UNIT 5: MLN Flashcards
(122 cards)
1
Q
What is common is the involvement of particular cell line:
A
lymphoid cells
2
Q
lymphoproliferative diseases wherein the primary site is the blood or
bone marrow
A
LEUKEMIA
3
Q
group of disorders wherein the localization of the disease is in the
lymph nodes or the spleen
A
LYMPHOMA
4
Q
stimulates entry to cell cycle
A
MYC
5
Q
suppresses apoptosis
A
BCL2
6
Q
suppresses transcription of other genes
needed for cell growth
A
BCL6
7
Q
Malignant transformation of lymphoid cells at various stages of
development
A
mature lymphoid neoplasms
8
Q
both recognized as B
cell disorders
A
hodgkin lymphoma and hairy cell leukemia
9
Q
unmutated heavy chains,
representation of pre-terminal center event and hypermutation
heavy chains, suggestive of latter stage of disease development
A
CLL
10
Q
adult T-cell lymphoma / leukemia, prolymphocytic leukemia,
mantle cell lymphoma, Burkitt’s lymphoma, multiple myeloma
A
aggressive
11
Q
chronic lymphocytic leukemia, hairy cell leukemia, mycosis
fungoides, MZL
A
indolent
12
Q
Asymptomatic to fulminant illness – CBC
■ Fever, weight loss, night sweats, adenopathies
A
MLN
13
Q
MLN are Nonspecific but reflective of tumor
A
TRUE
14
Q
Painless cervical adenopathy – in young adults, most often associated
with
A
hodgkin’s lymphoma
15
Q
unexplained weight loss more than 10% of body weight
6 months prior to the stage of the disease,
unexplained or persistent recurrent
fever greater than 38C
or recurrent drenching night sweats during previous
month.
A
systemic b symptoms
16
Q
first lab test ordered in investigation of MLNs
A
hematological studies
17
Q
Most significant: multiple myeloma / plasma cell myeloma
A
TRUE
18
Q
ATM and TP53 genes affected
A
CLL
19
Q
incorporate data from lab test information, anatomic pathology
analysis and radiographic studies, along with physical exams
A
staging system
20
Q
For detection of hodgkin’s lymphoma in order to quantify nodal and extranodal sites of
involvement
A
ANN ARBOR SS
21
Q
Descriptive; would depend on the number of nodules present and the area where these
nodules are found
A
ANN ARBOR SS
22
Q
Presence of 1 node involved or single extranodal site
A
STAGE 1
23
Q
Two or more nodes or groups of lymphatic structures on the
same side of the diaphragm
Or the involvement of limited contiguous extra lymphatic organs
or tissues
A
STAGE 2
24
Q
Nodes on both sides of diaphragm with involvement of the
spleen
Or limited contiguous extra lymphatic organs or tissue involvement
Or BOTH
A
STAGE 3 - ADVANCED
25
Diffused or disseminated foci of involvement of one or more
extra lymphatic organs or tissues with or without associated
lymphatic involvement.
STAGE 4
26
Simplification of Ann Arbor
Used for Non-Hodgkin Lymphoma
Used to group patients into early/limited stages and advanced stages
LUGANO CLASSIFICATION
27
B cells
Median age: 72 years
Male > Female
Asymptomatic in most cases
CLL
28
B cells ≥ 5,000/cumm for >3 months
Flow cytometry: clonality
CLL
29
Small, mature lymphocytes - 85%
Scanty cytoplasm
Dense nucleus with condensed chromatin -
“cobblestone” or
“soccer ball”
CLL
30
Prolymphocytes (<10%) or atypical lymphocytes (>15%)
Smudge cells
CLL
31
20%, more aggressive
ATYPICAL CLL
32
commonly
found in the peripheral blood of
patients with CLL
smudge cells
33
B cell markers: CD19, CD20, CD23
T cell marker: CD5
CLL
34
>15% - large atypical lymphoid cells with nuclear indentation
mixed type/atypical CLL
35
based on clinical criteria that have
prognostic implications for CLL.
RAI AND BINET
36
important prognostic determinant for CLL and is used to
guide clinical management.
STAGING
37
PROGNOSIS OF CLL
del(17p) and TP53
38
marker for subtypes
IGHv
39
IGHv - more aggressive
UNMUTATED
40
is a strong predictor of survival and, in some cases,
associated with the phenotypes of certain CLL variants
KARYOTYPING
41
Circulating lymphocyte - < 5 x 109/L
Progression to CLL – 1-2% per year
MBL
42
Circulating lymphocyte - < 5 x 109/L
Adenopathy or organomegaly
SLL
43
T and B forms exist – separate disorders
PLL
44
1-2% of lymphoid leukemia (rare but aggressive)
Median age: 70 years old
Male = Female
T-PLL
45
Medium to Large in size
Ample cytoplasm
Open chromatin pattern
Prominent nucleolus
B-CELL SUBTYPE OF PLL
46
accounts for 75% of the
cases of PLL
B CELL SUBTYPE
47
Massive splenomegaly
Lymphocytosis – 100 × 10⁹/L
Minimal adenopathy
PLL
48
what PLL SUBTYPE Tissue involvement (skin)
T CELL SUBTYPE
49
Primarily, manifestations are found in
the peripheral blood, bone marrow, and
spleen.
A rapid increase in the leukocyte count
is a clue that the diagnosis is not CLL.
PLL = 100x 109/L
50
Indolent disease of B cell lineage
Median age – 50 years
Male > Female
HAIRY CELL LEUKEMIA
51
Round oval nuclei
●Lack nucleoli
Abundant cytoplasm with ragged projections
Variant
○ Less hairs - kidney-bean-shaped nucleus
hairy cell leukemia
52
Spleen, blood, and bone marrow
Splenomegaly, cytopenias
Bone marrow (BM) fibrosis
HAIRY CELL LEUKEMIAS
53
ANTI CD20
HAIRY CELL LEUKEMIA
54
Median age – 60 years
15% of circulating lymphocytes
disease of older adults (60 y/o) and most
patients are asymptomatic.
LGL
55
Abundant pale blue cytoplasm
Medium (M) to Large (L) azurophilic granules
Variant/atypical lymphocyte
LGL
56
increase in the number of large granular
lymphocytes may trigger a flag or blast flag in
hematology analyzers. They would be
reported as Atypical Lymphocytes
LGL
57
Most patients present with neutropenia, anemia, or both.
Autoimmune disease
LGL
58
CD3
CD8
CD57
T CELL LGL
59
CD 3(-)
CD57(+)
More aggressive
NK CELL LGL
60
Extramedullary areas:
○Spleen
○Lymph nodes
Leukemic phase
LYMPHOMAS
61
are less common but more aggressive
T CELL LYMPHOMA
62
Marked leukocytosis in leukemic phase
ATLL
63
M to L
Accentuated, convoluted nuclei with course clumped chromatin
Basophilic cytoplasm
“Flower cell”
ATLL
64
Osteolytic lesions
Hypercalcemia
Immunosuppression resulting to infection
ATLL
65
CD3
CD4
CD25
CDR4
IL2
ATLL
66
Consistent with T helper cells
Absent are CD7 and CD8
ATLL
67
Mature B Cells
Aggressive cancer
BURKITT LYMPHOMA
68
M to L
FInely clumped chromatin
Basophilic cytoplasm
Vacuoles present
“Starry sky”
BL
69
Results from the interspersed tingible body
macrophages in a sea of malignant cells in the BM
STARRY SKY OF BL
70
Geographical
distribution is like
Malaria.
There is extranodal
involvement.
It affects the orbits and
the mandible.
EBV genome has been
seen present in
neoplastic cells.
ENDEMIC BL
71
Extranodal involvement
is not common, only the
BM and CNS
sporadic
72
Expression of CD10 and
absence of immature markers
CD34 and TdT.
BL
73
important in
the characterization of Burkitt
Lymphoma.
Flow cytometry
74
Negative for CD5 unlike CLL and SLL
CD20 (+), CD19 (+), CD10 (+)
BL
75
It is highly responsive to
chemotherapy
BL
76
Germinal B cells
FL
77
Incurable with current therapies
Recurring relapse after chemotherapy
Painless adenopathy
FL
78
Accounts for 12% of non-hodgkin
lymphoma.
It is a disease of middle to older ages.
FL
79
PB involvement:
10% of cases condensed chromatin pattern
Distinct nuclear cleft
FL
80
BM:
Localized tumor cells
○Small FL cells angular appearance
■ Centrocytes
○Large round to ovoid nuclei with 1-3 nucleoli
■ Centroblast
FL
81
3-6% of NHL, aggressive
Median age – 68 years
Male > female
MCL
82
There is extranodal
involvement which is basically
involving the GI tract as its
primary area.
MCL
83
Extensive lymphadenopathy
Extranodal disease is not uncommon, with the GI tract acting as a primary area of involvement.
MCL - GI TRACT
84
In most cases, the disease is
restricted to the blood, BM,
and spleen.
MCL
85
De novo or transform from CLL/SLL, FL, MZL
Aggressive but curable
DLBCL
86
Disease of the elderly but can be
seen in younger individuals
Painless adenopathy
○One or more sites
DLBCL
87
BCL6 translocation
○30%
BCL2 (t14;18) - Follicular lymphoma
○ 20-30%
MYC abnormality with BCL6 and BCL2 translocation
○ 10%
○ “double” or triple” hit
DLBCL
88
B CELL LYMPHOMA
MZL
89
Indolent B cell lymphoma like hairy cell
MZL
90
Chronic antigen stimulation following infection or autoimmunity
○ One of the most commonly affected organ is the stomach
MZL
91
Most common
Helicobacter pylori
MALT MZL
92
Lymph nodes, BM
Waste paper basket
Hepatitis C
Cryoglobulinemia
No standard therapy
NODAL MZL
93
Cutaneous T cell lymphoma
○60-70%
CD4+ T cells
MF OR SS
94
Most familiar / common form
of T cell lymphoma
MF OR SS
95
malignant clone
has an effector
memory phenotype
MF
96
central memory T
cells
SS
97
large of small cell size (diverse) and can develop
transformation from a small to large cell variety.
SS
98
Confined in the skin but at the latter stages, other organs
such as lymph nodes and blood are involved.
SS
99
systemic disorder involving the
peripheral blood, has worse prognosis.
is characterized by erythroderma and
generalized lymph adenopathy and the
presence of clonal T cells in blood, skin,
and lymph nodes.
cell count of > 1 x 109/L is required
for the diagnosis.
Ss
100
a slow progressive
disease.
shows psoriatic-like
lesions that is seen through
frequent repeated biopsy
for a more definite
diagnosis.
MF
101
T cell disorder
CD30
ALCL
102
Heterogenous morphology
because some cells appear small
to medium
ALCL
103
CD30 expression in the cells
ALCL
104
Median age of 57 y/0
A subtype has been
associated with patients
who received breast
transplant/implants.
ALK-ALCL
105
Younger group
Better prognosis
10-30% of childhood lymphomas
ALK + ALCL
106
Nodal with extranodal involvement (skin)
BM - 20-30%
Anemia, thrombocytopenia, eosinophilia
T cell Lymphoma
PERIPHERAL T CELL LYMPHOMA NOT OTHERWISE SPECIFIED
107
Second most common hematologic malignancy
plasma cell neoplasm
108
only comprise 1-2% of all cancer cells
Involves terminally differentiated B cells
Multiple or plasma cell myeloma is the prototype
of the disorder
PCN
109
Benign form
MGUS
110
a benign form. It represents a precursor state of the myeloma
Conversion states are low but there is more than 60% chance of progression
over a 20-year period
must be differentiated from true PCM
MGUS
111
Circulating plasma cell:
○20% and >2 x 10⁹/L
Rouleaux formation
multiple (plasma cell) myeloma
112
Two Categories of MULTIPLE (PLASMA CELL) MYELOMA
Primary plasma cell
leukemia and Secondary plasma cell
leukemia
113
BM-based disease
Extramedullary extension to bones or soft tissues
Hypercalcemia, renal failure, anemia, osteolytic lesions
MULTIPLE (PLASMA CELL) MYELOMA
114
CD38
CD138
CD45
NK marker CD56
MULTIPLE (PLASMA CELL) MYELOMA
115
Low grade lymphoplasmacytic lymphoma
WALDENSTROM MACROGLOBULINEMIA
116
Secretion of IgM
Hyperviscosity Syndrome - plasmapheresis
Extramedullary involvement
○Nodal disease
○Hepatosplenomegaly
WALDENSTROM MACROGLOBULINEMIA
117
MYD88
WALDENSTROM MACROGLOBULINEMIA
118
High levels of IgM leads to hyperviscosity
syndrome and is corrected by plasmapheresis.
Affects liver and spleen.
WALDENSTROM MACROGLOBULINEMIA
119
It is a molecular marker for Waldenstrom
macroglobulinemia
MYD88
120
Lymph-node based disease affecting the young
First tumor cured – combination therapy
Model for therapeutic evolution
Patients with relapse can be cured by current therapies
HODGKIN LYMPHOMA
121
95% of cases
Bimodal age distribution –
30s then after 50
Reed Sternberg cell
○ B cell origin
○ CD30, CD15
CLASSIC HL
122
Absence of RS cell
Lymphocytic histiocytic cell
Large with scant cytoplasm
Folded single nucleus
“Popcorn cell”
LYMPHOCYTE-PREDOMINANT
