Multipe Myeloma and Related Disorders Flashcards Preview

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Flashcards in Multipe Myeloma and Related Disorders Deck (31):
1

What are the 5 different amylodoses?

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

2

AL amyloidosis
What is the amyloid protein?

Monoclonal free λ or κ chains

3

Hereditary amyloidosis
What is the amyloid protein?

Mutated thransthyretin (TTR), fibrinogen alpha chain

4

AA amyloidosis
What are the diseases associated with?

RA, IBD, familial mediterrancea fever, chronic infection

5

AA amyloidosis
What is the amyloid protein?

Seru amyloid A protein

6

Dialysis-related amyloidosis
What is the amyloid protein?

Beta-2 microglobulin

7

AL amyloidosis
What diseases are associated with?

MGUS
MM
Waldenstrom macroglobulinemia (rare)

8

Waldenstrom Macroglobulinemia
Where does it originate from?

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

9

Waldenstrom Macroglobulinemia
Dx

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

10

Smoldering Waldenstrom Macroglobulinemia
Dx

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
3. Asymptomatic

11

Waldenstrom Macroglobulinemia
Sx:

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

12

Waldenstrom Macroglobulinemia
Why bleeding diathesis?

1. Hyperviscosity;
2. Plt dysfunction
3. Hyperfibrinogenemia

13

Waldenstrom Macroglobulinemia
What the fundoscopy findings?

Retinal vein dilation;
Papilledema;
Flame hemorrhage

14

Waldenstrom Macroglobulinemia
What test to assess for prognosis?

Beta-2 microblogulin
Albumin
LDH

15

Waldenstrom Macroglobulinemia
Rx for hyperviscosity

If hyperviscosity: immediate plasmapheresis

16

Waldenstrom Macroglobulinemia
Rx

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

17

Waldenstrom Macroglobulinemia
Why sensorimotor polyneuropathy?

Antimyelin-associated glycoprotein acticity of the M protein

18

Cryoglobulinemia
What are the 3 types?

Type I:
Type II and III:mixed

19

Cryoglobulinemia
What are the disease associated with type I cryoglobulineia?

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

20

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

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

21

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

Hepatitis C

22

Cryoglobulinemia
Type I characteristics:

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

23

Cryoglobulinemia
Type II characteristics:

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

24

Cryoglobulinemia
Type III characteristics:

Polyclonal immunoglobulins with RF activity

25

Cryoglobulinemia
Type I associcated disease

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

26

Cryoglobulinemia
Type II associated diseases?

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

27

Cryoglobulinemia
Type III associated diseases?

Connective tissue disease,
lymphoproliferative disorders

28

Cryoglobulinemia
Type I Symptoms and signs?

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

Cryoglobulinemia
Type II and III Symptoms and signs?

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

30

Cryoglobulinemia
Rx for type I

If hyperviscosity: plasmapheresis
Underlying disease

31

Cryoglobulinemia
Rx for type II and III?

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