peripheral neuropathy can occur in WM