WBCII, Putthoff, lec Flashcards by Amanda Beach

What are the neoplasms of immature B and T cells

ALL from B cell or T cell

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what is the most common childhood cancer

ALL, 85% B cell

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where is precursor for B and T cell ALL

B from bone marrow

T from tymus

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peak incidence of B and T cell ALL

B is age 3

T is adolescence when thymus is full size

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what genotypes are assoc with B cell ALL

t(12;21) involves RUNX1 and ETV6
trisomy 4 7 and 10
philedelphia chromosome 9;22
hyperdiploidy

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what genotypes are assoc with T cell ALL

NOTCH1 mutations

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What are clinical features of ALL

abrupt onset Sx
fatigue, fever, infections, bleeding
bone pain, lymphadenopathy, HSM, testicular enlargement
HA, vomiting, nerve palsies from meningeal involvemnt

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what are the Sx specific to T cell ALL

complications from compression Large vessels and airways in the mediastinum

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What Sx of ALL are from problems assoc with marrow

fatigue, fever, infecitons, bleeding

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what Sx of ALL are assoc with neoplastic infiltration

bone pain
adenopathy
HSM
testicular involvement and the CNS Sx

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What is distribution in poplation of ALL

more common in hispanics
3W:B
more male than female

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what are good prognostic factors for ALL

between ages 2-10, low WBC, hyperdiploidy, trisomies 4,7,10 and t12;21

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what are bad prognostic factors of ALL

age less than 2 or greater than 10
peripheral blast count >100,000
the philadelphia translocation 9;22 BCR/-ABL fusion

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what do the nuclei look like on smear of ALL

spiculated nuclei

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What CD marker is assoc with B cell neoplasms

CD 10

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increased intracell terminal dinucleotide transferase TdT is assoc with what neoplasms

B and T cell ALL

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most common leukemia in adults in westerm world?

CLL or SLL

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What genotypes are assoc with CLL

trisomy 12

deletions:11q,13q,17p

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What is immunophenotype CLL

CD19, CD20, CD23, CD5

low level Ig on surface

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population affected by CLL

2M:1F

avg age 60

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Sx CLL

easy fatiguability, weight loss, anorexia
generalized lymphadenopathy, HSM
increased infections
hypogammaglobulinemia

sometimes hemolytic anemia or thrombocytopenia from auto Ab

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buzzwords for CLL

smudge cells, growth confined to proliferation centers, richter syndrome

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what is richter syndrome

the transformation of CLL to diffuse large B cell lymphoma or a prolymphocyte evolution
aggressive with less than 1 yr survivial

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What is absolute lymphocyte count in CLL

> 5000

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how do you Dx CLL

peripheral blood smear

Prognosis CLL

4-6 yrs. can be >10 if mild

Tx CLL

gentle chemo and immunotherapy with Ab to CD20 or inhibitors of BTK

What is the most common indolent NHL in US

follicular lymphoma

Where are follicular lymphomas from

germinal center B cells

genotypes assoc with follicular lymphoma

t14;18 which causes the BCL2-IgH fusion that causes overexpression and increased inhibition of apoptosis

population affected by follicular lymphomas

middle age males | rare in Asia

What is histo characteristics of follicular lymphomas

no apoptotic cells, follicular cells everywehre increased centrocytes and centroblasts lymphocytosis <20,000

Immunophenotype follicular lymphoma

CD 19, CD 20, CD10, surface Ig, BCL6!

what are Sx of follicular lymphoma

generalized painless lymphadenopathy | extranodal sites like GI CNS and testis is rare

Prognosis of follicular lymphoma

7-9 yrs

Tx follicular lymphoma

mild chemo, mainly palliative

Transofrmation of follicular lymphoma

diffuse large B cell lymphoma and tymor that resembles Burkitt lymphoma

mean survival if follicular lymphoma transforms

less than 1yr

what is the most common non hodgkin lymphoma

diffuse large B cell lymphoma

where are diffuse large B cell lymphomas derived from

germinal and post germinal

what mutations are assoc with diffuse large B cell lymphomas

BCL6 dysregulation chrom 3q27

how are the BCL2 dysregulation of diffuse large B cell differentiated from follicular lyphomas

there is absolutely no follicular formation

What are the subsets of diffuse large cell B lymphoma

``` immunodeficiency associated (EBV) primary pleural effusion lymphoma (KSHV/HHV8) ```

what will a slide looks like of large diffuse B cell lymphoma

large cell size with diffuse pattern of growth round/oval nucleus that looks vesicular nucleoli present abundant cytoplasm

immunophenotype of large diffuse b cell lymphoma

CD19 and 20 CD 10 and BCL6 most with surface Ig

what is the clinical presentation of diffuse large b cell lymphoma

rapidly enlarging mass at nodal and extranodal sites

where are common sites of diffuse large B cell lymphoma

waldeyer ring oropharyngeal lymph GI, skin, bones, brain

Prognosis of diffuse large b cell lymphoma

rapidly fatal without Tx

Tx diffuse large B cell lymphoma

aggressive chemo with Ab CD20

age and sex affected by diffuse large b cell lymphoma

male age 60 can occur in childhood and adolescence

Where are the cells derived from in burkitt lymphoma

germinal center B cells

mutations associated with burkitts lymphoma

t8;14 | MYC and IgH or even kappa and gamma light chains

What is the role of the MYC gene

increases expression of gene for aerobic glycolysis "warburg effect"

What is the subset of burkitt lymphoma

EBV associated

what are the categories of burkitt lymphoma

african (endemic) sporadic (nonendemic) HIV (aggressive)

which category of burkitt lymphoma has a mass that invades mandible commonly

endemic (african)

which category of burkitt lymphoma has a mass that invades ileocecum and peritoneum commonly

sporadic (nonendemic)

what is distribution of burkitt lymphoma

30% NHL childhood tumors | any age really

what is the fastest growing human tumor

burkitt lymphoma

what will histo look like of burkitt lymphoma

``` diffuse infiltrate of med size lymphoid cells several nucleoli, coarse chromatin high mitotic index many apoptotic cells "starry sky" phagocytes ```

what will histo of burkitt taken from bone marrow look like

nucleoli with royal blue cytoplasm with clear vacuoles

What does starry sky phagocytes look like

abundant clear cytoplasm

what is the immunophenotype of burkitt lymphoma

IgM,CD19 | CD20 CD10 BCL6

what surface marker is not expressed on burkitt even though it is a germinal tumor

BCL2

Tx burkitt lymphoma? prognosis

intensive chemo | most children cured, adults not as much

class II MHC molecules are expressed on what

macrophages, B lymphocytes and dendritic cells

role of B lymphocyte

neutralization of microbe, phagocytosis and C' activation

roles of helper T cells

activation of macrophages inflammation activation of T and B cells

how do T cells rapidly enter parenchyma & become memory

high endothelial venule