Plasma cell dyscrasia DSA Flashcards
Multiple Myeloma- essentials of dx
- 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)
Multiple Myeloma- bone dz due to?
-osteoclast hyperactivation
Multiple Myeloma- high paraprotein levels (monoclonal immunoglobulins)
- hyperviscosity
- light chain- kidney failure
- infections- underprod of normal immunoglobulins
Multiple Myeloma- sx and signs
- older adults
- anemia, bone pain, kidney dz, infection
- spinal cord compressoin- plasmacytoma
- hyperviscosity syndrome
Multiple Myeloma- lab findings
- anemia
- paraprotein on serum or urine PEP (protein electrophoresis) or IFE (immunofixation electrophoresis)
- monoclonal spine in gamma- or beta- region
- mostly IgG
Multiple Myeloma- imaging
-bone radiographs- lytic lesions- axial skeleton
MGUS vs MM
- bone marrow monoclonal plasma cells < 10% in the setting of a paraprotein < 3
- 1% transform to MM per year
MM- initial therapy
- immunomodulatory agent-thalidomide or lenalidomide
- OR proteasome inhibitor- bortezomib
- in combo with dexamethasone
- common regimen- lenalidomide, bortezomib, dexamethasone
MM- after initial therapy
- autologuos HSC transplantation
- localized radiotherapy for bone pain
MM- prognosis
-survival- >7 yrs
MM- international staging sytem
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)
spinal cord compression- essentials of dx
- 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
spinal cord compression- imaging
-MRI- done in a cancer pt with new-onset back pain
spinal cord compression- tx
- give corticosteroids immediately- dexamethasone
- emergent surgery
Hypercalcemia- essentials of dx
- usually symptomatic and severe (15 or more)
- most common paraneoplastic endocrine syndrome
- neoplasm is clinically apparent in nearly all cases when hypercalcemia is detected
mech for hypercalcemia from myeloma vs from solid tumors
- myeloma- osteolysis of bone
- solid tumor- systemic effects of tumor-released proteins, direct osteolysis of bone by tumor or vit-D mediated osteoabs
hypercalcemia- sx and signs
- anorexia, nausea, fatigue, constipation, polyuria
- m weakness, hyporeflexia, confusion, tremor, lethargy
MGUS- types
- 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
MGUS
- asymptomatic pt with a serum monoclonal immunoglobulin < 3
- metastatic bone survey!
MGUS- diagnostic criteria
- 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
Waldenstrom Macroglobulinemia- essentials of dx
- monoclonal IgM paraprotein
- infiltration of bone marrow by plasmacytic lymphocytes
- absence of lytic bone dz
Waldenstrom Macroglobulinemia- sx and signs
- in 60s and 70s
- fatigue- anemia
- hyperviscosity of serum- nausea, vertigo, visual disturbances, cold agglutinin dz
Waldenstrom Macroglobulinemia- lab findings
- bone marrow- infiltrated by plasmacytic lymphocytes
- hallmark- monoclonal IgM spike
- serum hyperviscosity
Waldenstrom Macroglobulinemia- tx
-when have hyperviscosity syndrome- plasmapheresis
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
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
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
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)
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
cryoglobulinemia- tx
- rituximab with cyclophosphamide and dexamethasone
- only tx if have symptoms
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
Primary amyloidosis- categories
- primary (light chain- AL)
- secondary (serum protein A- infl conditions- AA)
- hereditary (TTR)
- senile
- renal failure type (beta-2-microglobulin)
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
Primary amyloidosis- lab
- congo red positive amyloid protein on tissue bx
- kappa or lambda light chain paraprotein
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
