Multipe Myeloma and Related Disorders

Question 1

What are the 5 different amylodoses?

Answer 1

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

Question 2

AL amyloidosis

What is the amyloid protein?

Answer 2

Monoclonal free λ or κ chains

Question 3

Hereditary amyloidosis

What is the amyloid protein?

Answer 3

Mutated thransthyretin (TTR), fibrinogen alpha chain

Question 4

AA amyloidosis

What are the diseases associated with?

Answer 4

RA, IBD, familial mediterrancea fever, chronic infection

Question 5

AA amyloidosis

What is the amyloid protein?

Answer 5

Seru amyloid A protein

Question 6

Dialysis-related amyloidosis

What is the amyloid protein?

Answer 6

Beta-2 microglobulin

Question 7

AL amyloidosis

What diseases are associated with?

Answer 7

MGUS
MM
Waldenstrom macroglobulinemia (rare)

Question 8

Waldenstrom Macroglobulinemia

Where does it originate from?

Answer 8

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

Question 9

Waldenstrom Macroglobulinemia

Dx

Answer 9

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

Question 10

Smoldering Waldenstrom Macroglobulinemia

Dx

Answer 10

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

Question 11

Waldenstrom Macroglobulinemia

Sx:

Answer 11

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

Question 12

Waldenstrom Macroglobulinemia

Why bleeding diathesis?

Answer 12

1. Hyperviscosity;
2. Plt dysfunction
3. Hyperfibrinogenemia

Question 13

Waldenstrom Macroglobulinemia

What the fundoscopy findings?

Answer 13

Retinal vein dilation;
Papilledema;
Flame hemorrhage

Question 14

Waldenstrom Macroglobulinemia

What test to assess for prognosis?

Answer 14

Beta-2 microblogulin
Albumin
LDH

Question 15

Waldenstrom Macroglobulinemia

Rx for hyperviscosity

Answer 15

If hyperviscosity: immediate plasmapheresis

Question 16

Waldenstrom Macroglobulinemia

Rx

Answer 16

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

Question 17

Waldenstrom Macroglobulinemia

Why sensorimotor polyneuropathy?

Answer 17

Antimyelin-associated glycoprotein acticity of the M protein

Question 18

Cryoglobulinemia

What are the 3 types?

Answer 18

Type I:

Type II and III:mixed

Question 19

Cryoglobulinemia

What are the disease associated with type I cryoglobulineia?

Answer 19

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

Question 20

Cryoglobulinemia

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

Answer 20

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

Question 21

Cryoglobulinemia

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

Answer 21

Hepatitis C

Question 22

Cryoglobulinemia

Type I characteristics:

Answer 22

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

Question 23

Cryoglobulinemia

Type II characteristics:

Answer 23

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

Question 24

Cryoglobulinemia

Type III characteristics:

Answer 24

Polyclonal immunoglobulins with RF activity

Question 25

Cryoglobulinemia | Type I associcated disease

Answer 25

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

Question 26

Cryoglobulinemia | Type II associated diseases?

Answer 26

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

Question 27

Cryoglobulinemia | Type III associated diseases?

Answer 27

Connective tissue disease, | lymphoproliferative disorders

Question 28

Cryoglobulinemia | Type I Symptoms and signs?

Answer 28

``` 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) ```

Question 29

Cryoglobulinemia | Type II and III Symptoms and signs?

Answer 29

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

Question 30

Cryoglobulinemia | Rx for type I

Answer 30

If hyperviscosity: plasmapheresis | Underlying disease

Question 31

Cryoglobulinemia | Rx for type II and III?

Answer 31

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