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

Plasma dyscrasias, WBC III, Putthoff, lec Flashcards

1
Q

What are the plasma cell neoplasms

A 
multiple myeloma
lymphoplasmacytic lymphoma
monoclonal gammopathy of undetermined significance
heavy chain disease
primary or immunocyte-assoc amyloidosis
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2
Q

bence jones proteins

A

light chains of Ig in the urine

indicative of multiple myeloma

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3
Q

rouleaux formation of RBC is indicative of what

A

multiple myeloma

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4
Q

Dx multiple myeloma

A

bone marrow exam

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5
Q

what two cancers may progress to multiple myeloma

A

solitary plasmacytoma

smoldering myeloma

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6
Q

what is the most common plasma cell dyscrasia

A

MGUS

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7
Q

Sx multiple myeloma

A

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

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8
Q

peak age incidence mutliple myeloma

A

65-70 yrs

men and african descent

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9
Q

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

A

more than 3 gm/dL serum Ig

more than 6 gm/dL bence jones

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10
Q

What are M proteins and most common in multiple myeloma?

A

Ig

IgG, then IgA

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11
Q

which Ig lead to hyperviscosity in multiple myelmoa

A

IgA or IgG3

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12
Q

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

A

no

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13
Q

CD markers for multiple myeloma

A

CD138 and adhesion molecule syndecan-1

CD56 commonly

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14
Q

What are Sx of hypercalcemia

A

confusion, weakness, lethargy, constipation and polyuria

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15
Q

decreased amounts of Ig clinically presents how

A

recurrent infections especially bacterial

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16
Q

how is the immune system in multiple myeloma

A

humoral affected greatly

cellular relatively unaffected

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17
Q

what causes the hypercalemia in multiple myeloma

A

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

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18
Q

which IL level is elevated in multiple myeloma

A

IL6

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19
Q

what is a solitary plasmacytoma

A

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

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20
Q

which plasmacytoma can be cured by resection

A

the extraosseous, usually involves upper respiratory tract

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21
Q

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

A

smoldering myeloma

22
Q

What is next step for patient Dx with MGUS

A

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

23
Q

Which B cell neoplasm terminally differentiates into a plasma cell

A

lymphoplasmacytic lymphoma

24
Q

What neoplasms is assoc with MYD88 that activates NFKb

A

lymphoplasmacytic lymphoma

25
Q

What is Waldenstrom macroglobulinemia

A

cause hyperviscosity syndrome, secretes monoclonal IgM

26
Q

CD related to lymphoplasmacytic lymphoma

A

CD20, surface Ig

then when a plasma cell is IgM or IgG or IgA

27
Q

plasma cell dyscrasia that is secreting IgM

A

lymphoplasmacytic lymphoma

28
Q

Sx of lymphoplasmacytic lymphoma

A

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

29
Q

cancer associated with autoimmune hemolysis from cold agglutinins

A

lymphoplasmacytic lymphoma

30
Q

Tx lymphoplasmacytic lymphoma

A

plasmapheresis, anti CD20 Ab

31
Q

prognosis lymphoplasmacytic lymphoma

A

4 yr survival

incurable

32
Q

distribution of mantle cell lymphoma

A

50-60 yrs old

male

33
Q

11;14 translocation

A

mantle cell lymphoma

34
Q

in mantle cell lymphoma what genes are affected

A

chrom 14 IgH locus

chrom 11 cyclin D1 overexpression

35
Q

how is mantle cell lymphoma Dx

A

standard karyotyping and fluorescence in situ hybridization

36
Q

what does upregulation cyclin D1 do

A

promotes G1 to S phase progression

37
Q

clinical features of mantle cell lymphoma

A

painless lymphadenopathy

spleen and gut invovled

38
Q

what are the variants of mantle cell lymphoma

A

blastoid and proliferative expression

39
Q

what is prognosis of mantle cell lymphoma

A

3-4 yr because organ dysfunction from organ infiltration

40
Q

how do you differentiate CLL and mantle cell lymphoma

A

no proliferation centers in mantle cell lymphoma

also mantle cell lymphoma is CD23 -

41
Q

lymphomatoid polyposis found in patient in colon

most likely Dx

A

mantle cell lymphoma

42
Q

immunophenotype mante cell lymphoma

A

cyclin D1, CD19 CD20 IgM IgD

CD5 and CD23 -!!

43
Q

Tx mantle cell lymphoma

A

stem cell transplantation has shows promise

44
Q

What are marginal cell lymphomas

A

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

45
Q

what cancers have transformation from polyclonal to monoclonal

A

EBV induced lymphoma

marginal zone lymphoma

46
Q

what are characteristics of marginal cell lymhomas

A

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
Q

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

A

hairy cell leukemia

48
Q

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

A

CD19 CD20
CD11c!!
CD25 CD103 and annexin A1

49
Q

prognosis hairy cell leukemia

A

excellent indolent

50
Q

what neoplasms resemble mature T cells and NK cells

A 
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
Q

peripehral T cell and NK neoplasms are more common where

A

T- Asia

NK- far east

Year 2 Heme lymph (28 decks)

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