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Heme/Onco > Multipe Myeloma and Related Disorders > Flashcards

Multipe Myeloma and Related Disorders Flashcards

1
Q

What are the 5 different amylodoses?

A
  1. AL (immunoglobulin light-chain) amyloidosis
  2. Hereditary amyloidosis
  3. AA (secondary) amyloidosis
  4. Age-related (senile) amyloidosis
  5. Dialysis-related anyloidosis
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2
Q

AL amyloidosis

What is the amyloid protein?

A

Monoclonal free λ or κ chains

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3
Q

Hereditary amyloidosis

What is the amyloid protein?

A

Mutated thransthyretin (TTR), fibrinogen alpha chain

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4
Q

AA amyloidosis

What are the diseases associated with?

A

RA, IBD, familial mediterrancea fever, chronic infection

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5
Q

AA amyloidosis

What is the amyloid protein?

A

Seru amyloid A protein

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6
Q

Dialysis-related amyloidosis

What is the amyloid protein?

A

Beta-2 microglobulin

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7
Q

AL amyloidosis

What diseases are associated with?

A

MGUS
MM
Waldenstrom macroglobulinemia (rare)

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8
Q

Waldenstrom Macroglobulinemia

Where does it originate from?

A

B-cell NHL, production of an IgM λ or κ M protein

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9
Q

Waldenstrom Macroglobulinemia

Dx

A
  1. Neoplastic infiltrate consisting of clonal lymphocytes, plasmacytoid lymphocytes, plasma cells, and immunoblasts comprising 10% or more of the bone marrow cellularity;
  2. M protein >=3g/dL
  3. Symptomatic
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10
Q

Smoldering Waldenstrom Macroglobulinemia

Dx

A
  1. Neoplastic infiltrate consisting of clonal lymphocytes, plasmacytoid lymphocytes, plasma cells, and immunoblasts comprising 10% or more of the bone marrow cellularity;
    OR 2. M protein >=3g/dL
  2. Asymptomatic
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11
Q

Waldenstrom Macroglobulinemia

Sx:

A

“B symptoms”
Bleeding diathesis;
Hyperviscosity: HA, blurry vision, dizziness, tinnitus, hearing loss;
Sensorimotor polyneuropathy

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12
Q

Waldenstrom Macroglobulinemia

Why bleeding diathesis?

A
  1. Hyperviscosity;
  2. Plt dysfunction
  3. Hyperfibrinogenemia
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13
Q

Waldenstrom Macroglobulinemia

What the fundoscopy findings?

A

Retinal vein dilation;
Papilledema;
Flame hemorrhage

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14
Q

Waldenstrom Macroglobulinemia

What test to assess for prognosis?

A

Beta-2 microblogulin
Albumin
LDH

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15
Q

Waldenstrom Macroglobulinemia

Rx for hyperviscosity

A

If hyperviscosity: immediate plasmapheresis

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16
Q

Waldenstrom Macroglobulinemia

Rx

A

Inital: rituximab monotherapy or with chemo (either cyclophosphamide, bendamustine) or a purine analog (fludarabine, cladribine) with/w/o steroids
HSCT

17
Q

Waldenstrom Macroglobulinemia

Why sensorimotor polyneuropathy?

A

Antimyelin-associated glycoprotein acticity of the M protein

18
Q

Cryoglobulinemia

What are the 3 types?

A

Type I:

Type II and III:mixed

19
Q

Cryoglobulinemia

What are the disease associated with type I cryoglobulineia?

A

Underlying plasma cell dyscrasias or lymphoproliferative disorder (B cell NHL)

20
Q

Cryoglobulinemia

What are the disease associated with type II and III cryoglobulinemia?

A

Chronic infection
Autoimmunedisease
Underlying plasma cell dyscrasias or lymphoproliferative disorder (B cell NHL)

21
Q

Cryoglobulinemia

What is the most common disease associated with type II and III cryoglobulinemia?

A

Hepatitis C

22
Q

Cryoglobulinemia

Type I characteristics:

A

Monoclonal immunoglobulin (lgG, lgM, or lgA) with no RF activity

23
Q

Cryoglobulinemia

Type II characteristics:

A

Polyclonal immunoglobulins + monoclonal immunoglobulin (lgM, lgA) with RF activity

24
Q

Cryoglobulinemia

Type III characteristics:

A

Polyclonal immunoglobulins with RF activity

25
Q

Cryoglobulinemia

Type I associcated disease

A

PCDs and lymphoproliferative disorders (MGUS, multiple myeloma, Waldenstrom macroglobulinemia, B-cell non-Hodgkin lymphoma, CLL)

26
Q

Cryoglobulinemia

Type II associated diseases?

A

Infection (hepatitis C), connective tissue disease (Sjogren syndrome, SLE), lymphoproliferative disorders (Waldenstri:im macroglobulinemia, B-cell non-Hodgkin lymphoma, CLL)

27
Q

Cryoglobulinemia

Type III associated diseases?

A

Connective tissue disease,

lymphoproliferative disorders

28
Q

Cryoglobulinemia

Type I Symptoms and signs?

A 
Acrocyanosis;
Digital ischemia, livedo reticularis, skin ulcers and necrosis, lower extremity purpura;
Peripheral neuropathy (more common with types
II and Ill), hyperviscosity, arthralgia, membranoproliferative glomerulonephritis (more common with types II and Ill)
29
Q

Cryoglobulinemia

Type II and III Symptoms and signs?

A

Acrocyanosis;
Digital ischemia;
Fatigue;
Arthralgia;
Lower extremity purpura; skin ulcers and necrosis; Peripheral neuropathy;
Membranoproliferative glomerulonephritis;
Pulmonary, CNS, and gastrointestinal vasculitis

30
Q

Cryoglobulinemia

Rx for type I

A

If hyperviscosity: plasmapheresis

Underlying disease

31
Q

Cryoglobulinemia

Rx for type II and III?

A

Underlying disease

Immunosuppressive therapy for severe manifestations of vasculitis (rituximab/cyclophosphamide + steroids)

Heme/Onco (19 decks)

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