Chapter 13 - Dz of WBCs, LNs, Spleen and Thymus Flashcards
1
Q
lymphoid progenitor cells give rise to
A
- NK cells
- B cells
- T cells
2
Q
myeloid progenitor cells give rise to
A
- myeloblasts > NPs
- monblast > monocyte
- eosinophilblast > eos
- basophiloblast > baso
- megakaryoblast > platelet
- erythroblast > RBC
3
Q
increased ratio fat cells:hematopoetic elements
A
hypoplastic state (ex aplastic anemia)
4
Q
decreased ratio fat cells:hematopoetic elements
A
direct involvement of marrow (ex hematopoetic tumors) and compensation hyperplasias (ex hemolytic anemia)
5
Q
expanision of leuks
A
leukocytosis
6
Q
def of leuks
A
leukopenia
7
Q
lymphopenia MC ass’d with
A
- HIV
- DiGeorge syndrome
8
Q
agranulocytosis increases susceptibility to
A
bacterial and fungal infections
9
Q
significant reduction in NPs
A
agranulocytosis
10
Q
MCC of agranulocytosis
A
drug toxicity
11
Q
drugs that casue agranulocytosis
A
- aminopyrine
- cholaramphenicol
- sulfonamides
- chlorpromazine
- thiouracil
- phenylbutazone
12
Q
necrotizing lesions of the gingiva, floor of mouth, buccal mucoas, pharynx or elsewhere in the oral cavity
A
common consequence if agranulocytosis
13
Q
netropehic pts are at high risk of inf caused by
A
candida and aspergillus
14
Q
leukocytosis from increased production in marrow
A
- chronic inf
- paraneoplastic
- myeloproliferative disorder
15
Q
leukocytosis from increased release from marrow stores
A
- endotoxemia
- inf
- hypoxia
16
Q
leukocytosis from decreased margination
A
- exercise
- catecholamines
17
Q
leukocytosis from decreased extravasation into tissues
A
glucocorticoids
18
Q
neurophils with coarse purple cytoplasmic granules
A
toxic granulation
19
Q
sky-blue cytoplasmic patches of distended ER (“puddles”)
A
Dohle bodies
20
Q
NPs with toxic granulations and Dohle bodies suggest
A
bacterial sepsis
21
Q
causes of follicular hyperplasia
A
- RA
- toxo
- early stages of HIV
22
Q
causes of paracortical hyperplasia
A
viral infections, T cell mediated (ex mono)
23
Q
sinus histocytosis (reticular hyperplasia) may be prominent in
A
LNs draining cancers
24
Q
common feature of all forms of hemophagocytic lymphohistiocytosis
A
systemic activation of MPs and CD8+ cytotoxic T cells
25
MC trigger for HLH
EBV infection
26
- HSM
- anemia
- thrombocytopenia
- high levels of plasma ferratin and IL-2 receptor
- elevated LFTs
- possible DIC
HLH
27
lymphotrophic viruses ass'd as causative agents of lymphomas
- HTLV-1
- EBV
- HHV-8/KSHV
28
smokers have a 1.3-2 fold increased risk of
AML
29
ALLs are compose of
immature B or T cells (lymphoblasts)
30
B-ALLs MC present as
childhood "leukemias"
31
T-ALLs MC present at
adolescent male thymic "lymphomas"
32
MC cancer of children
ALL
33
- myeloperoxidase negative
| - periodic acid-Shiff-positive cytoplasmic material
lymphoblasts
34
TbT immunotyping
pre-B and pre-T lymphoblasts, + in more than 95% of B/T-ALLs
35
- CD10
- CD19
- PAX5
B-ALL
36
- CD1
- CD2
- CD5
- CD7
T-ALL
37
pathognmonic fo CLL/SLL
proliferation centers in LNs
38
- CD19
- CD20
- CD23
- CD5
CLL/SLL
39
5-10% of CLL/SLL pts can progress to
Richter synd - diffuse large B-cell lymphoma
40
translocations involving BCL2
follicular lymphoma
41
t(14;18)
follicular lymphoma
42
small cells with irregular or cleaved nuclear contours and scant cytoplasm
centrocytes
43
larger cells with open nuclear chromtin, severla nucleoli, and modest amounts of cytoplasm
centroblasts
44
centrocytes and centroblasts seen in
follicular lymphoma
45
MC form of NHL
diffuse large B-cell lymphoma (DLBCL)
46
breakpoint in BCL6 at chromasome 3q27
DLBCL
47
- DLBCL subtype
- KSS/HHV-8 infection
- clonal IgH gene rearrangements
primary effusion lymphoma
48
MYC gene translocation
Burkitt lymphoma
49
"starry sky"
Burkitt lmyphoma
50
- CD19+
- CD20+
- CD10+
- BCL6+
- BCL2-
Burkitt lymphoma
51
mass involving the iliocecum
sporadic burkitt
52
mass involving the mandible
endemic burkitt
53
dyscrasias
plasma cell neoplasms
54
Bence Jones proteins
free light chains in the urine - plasma cell tumors
55
tumorous masses scattered throughout the skeletal system
multiple myeloma
56
single mass in bone or soft tissue (plasma cell neoplasm)
solitary myelomas
57
lack of symptoms, high plasma M component
smoldering myeloma
58
- older adult
- high levels of IgM
- hyperviscosity of blood
- MC ass'd with lymphoplasmacytic lymphoma
Waldenstrom macoglobulinemia
59
ass'd with lymphoplasmacytic lymphoma and small bowel marginal zone lymphoma
heavy chain disease
60
- older pt
- lack of s/s
- moderately large M component in blood
monclonal gammopathy of undetermined significance (MGUS)
61
- lytic bone lesions
- hypercalcemia > bone fractures
- renal failure
- aquired immun abnormalities
multiple myeloma
62
- flame cells
- Mott cells
- cells with other inclusions
multiple myeloma
63
globular inclusions in cytoplasm
Russell bodies
64
globular inclusion in nucleus
Dutcher bodies
65
high IgM leads to linear arrays of RBCs stuck together
rouleaux formation
66
rouleaux formations seen in
- multiple myeloma
- lupus
- early HIV inf
67
- CD138 +
- CD56+
- syndecan-1 +
MM
68
acquired mutations in MYD88
lymphoplasmocytic lymphoma
69
periodic acid-shiff-positive inclusions
lymphoplasmocytic lymphoma
70
t(11;14) involving IgH locus on Ch14 and cyclin D1 locus on Ch11 > overexpression of cyclin D1
mantle cell lymphoma
71
polyp like lesions
mantle cell lymphoma
72
- high levels of cyclin D1
- CD19+
- CD20+
- high levels of surface Ig - usually M or D
- CD5+
- CD23 -
mantle cell lymphoma
73
- CD19+
- CD20+
- surface Ig - usually IgG
- CD11c+
- CD25+
- CD103+
- annexin A1
hairy cell leukemias
74
"massive" splenomegaly
hairy cell leukemia
75
atypical mycobacterial infection
hairy cell leukemia (TB stains acid fast!)
76
rearrangements in the ALK gene on Ch2p23
anaplastic large-cell lymphoma
77
"horseshoe-like" or "embryoid" nuclei
anaplastic large-cell lymphoma
78
neoplastic T cells with cerebriform appearance
mycosis fungoides
79
- CLA+
- CCR4+
- CCR10+
mycosis fungoides/sezary syndrome
80
T cell variant of large granular lymphocytic leukemia
CD3+
81
NK cell variant of large granular lymphocytic leukemia
CD56+, CD3-
82
RA, splenomegaly, neutopenia
Felty syndrome
83
felty sydrome is ass'd with
large granular lymphocytic leukemia
84
- destructive nasoparyngeal mass
- sometimes involves testis and skin
- highly ass'd w/ EBV
- seen in Asia
- CD3 negative
extranodal NT/T-cell lymphoma
85
mutations in BRAD serine/threonine kinase
hairy cell leukemia
86
- aggressive tumor of CD4+ T cells
| - ass'd with HTLV-1 infection
adult T cell leukemia/lymphoma
87
STAT3 mutations
large granular lymphocytic leukemia
88
neoplastic giant cells found in hodgkin lymphoma
Reed Sternburg cells
89
distinctive B-cell immnuophenotype of Reed-Sternburg cells
lyphocyte predominance Hodgkin lymphoma
90
MC form of Hodgkin lymphoma
Nodular sclerosis type
91
- lacunar type reed-sternberg cells
| - collagen deposition in bands that divide involved LNs into circumscribed nodules
nodular sclerosis type HL
92
- PAX5+
- CD15+
- CD30+
"classic" immunophenotype of RS cells in HL
93
classic HL where 90% are EBV +
lymphocyte depletion HL
94
least common classic HL
lymphocyte depletion HL
95
- nodular nucleus ("popcorn cell")
- CD20+
- BCL6+
L&H variant RS cell
96
L&H variant RS cells found in
lymphocyte predominant HL
97
subtypes of HL that are ML to have constitutional symptoms
disseminated disease (stage III or IV), mixed cellularity type, lymphocyte depletion type (these are both the "classic" EBV+ HLs!)
98
spread of HL
nodal dz > splenic dz > hepatic dz
99
dx of AML is based on
presence of at least 20% blasts in the BM
100
- myeloperoxidase pos
- aeur rods
- 2-4 nucleoi
myeloblasts
101
- folded lobulated nuclei
| - nonspecific esterase positive
monoblasts
102
marker or mature myeloid cells
CD64
103
- CD34+
- CD33+
- CD15+ (a little more mature)
myeloblast markers
104
erythroblasts with iron-laden mitochondria visible in perinucleear granules on prussian blue stain
ring sideroblasts
105
neutrophils with only two nuclear lobes
pseudo-pelger-huit cells
106
megakaryocytes with single nuclear lobes or multiple seperate nuclei
pawn ball megakaryocytes
107
- ring sideroblasts
- pseudo-pelger-huit cells
- pawn ball megakaryocytes
myelodysplastic syndrome (MDS)
108
BCR-ABL fusion gene
chronic myelogenous leukemia
109
scattered MPs with abundant wrinkled, green-blue cytoplasm (sea-blue histiocytes)
chronic myelogenous leukemia
110
activating point mutations in JAK2
polycythemia vera
111
progression of PCV to spent phase is characterized by
marrow fibrosis
112
marked thrombocytosis and giant platelets (almost as big as RBCs) on peripheral blood smear
essential thrombocytosis
113
erythromelalgia is seen in
essential thrombocytosis and PCV
114
- obliterative marrow fibrosis
| - extensive deposition of collagen in marrow by non-neoplastic fibroblasts
primary myelfibrosis
115
teardrop shaped RBCs
primary myelfibrosis
116
- abundant, vacuolated cytoplasm
- vesicular nuclei with linear grooves or folds
- bierbeck granules
langerhans cell
117
pentilaminar tubules with dilated terminal forming a tennis racket like appearance
bierbeck granules
118
- seborrheic eruptions of the trunk and scalp
- HSM
- LAD
- pulmonary lesions
- destructive osteolytic bone lesions
multifocal multisystem langerhans cell histiocytosis
119
- calvarial bone defects
- diabetes incipidus
- exopthamos
hand-schuller-christian triad
120
hand-schuller-christian triad
multifocal unisystem langerhans cell histiocytosis (hisiocytosis X)
121
- MC effects skeletal system in older children or adults
- pathologic fractures
- bierbeck granules
unifocal unisystem langerhans cell histiocytosis
122
- adult smoker
| - BRAF mutation
pulmonary langerhans cell histiocytosis
123
neoplastic langerhans cells are different from normal epi langerhans cells d/t
CCR7+ - both are CCR6+
124
- dragging senation in LUQ
| - discomfort after eating
splenomegaly
125
hypersplenism
-anemia
-leukopenia
-thrombocytopenai
(alone or in combo)
126
hemolytic streptococcus can cause this in the spleen
white pulp follicle necrosis
127
main cause of massive congestive splenomegaly (1-5 kg)
cirrhosis of the liver
128
MC primary tumors of the spleen
lymphangiomas and hemangiomas
129
MC tumors that cause splenomegaly
myeloid and lymphoid tumors
130
other congenital defects ass'd with asplenia
situs inversus and cardiac malformations
131
consideration of an accessory spleen is esp impt in
hereditary spherocytosis and immune thrombocytopenia purpura
132
predisposing conditions for splenic rupture
- mono
- malaria
- typhoid fever
- lymphoid neoplasms
133
- hypoplasia or aplasia of the thymus
| - part of 22q11 deletion syndrome
DiGeorge syndrome
134
MC disorders ass'd with thymic hyperplasia
- myasthenia gravis (up to 75%)
- graves
- SLE
- scleroderma
- RA
135
tumor of thymic epi cells
thymoma
136
- swirling arrangement of thymic epi cells
- oval/elongated nuclei
- inconspicuous nucleoi
benign thymoma, medullary type (MC)
137
- tumor penetrates though the capsule into the surrounding structures
- MC of the cortical variety
- polygonal epi cells with round, bland nuclei
invasive thymoma - malignant, type I
138
- sheets of cells with indistinct borders
- bear close resemblance to NP carcinomas
- most have monoclonal EBV genomes
lyphoepithelioma-like carcinoma of the thymus
139
MC type of thymic carcinoma
sq cell carcinoma
140
- S-100 +
| - CD1a +
langerhans cell
141
decreased splenic function causes increased risk of infection with
encapsulated bacteria - pneumococcus, H flu, meningococcus
142
medulla of thymus is derived from
third pharyngeal pouch
143
cortex of thymus is derived from
third pharyngeal cleft
144
cortical thymomas rich in thymocytes are more likely to be ass'd with
autoimmune disorders
145
lymphoepithelioma type of thymic carcinoma can be ass'd with
EBV
146
interconnecting pattern of keratin-immunoreactivity
thymoma
147
smudge cells
CLL/SLL
148
M2 AML
- AML w/ myelocytic maturation
| - t(8;21)
149
M4 AML
- AML with myelomonocytic matureation (AMML)
- inv(16)
- nonspecific exterase positive
150
can turn into DLBCL
- CLL
- follicular lymphoma
- marginal cell lymphoma
- lymphoplasmocytic lymphoma
