WBCII, Putthoff, lec Flashcards
What are the neoplasms of immature B and T cells
ALL from B cell or T cell
what is the most common childhood cancer
ALL, 85% B cell
where is precursor for B and T cell ALL
B from bone marrow
T from tymus
peak incidence of B and T cell ALL
B is age 3
T is adolescence when thymus is full size
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
what genotypes are assoc with T cell ALL
NOTCH1 mutations
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
what are the Sx specific to T cell ALL
complications from compression Large vessels and airways in the mediastinum
What Sx of ALL are from problems assoc with marrow
fatigue, fever, infecitons, bleeding
what Sx of ALL are assoc with neoplastic infiltration
bone pain
adenopathy
HSM
testicular involvement and the CNS Sx
What is distribution in poplation of ALL
more common in hispanics
3W:B
more male than female
what are good prognostic factors for ALL
between ages 2-10, low WBC, hyperdiploidy, trisomies 4,7,10 and t12;21
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
what do the nuclei look like on smear of ALL
spiculated nuclei
What CD marker is assoc with B cell neoplasms
CD 10
increased intracell terminal dinucleotide transferase TdT is assoc with what neoplasms
B and T cell ALL
most common leukemia in adults in westerm world?
CLL or SLL
What genotypes are assoc with CLL
trisomy 12
deletions:11q,13q,17p
What is immunophenotype CLL
CD19, CD20, CD23, CD5
low level Ig on surface
population affected by CLL
2M:1F
avg age 60
Sx CLL
easy fatiguability, weight loss, anorexia
generalized lymphadenopathy, HSM
increased infections
hypogammaglobulinemia
sometimes hemolytic anemia or thrombocytopenia from auto Ab
buzzwords for CLL
smudge cells, growth confined to proliferation centers, richter syndrome
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
What is absolute lymphocyte count in CLL
> 5000
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
