Plasma dyscrasias, WBC III, Putthoff, lec Flashcards Preview

Year 2 Heme lymph > Plasma dyscrasias, WBC III, Putthoff, lec > Flashcards

Flashcards in Plasma dyscrasias, WBC III, Putthoff, lec Deck (51):
1

What are the plasma cell neoplasms

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

2

bence jones proteins

light chains of Ig in the urine
indicative of multiple myeloma

3

rouleaux formation of RBC is indicative of what

multiple myeloma

4

Dx multiple myeloma

bone marrow exam

5

what two cancers may progress to multiple myeloma

solitary plasmacytoma
smoldering myeloma

6

what is the most common plasma cell dyscrasia

MGUS

7

Sx multiple myeloma

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

8

peak age incidence mutliple myeloma

65-70 yrs
men and african descent

9

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

more than 3 gm/dL serum Ig
more than 6 gm/dL bence jones

10

What are M proteins and most common in multiple myeloma?

Ig
IgG, then IgA

11

which Ig lead to hyperviscosity in multiple myelmoa

IgA or IgG3

12

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

no

13

CD markers for multiple myeloma

CD138 and adhesion molecule syndecan-1
CD56 commonly

14

What are Sx of hypercalcemia

confusion, weakness, lethargy, constipation and polyuria

15

decreased amounts of Ig clinically presents how

recurrent infections especially bacterial

16

how is the immune system in multiple myeloma

humoral affected greatly
cellular relatively unaffected

17

what causes the hypercalemia in multiple myeloma

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

18

which IL level is elevated in multiple myeloma

IL6

19

what is a solitary plasmacytoma

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

20

which plasmacytoma can be cured by resection

the extraosseous, usually involves upper respiratory tract

21

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

smoldering myeloma

22

What is next step for patient Dx with MGUS

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

23

Which B cell neoplasm terminally differentiates into a plasma cell

lymphoplasmacytic lymphoma

24

What neoplasms is assoc with MYD88 that activates NFKb

lymphoplasmacytic lymphoma

25

What is Waldenstrom macroglobulinemia

cause hyperviscosity syndrome, secretes monoclonal IgM

26

CD related to lymphoplasmacytic lymphoma

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

27

plasma cell dyscrasia that is secreting IgM

lymphoplasmacytic lymphoma

28

Sx of lymphoplasmacytic lymphoma

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

29

cancer associated with autoimmune hemolysis from cold agglutinins

lymphoplasmacytic lymphoma

30

Tx lymphoplasmacytic lymphoma

plasmapheresis, anti CD20 Ab

31

prognosis lymphoplasmacytic lymphoma

4 yr survival
incurable

32

distribution of mantle cell lymphoma

50-60 yrs old
male

33

11;14 translocation

mantle cell lymphoma

34

in mantle cell lymphoma what genes are affected

chrom 14 IgH locus
chrom 11 cyclin D1 overexpression

35

how is mantle cell lymphoma Dx

standard karyotyping and fluorescence in situ hybridization

36

what does upregulation cyclin D1 do

promotes G1 to S phase progression

37

clinical features of mantle cell lymphoma

painless lymphadenopathy
spleen and gut invovled

38

what are the variants of mantle cell lymphoma

blastoid and proliferative expression

39

what is prognosis of mantle cell lymphoma

3-4 yr because organ dysfunction from organ infiltration

40

how do you differentiate CLL and mantle cell lymphoma

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

41

lymphomatoid polyposis found in patient in colon
most likely Dx

mantle cell lymphoma

42

immunophenotype mante cell lymphoma

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

43

Tx mantle cell lymphoma

stem cell transplantation has shows promise

44

What are marginal cell lymphomas

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

45

what cancers have transformation from polyclonal to monoclonal

EBV induced lymphoma
marginal zone lymphoma

46

what are characteristics of marginal cell lymhomas

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

47

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

hairy cell leukemia

48

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

CD19 CD20
CD11c!!
CD25 CD103 and annexin A1

49

prognosis hairy cell leukemia

excellent indolent

50

what neoplasms resemble mature T cells and NK cells

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

51

peripehral T cell and NK neoplasms are more common where

T- Asia
NK- far east