Plasma dyscrasias, WBC III, Putthoff, lec

Question 1

What are the plasma cell neoplasms

Answer 1

multiple myeloma
lymphoplasmacytic lymphoma
monoclonal gammopathy of undetermined significance
heavy chain disease
primary or immunocyte-assoc amyloidosis

Question 2

bence jones proteins

Answer 2

light chains of Ig in the urine

indicative of multiple myeloma

Question 3

rouleaux formation of RBC is indicative of what

Answer 3

multiple myeloma

Question 4

Dx multiple myeloma

Answer 4

bone marrow exam

Question 5

what two cancers may progress to multiple myeloma

Answer 5

solitary plasmacytoma

smoldering myeloma

Question 6

what is the most common plasma cell dyscrasia

Answer 6

MGUS

Question 7

Sx multiple myeloma

Answer 7

lytic bone lesions, hypercalcemia, renal failure and acquired immune abnormalities

Question 8

peak age incidence mutliple myeloma

Answer 8

65-70 yrs

men and african descent

Question 9

what levels of serium Ig and bence jones are assoc with multiple myeloma

Answer 9

more than 3 gm/dL serum Ig

more than 6 gm/dL bence jones

Question 10

What are M proteins and most common in multiple myeloma?

Answer 10

Ig

IgG, then IgA

Question 11

which Ig lead to hyperviscosity in multiple myelmoa

Answer 11

IgA or IgG3

Question 12

does the absence of detectable M proteins exclude Dx of multiple myeloma

Answer 12

no

Question 13

CD markers for multiple myeloma

Answer 13

CD138 and adhesion molecule syndecan-1

CD56 commonly

Question 14

What are Sx of hypercalcemia

Answer 14

confusion, weakness, lethargy, constipation and polyuria

Question 15

decreased amounts of Ig clinically presents how

Answer 15

recurrent infections especially bacterial

Question 16

how is the immune system in multiple myeloma

Answer 16

humoral affected greatly

cellular relatively unaffected

Question 17

what causes the hypercalemia in multiple myeloma

Answer 17

upregulate RANKL which activates osteoclast and release mediators that inhibit osteoblasts
increasing bone resorption and thus increase Ca [ ]

Question 18

which IL level is elevated in multiple myeloma

Answer 18

IL6

Question 19

what is a solitary plasmacytoma

Answer 19

ossesou that inevitably progresses to multiple myeloma over 10-20 yrs

Question 20

which plasmacytoma can be cured by resection

Answer 20

the extraosseous, usually involves upper respiratory tract

Question 21

asymptomatic patient with 30% plasma cells in marrow and serum M protein level above 3 gm/dL
Dx?

Answer 21

smoldering myeloma

Question 22

What is next step for patient Dx with MGUS

Answer 22

periodic assessment of serum M protein and bence jones becase may progress to full blown multiple myeloma

Question 23

Which B cell neoplasm terminally differentiates into a plasma cell

Answer 23

lymphoplasmacytic lymphoma

Question 24

What neoplasms is assoc with MYD88 that activates NFKb

Answer 24

lymphoplasmacytic lymphoma

Question 25

What is Waldenstrom macroglobulinemia

Answer 25

cause hyperviscosity syndrome, secretes monoclonal IgM

Question 26

CD related to lymphoplasmacytic lymphoma

Answer 26

CD20, surface Ig | then when a plasma cell is IgM or IgG or IgA

Question 27

plasma cell dyscrasia that is secreting IgM

Answer 27

lymphoplasmacytic lymphoma

Question 28

Sx of lymphoplasmacytic lymphoma

Answer 28

weakness, fatigue, Lb loss, HSM, lymphadenopathy, anemia

Question 29

cancer associated with autoimmune hemolysis from cold agglutinins

Answer 29

lymphoplasmacytic lymphoma

Question 30

Tx lymphoplasmacytic lymphoma

Answer 30

plasmapheresis, anti CD20 Ab

Question 31

prognosis lymphoplasmacytic lymphoma

Answer 31

4 yr survival | incurable

Question 32

distribution of mantle cell lymphoma

Answer 32

50-60 yrs old | male

Question 33

11;14 translocation

Answer 33

mantle cell lymphoma

Question 34

in mantle cell lymphoma what genes are affected

Answer 34

chrom 14 IgH locus | chrom 11 cyclin D1 overexpression

Question 35

how is mantle cell lymphoma Dx

Answer 35

standard karyotyping and fluorescence in situ hybridization

Question 36

what does upregulation cyclin D1 do

Answer 36

promotes G1 to S phase progression

Question 37

clinical features of mantle cell lymphoma

Answer 37

painless lymphadenopathy | spleen and gut invovled

Question 38

what are the variants of mantle cell lymphoma

Answer 38

blastoid and proliferative expression

Question 39

what is prognosis of mantle cell lymphoma

Answer 39

3-4 yr because organ dysfunction from organ infiltration

Question 40

how do you differentiate CLL and mantle cell lymphoma

Answer 40

no proliferation centers in mantle cell lymphoma | also mantle cell lymphoma is CD23 -

Question 41

lymphomatoid polyposis found in patient in colon | most likely Dx

Answer 41

mantle cell lymphoma

Question 42

immunophenotype mante cell lymphoma

Answer 42

cyclin D1, CD19 CD20 IgM IgD | CD5 and CD23 -!!

Question 43

Tx mantle cell lymphoma

Answer 43

stem cell transplantation has shows promise

Question 44

What are marginal cell lymphomas

Answer 44

B cell tumors that arise in lymph nodes, spleen or extranodal tissues

Question 45

what cancers have transformation from polyclonal to monoclonal

Answer 45

EBV induced lymphoma | marginal zone lymphoma

Question 46

what are characteristics of marginal cell lymhomas

Answer 46

arise in tissues with chronic inflammatino of autoimmune or infectious salivary- sjogren syndrome, salivary gland in hashimotos thyroiditis, stomach in helicobacter sp gastritis remain localized and may regress

Question 47

point mutation(valine to glutamate) in serine threnoin kinase BRAF

Answer 47

hairy cell leukemia

Question 48

massive splenomegaly in a 55 yr old white male with an atypical mycobacterial infection what CD will you lok for

Answer 48

CD19 CD20 CD11c!! CD25 CD103 and annexin A1

Question 49

prognosis hairy cell leukemia

Answer 49

excellent indolent

Question 50

what neoplasms resemble mature T cells and NK cells

Answer 50

``` peripheral T cell lymphoma Anaplastic large cell lymphoma adult T cell leukemia/lymphoma mycosis fungoides/sezary syndrome large granular lymphocytic leukemia extranodal NK/T cell lymphoma ```

Question 51

peripehral T cell and NK neoplasms are more common where

Answer 51

T- Asia | NK- far east