Precursor B and T cells neoplasms/ mature B and T 2 Flashcards
1
Q
Most ALL are what type of cells
A
pre-B cells
2
Q
pre-B ALL and pre-T ALL which ones are usually leukemic or lymphomic
A
B: leukemic
T: lymphomic
3
Q
what is the common cancer of Children
A
ALL
4
Q
at what age is Pre-B ALL have the highest incidence
A
4
5
Q
at what age is Pre-T ALL have the highest incidence
A
adolescence
6
Q
when do the symptoms appear for precursor B- and T- cell neoplasms
A
“stormy” onset
7
Q
If a child says they have bone pain, what should be in your differential diagnosis
A
ALL
8
Q
Pre-T neoplasm is associated with what other parts of the body
A
- airway compression ( medastinal mass)
- testicular involvement
9
Q
what do ALL cells look like? what do they not have?
A
high N:C ratio
irregular nucleus
- no MPO
10
Q
what do both precursor B- and T- cell neoplasms have on their immunophenotype
A
CD34
TdT
11
Q
CD 19, 22, 10
A
Immunophenotpye of pre-B
12
Q
CD 1a, 2, 5, 7
A
Immunophenotpye of pre-T
13
Q
what are the two cytogenetic abnormality for Pre-B? who usually gets which one
A
T (12;21) children
T (9;22) adults
14
Q
ALL prognosis for children
A
good
15
Q
ALL prognosis for adults
A
not good
16
Q
how is ALL treated
A
aggressive chemo plus CNS prophylaxis
17
Q
what has improved prognosis in t(9;22) in pre-B ALL
A
BCR-ABL Tyrosine kinase inhibitor therapy
18
Q
what is the most common leukemia of adults in western world
A
CLL/SLL
19
Q
CLL/SLL which one is leukemic and which one is lymphomatous
A
CLL: leukemic
SLL: lymphomatous
20
Q
Who usually presents with CLL/SLL
A
over 50 years of age
21
Q
what are symptoms for CLL/SLL
A
a symptomatic
22
Q
name 3 complications of CLL/SLL
A
- hypogammaglobulinemia
- autoimmune hemolytic anemia
- Richter transformation
23
Q
what does the histo slide look like with CLL
A
smudge cells
hyperclumped nuclear chromatin
lymphadenopathy
24
Q
what does the histo slide look like for SLL
A
proliferation centers mimic germinal centers
25
CD 19,5,23
| CD 20- dim surface light chain restricted
immunophenotype for CLL/SLL
26
what is Richter transformation
transforms to large B-cell lymphoma
| -enlarging lymph node or spleen
27
what is the prognosis for CLL/SLL
poor, die from other problems
28
Follicular lymphoma strongly associated with
translocations involving BCL2
29
Characteristic of lymph nodes for follicular lymphoma
painless lymphadenopathy
30
what's the involvement of BM and PB for follicular lymphoma
BM involved
| PB not
31
what are 2 cell types in follicluar lymphoma in lypms
centrocytes: small cleaved cells
centroblasts: larger cells; count for grading
32
what is seen in the spleem for follicular lymphoma
white pulp; portal tracts
33
CD 19, 20, 10
surface light chain
BCL2
blc6
what is the immunophenotype for follicular lymphoma
34
what is the cytogenetics of follicular lymphoma? what is on each chromosome
t ( 14;18)
14- IgH
18- BCL2
35
prognosis for follicular lymphoma?
incurable
| - transformation to diffuse large b cell lymphoma
36
for mantle cell lymphoma, tumor cells express what
CD 5
| over express cyclin D1
37
what is the cytogenetics of mantle cell lympohma
t (11;14)
38
what is the PB and MB involvement for mantle cell lymphoma
PB: varies
BM: most involvement
39
what is characteristic of the lymph nodes in mantle cell lymphoma
painless, generalized lemphadenopathy
40
mantle cell lymphoma involves what other organ disorder
lymphomatoid polyposis= GI invovlment
41
CD 19, 20, 5
| bright surface light chain
what is the immunophenotype for mantle cell lymphoma
42
Immunophenotype for mantle cell lymphoma is negative for
CD 23
43
what is the cytogenetics for mantle cell lymphoma? what do each chromosome express?
t(11;14)
11: cyclin D1
14: IgH
44
what does increased expression of cyclin D1 in mantle cell lymphoma do to cell
promotes G1 to S phase
45
what is the prognosis for mantle cell lymphoma
not curable
46
what is the treatment for mantle cell lymphoma
Retuximab and chemotherapy
47
marginal zone lymphoma is formed by
post-germinal center memory B cells
48
marginal zone lymphoma is involved in what type of disorders
chronic inflammatory
49
histo what does morginal zone lymphoma have?
monocytoid B-cells
| plasmacytoid celld
50
CD19, 20
| surface light chain
Immunophenotype for marginal zone lymphoma
51
what is the cytogenetics for marginal zone lyphoma
t ( 11;18) or t (11;14)
52
what does lymphoplasacytic lymphoma secrete
monoclonal IgM
| -Waldenstorm macroglobulinemia ( hyper viscosity)
53
what are symptoms for waldenstorm's macroglobulinemia from the IgM
visual
neurologic
bleeding
cryoglobulinemia (IgM precipitates at low temp)
54
how do you alleviate symptoms of IgM from Waldenstorm's macroglobulinemia
plasmapheresis
55
lymphoplasmacytic lymphoma is a mutation in what
MYD88
56
Hairy cell leukemia is a mutation in what
BRAF
57
what does hairy cell leukemia involve
PB
BM
red pulp spleen
58
clinical features for hairy cell leukemia
splenomegaly
pancytopenia
monocytopenia
cytopenias
59
what's the bone tap for hairy cell leukemia
dry tap
60
what is the histo for hairy cell leukemia
diffuse fried eggs
61
CD 19, 20
surface light chain
CD 11c, 25, 103 ( diagnostic)
Immunophenotype for hairy cell leukemia
62
what do both precursor B- and T- cell neoplasms not have on their immunophenotype
surface light chain
63
what do plasma cell neoplasms secrete
M-component
| Bence Jones protien
64
what are 3 important things for multiple myelome
M-protein in blood or urine
clonal plasma cells
end organ damage
65
CD 38 / 56
| intracytoplasmic monoclonal light chain
what is the immunophenotpye for plasma cell neoplasms
66
what is the CBC for multiple myeloma
anemia sometimes pancytopenia
67
metabolic panal for multiple myeloma
hypercalcemia
| increased creatine
68
what does monoclonal protein mean in multiple meyloma
one class of Ig will be elevated rest will be decreased
69
what does the radiology x-ray look like for multiple myeloma
bony lytic lesions
70
tumor cells in multiple meyoloma produce what
IL-6 ( growth factor for tumor cells)
71
in mulitple myeloma tumor cells have what influence
activation of osteoclasts
| inhibition of osteoblasts
72
for multiple myeloma what is required for diagnosis
bone marrow
73
what is seen in the peripheral blood for multiple myeloma
roulex due to increased protein
| -excess immunoglobulins
74
MGUS what is seen in blood and urine
BJ protein and M protein levels
75
plasmacytoma
solitary bone or soft tissue clonal plasma cell masses with no evidence of marrow or organ disease
76
1% of MGUS develop what after one year
multiple myeloma
77
where does plasmacytoma most likely occur in bone and soft tissue
spine
| lung and oronasopharynx
78
what is plasmacytoma treated
local field radiation therapy
79
Diffuse large B cell lymphoma define
high grade
| heterogenous group
80
what virus is associated with diffuse large B cell lymphoma
EBV
81
what are clinical features of the disease diffuse large B-cell Lymphoma
rapidly enlarging
B-type symptoms
BM involvement later
82
what is the most common form of NHL
diffuse large B-cell Lymphoma
83
what is the histology for DLBCL
1-3 nuclei, pale cytoplasm
| indistinct cell borders
84
CD 19, 20
| surface light chain restriction
immunophenotype for DLBCL
85
what are the cytogenetic mutations for DLBCL
BCL6
BCL2
MYC
double hit lymphomas
86
what is the role of BCL6
represses normal B-cell differentiation
87
define Burkitt Lymphoma
high grade
88
what are the 3 different types of Burkitt Lymphomas and where do some of them occur
endemic EBV ( african Malaria belt)
sporadic ( US children)
HIV
89
what is the clincal menifestation for the endemic burkitt lymphoma
mandibular mass
| abdominal viscera
90
what is the clinical manifestation for sporadic burkitt lymphoma
abdominal mass in ileocecum and peritoneum
91
what are two broad clinical features of Burkitt Lymphoma
Tumor Lysis syndrome
| CNS disease
92
what is the buzzword for Burkitt lymphoma
Starry sky
93
what are two characteristics of Burkitt lymphoma cells
high mitotic activity
| apoptotic cells
94
why does Burkitt lymphoma cell grow so fast
TRANSLOCATION OF MYC
95
CD 19, 20, 10
surface light chain restriction
bcl6
what is the immunophenotype for Burkitt Lymphoma
96
what are the translocations for Burkitt lymphoma
t (8;14)
t ( 2;8)
t ( 8;22)
97
treatment for Burkitt lymphoma
aggresive chemotherapy with intrathecal CNS chemotherapy
98
PCR is used to identify what in mature T cell neoplasms
id monoclonal rearrangements of at least one TCR locus
99
define peripheral t cell lymphoma, not otherwise specified ( PTCL-NOS)
T cell counterpart of DLBCL
100
clinical features for PTCL-NOS
generalized lymphadoenopathy
eosinophilia
pruritis, fever, weight loss
101
prognosis for PTCL-NOS
worse than B
102
immunophenotype for PTCL-NOS
mature T cell phenotype
103
define anaplastic large cell lymphoma
translocation involving ALK ( behave like tyrosine kinase) gene on 2p23
104
clinical presentation for anaplastic large cell lymphoma
advanced stage with adenopathy
extranodal infiltrates
BM involvement
105
what is the hallmark cells for anaplastic large cell lympohoma
horsehoe or wreath shaped nuclei
106
what is the sinusoidal pattern of anaplastic large celll lympohma
neoplastic cells around venules in sinuses of node
107
what is the prognosis for kids ALCL lymphoma +
good with chemo
108
prognosis for adutls ALCL lympohma + or -
negative less favorable
109
define Adult T-cell leukemia/lymphoma
CD4 t cells infected with retrovirus HTLV-1
110
what are two different presentations for ATCL and prognosis
leukmic = rapidly progressive
| skin localized - less aggresive
111
histo cells of ATCL
flower/cloverleaf cells
112
immunophenotype for ATCL
CD4+/CD7-
| HTVL-1+
113
Mycosis Fungoides/Sezary syndrome (MF/SS)
different manifestations of tumor of CD4+ helper T cells that home to the skin
114
clinical features of MF
patch
plaque
tumor
115
clinical features of SS
generalized exfoliative erythroderma plus leukemia
116
histo what does neoplastic and sezary cell look like
neoplast: CEREBRIFORM nuclear contours
sezary: neoplastic in BLOOD
117
Large granular lymphocytic leukemia define
T cell type and NK
118
Large granular lymphocytic leukemia associated disease
Felty syndrome
119
Large granular lymphocytic leukemia what happens
neutropenia with associated decreased myeloid maturation in BM
120
Large granular lymphocytic leukemia pathology
STAT 3 mutations
| large granular lymphocytes
