Plasma Cell Neoplasms Flashcards
All Plasma Cell Neoplasms are most common in what age group?
seen in older adults, not children
What is the most common plasma cell neoplasm?
Monoclonal Gammopathy of Undetermined Significance (MGUS)
What is the Serum M-protein level in a patient with MGUS?
<3g/dL
What is the clinical presentation of a patient with MGUS?
asymptomatic with no bony lesions or renal disease
What is Multiple Myeloma? What is its clinical triad?
malignant plasma cell neoplasm
- Clinical Triad
-
Monoclonal protein
- Serum M-protein >3 g/dL
- Urine M-protein > 500mg/24 hr
-
Monoclonal plasmacytosis (in BM)
- plasma cells = 10-60% BM cells
-
Disease-related organ damage (CRAB)
- Hypercalcemia
- Renal insufficiency
- Anemia
- Bone lesions
-
Monoclonal protein
Multiple Myeloma is commonly preceded by what condition?
MGUS
What risk factors are associated with Multiple Myeloma?
- Chronic antigenic stimulation (autoimmune, infectious, chronic inflammatory conditions)
-
Radiation / toxins
- herbicides, pesticides, asbestos
- rubber, plastic, wood, or petroleum
- Declining immune function
-
Genetic predisposition
- black:white = 2:1
What is clinical presentation of a patient with Multiple Myeloma?
- middle-aged to older African American
- asymptomatic to aggressive
- bone pain
- fatigue
- weight loss
- pallor
- infection
- bleeding
What type of end organ damage from Multiple Myeloma is shown in the provided image?
Kidney- myeloma cast neuropathy
- Fragmented pieces tubular cast (open arrow)
- Gian cell reaction (solid arrow)
The provided images are characteristic of what condition?
Multiple Myeloma
punched out lesion on skull & lytic lesions on X-Ray
What are the names of the cells indicated by the arrows in the provided images? They are seen in what condition?
Multiple Myeloma
- Left
- Dutcher bodies- myeloma cells with nuclear pseudoinclusions (cytoplasmic, but look like they are invaginating the nucleus)
- Right
- White arrow
- Russel Bodies- cytoplasmic immunoglobulins accumulations appearing as uniform, round, colorless globules
- Black arrow
- Mott cells- myeloma cells with multiple Russell bodies
- White arrow
Multiple Myeloma has what type of BM involvement?
patchy
What RBC pathology is commonly seen in Multiple Myeloma?
Rouleaux
What is the usual cause of death for a patient with Multiple Myeloma?
bleeding or infection
the malignant plasma cells outgrow & suppress the production of normal cells, which leads to impaired immunity and blood cytopenias
Multiple Myeloma may develop into what complicating conditions?
amyloidosis of AL types
What is the prognosis of a patient with Multiple Myeloma?
varies - few months to >10 yrs
What is Smoldering Myleloma? What percent progress to Multiple Myeloma?
asymptomatic myeloma with myeloma level plasmacytosis or M-protein
NO end-organ damage or lytic bone lesion (CREST)
75% progress over 15 yrs
What is primary amyloidosis?
extracellular deposition of fibrillar proteins with beta-pleated sheet tertiary structure
What is AL amyloid composed of?
immunoglobulin light chains
What is the clinical picture of a patient with Primary Amyloidosis?
- Older adult
- Symptoms
- organomegaly, purpura, bone pain, peripheral neuropathy, hemorrhage, CHF, neuropathic & malabsorption syndromes
What condition is depicted by the provided images?
Primary Amyloidosis
involved tissue may have waxy gross appearance
- Congo Red staining, & will have apple-green birefringence on polarized light microscopy (all forms amyloid)
What is a solitary plasmacytoma?
Solitary plasma cell neoplasm of monotypic plasma cells
What are the 2 forms of solitary plasmacytoma?
Intraosseous (intramedullary)
Extraosseous (extramedullary)
Solitary plasmacytomas most commonly affect what age group?
How are they differentiated from other myelomas?
middle-aged
-
NO clinical feature of myeloma
- polyclonal immunoglobulins are normal
- NO anemia, hypercalcemia, or renal failure
