Plasma cell dyscrasia DSA Flashcards
(35 cards)
1
Q
Multiple Myeloma- essentials of dx
A
- bone pain- spine, ribs, proximal long bones
- monoclonal paraprotein by serum or urine protein electrophoresis or immunofixation
- clonal plasma cells in the bone marrow or in a tissue bx, or both
- organ damage due to plasma cells (bones, kidneys, hypercalcemia, anemia)
2
Q
Multiple Myeloma- bone dz due to?
A
-osteoclast hyperactivation
3
Q
Multiple Myeloma- high paraprotein levels (monoclonal immunoglobulins)
A
- hyperviscosity
- light chain- kidney failure
- infections- underprod of normal immunoglobulins
4
Q
Multiple Myeloma- sx and signs
A
- older adults
- anemia, bone pain, kidney dz, infection
- spinal cord compressoin- plasmacytoma
- hyperviscosity syndrome
5
Q
Multiple Myeloma- lab findings
A
- anemia
- paraprotein on serum or urine PEP (protein electrophoresis) or IFE (immunofixation electrophoresis)
- monoclonal spine in gamma- or beta- region
- mostly IgG
6
Q
Multiple Myeloma- imaging
A
-bone radiographs- lytic lesions- axial skeleton
7
Q
MGUS vs MM
A
- bone marrow monoclonal plasma cells < 10% in the setting of a paraprotein < 3
- 1% transform to MM per year
8
Q
MM- initial therapy
A
- immunomodulatory agent-thalidomide or lenalidomide
- OR proteasome inhibitor- bortezomib
- in combo with dexamethasone
- common regimen- lenalidomide, bortezomib, dexamethasone
9
Q
MM- after initial therapy
A
- autologuos HSC transplantation
- localized radiotherapy for bone pain
10
Q
MM- prognosis
A
-survival- >7 yrs
11
Q
MM- international staging sytem
A
relies on beta-2-microglobulin and albumin
- 1- b2m < 3.5 and albumin > 3.5 (Survival > 5 yrs)
- 2- b/w 1 and 3
- 3- b2m > 5.5 (survival < 2 yrs)
12
Q
spinal cord compression- essentials of dx
A
- complication of metastatic solid tumor, lymphoma, or MM
- back pain- most common sx
- prompt dx essential- if a severe neuro deficit develops, it is often irreversible
- emergent tx- can prevent/reverse paresis and urinary/bowel incontinence
13
Q
spinal cord compression- imaging
A
-MRI- done in a cancer pt with new-onset back pain
14
Q
spinal cord compression- tx
A
- give corticosteroids immediately- dexamethasone
- emergent surgery
15
Q
Hypercalcemia- essentials of dx
A
- usually symptomatic and severe (15 or more)
- most common paraneoplastic endocrine syndrome
- neoplasm is clinically apparent in nearly all cases when hypercalcemia is detected
16
Q
mech for hypercalcemia from myeloma vs from solid tumors
A
- myeloma- osteolysis of bone
- solid tumor- systemic effects of tumor-released proteins, direct osteolysis of bone by tumor or vit-D mediated osteoabs
17
Q
hypercalcemia- sx and signs
A
- anorexia, nausea, fatigue, constipation, polyuria
- m weakness, hyporeflexia, confusion, tremor, lethargy
18
Q
MGUS- types
A
- non-IgM- most common- can progress to smoldering MM or MM
- IgM- 15%- progress to Waldenstrom macroglobulinemia
- light chain- progress to Bence Jones proteinuria and light chain MM
19
Q
MGUS
A
- asymptomatic pt with a serum monoclonal immunoglobulin < 3
- metastatic bone survey!
20
Q
MGUS- diagnostic criteria
A
- serum monoclonal protein < 3
- <10% plasma cells in BM
- no sx- absence of lytic bone lesions, anemia, hypercalcemia, and renal insuff
- no leukocytosis, abnormal circulating cells, LA, hepatosplenomegaly, or undx mass lesions
21
Q
Waldenstrom Macroglobulinemia- essentials of dx
A
- monoclonal IgM paraprotein
- infiltration of bone marrow by plasmacytic lymphocytes
- absence of lytic bone dz
22
Q
Waldenstrom Macroglobulinemia- sx and signs
A
- in 60s and 70s
- fatigue- anemia
- hyperviscosity of serum- nausea, vertigo, visual disturbances, cold agglutinin dz
23
Q
Waldenstrom Macroglobulinemia- lab findings
A
- bone marrow- infiltrated by plasmacytic lymphocytes
- hallmark- monoclonal IgM spike
- serum hyperviscosity
24
Q
Waldenstrom Macroglobulinemia- tx
A
-when have hyperviscosity syndrome- plasmapheresis
25
Heavy Chain Diseases
- presence of an M protein consisting of a portion of the immunoglobulin heavy chain in serum, urine, or both
- 3 types- y-HCD, alpha-HCD, and u-HCD
26
y-HCD
- lymphoma-like illness
- hepatosplenomegaly and LA- in 60% of pts
- anemia
- dx- isolated monoclonal y heavy chain on serum immunofixation, without evidence of K or λ light chain expression
27
alpha-HCD
- most common form- Middle East pts; 2-3 decade of life
- GI tract most commonly involved- diarrhea, steatorrhea, weight loss
- plasma cell infiltration of jejunal mucosa
- dx- monoclonal alpha heavy chain on serum immunofixation
- progressive and fatal without therapy
28
cryoglobulinemia- is what?
- cryoglobulins- plasma proteins that precipitate when cooled and dissolve when heated
- type 1 (monoclonal)
- type II (mixed monoclonal plus polyclonal)
- type III (polyclonal)
29
type 1 cryoglobulinemia
- assoc with macroglobulinemia, MM, or MGUS
- mostly asymptomatic
- if in range 1-2- vasculitis with pain, purpura, raynaud, cyanosis, ulceration on exposure to cold
30
cryoglobulinemia- tx
- rituximab with cyclophosphamide and dexamethasone
| - only tx if have symptoms
31
Primary amyloidosis- essentials of dx
- congo red positive amyloid protein on tissue bx
- amyloid protein is kappa or lambda immunoglobulin light chain
- serum or urine (or both) light chain paraprotein
32
Primary amyloidosis- categories
- primary (light chain- AL)
- secondary (serum protein A- infl conditions- AA)
- hereditary (TTR)
- senile
- renal failure type (beta-2-microglobulin)
33
Primary amyloidosis- signs and sx's
- localized- single affected organ
- systemic- HF, nephrotic syndrome, malabs and weight loss, hepatic dysfxn, autonomic insuff, carpal tunnel syndrome, sensorimotor peripheral neuropathy
- dx made late in the dz porcess
34
Primary amyloidosis- lab
- congo red positive amyloid protein on tissue bx
| - kappa or lambda light chain paraprotein
35
Primary amyloidosis- prognosis
- untreated- progressive end-organ failure and death
- cardiac or autonomic n presentations- survive 3-9 months
- carpal tunnel syndrome or nephrosis- 1.5-3 yrs
- peripheral neuropathy- 5 yrs
- MM-like tx- doubles survival
