Plasma cell dyscrasia DSA Flashcards

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)
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2
Q

Multiple Myeloma- bone dz due to?

A

-osteoclast hyperactivation

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3
Q

Multiple Myeloma- high paraprotein levels (monoclonal immunoglobulins)

A
  • hyperviscosity
  • light chain- kidney failure
  • infections- underprod of normal immunoglobulins
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4
Q

Multiple Myeloma- sx and signs

A
  • older adults
  • anemia, bone pain, kidney dz, infection
  • spinal cord compressoin- plasmacytoma
  • hyperviscosity syndrome
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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
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6
Q

Multiple Myeloma- imaging

A

-bone radiographs- lytic lesions- axial skeleton

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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
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8
Q

MM- initial therapy

A
  • immunomodulatory agent-thalidomide or lenalidomide
  • OR proteasome inhibitor- bortezomib
  • in combo with dexamethasone
  • common regimen- lenalidomide, bortezomib, dexamethasone
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9
Q

MM- after initial therapy

A
  • autologuos HSC transplantation

- localized radiotherapy for bone pain

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10
Q

MM- prognosis

A

-survival- >7 yrs

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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)
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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
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13
Q

spinal cord compression- imaging

A

-MRI- done in a cancer pt with new-onset back pain

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14
Q

spinal cord compression- tx

A
  • give corticosteroids immediately- dexamethasone

- emergent surgery

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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
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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
Q

Heavy Chain Diseases

A
  • 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
Q

y-HCD

A
  • 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
Q

alpha-HCD

A
  • 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
Q

cryoglobulinemia- is what?

A
  • cryoglobulins- plasma proteins that precipitate when cooled and dissolve when heated
  • type 1 (monoclonal)
  • type II (mixed monoclonal plus polyclonal)
  • type III (polyclonal)
29
Q

type 1 cryoglobulinemia

A
  • 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
Q

cryoglobulinemia- tx

A
  • rituximab with cyclophosphamide and dexamethasone

- only tx if have symptoms

31
Q

Primary amyloidosis- essentials of dx

A
  • 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
Q

Primary amyloidosis- categories

A
  • primary (light chain- AL)
  • secondary (serum protein A- infl conditions- AA)
  • hereditary (TTR)
  • senile
  • renal failure type (beta-2-microglobulin)
33
Q

Primary amyloidosis- signs and sx’s

A
  • 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
Q

Primary amyloidosis- lab

A
  • congo red positive amyloid protein on tissue bx

- kappa or lambda light chain paraprotein

35
Q

Primary amyloidosis- prognosis

A
  • 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