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Heme & Lymph > Chapter 13 - Dz of WBCs, LNs, Spleen and Thymus > Flashcards

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
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2
Q

myeloid progenitor cells give rise to

A
  • myeloblasts > NPs
  • monblast > monocyte
  • eosinophilblast > eos
  • basophiloblast > baso
  • megakaryoblast > platelet
  • erythroblast > RBC
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3
Q

increased ratio fat cells:hematopoetic elements

A

hypoplastic state (ex aplastic anemia)

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4
Q

decreased ratio fat cells:hematopoetic elements

A

direct involvement of marrow (ex hematopoetic tumors) and compensation hyperplasias (ex hemolytic anemia)

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5
Q

expanision of leuks

A

leukocytosis

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6
Q

def of leuks

A

leukopenia

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7
Q

lymphopenia MC ass’d with

A
  • HIV

- DiGeorge syndrome

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8
Q

agranulocytosis increases susceptibility to

A

bacterial and fungal infections

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9
Q

significant reduction in NPs

A

agranulocytosis

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10
Q

MCC of agranulocytosis

A

drug toxicity

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11
Q

drugs that casue agranulocytosis

A
  • aminopyrine
  • cholaramphenicol
  • sulfonamides
  • chlorpromazine
  • thiouracil
  • phenylbutazone
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12
Q

necrotizing lesions of the gingiva, floor of mouth, buccal mucoas, pharynx or elsewhere in the oral cavity

A

common consequence if agranulocytosis

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13
Q

netropehic pts are at high risk of inf caused by

A

candida and aspergillus

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14
Q

leukocytosis from increased production in marrow

A
  • chronic inf
  • paraneoplastic
  • myeloproliferative disorder
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15
Q

leukocytosis from increased release from marrow stores

A
  • endotoxemia
  • inf
  • hypoxia
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16
Q

leukocytosis from decreased margination

A
  • exercise

- catecholamines

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17
Q

leukocytosis from decreased extravasation into tissues

A

glucocorticoids

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18
Q

neurophils with coarse purple cytoplasmic granules

A

toxic granulation

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19
Q

sky-blue cytoplasmic patches of distended ER (“puddles”)

A

Dohle bodies

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20
Q

NPs with toxic granulations and Dohle bodies suggest

A

bacterial sepsis

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21
Q

causes of follicular hyperplasia

A
  • RA
  • toxo
  • early stages of HIV
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22
Q

causes of paracortical hyperplasia

A

viral infections, T cell mediated (ex mono)

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23
Q

sinus histocytosis (reticular hyperplasia) may be prominent in

A

LNs draining cancers

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24
Q

common feature of all forms of hemophagocytic lymphohistiocytosis

A

systemic activation of MPs and CD8+ cytotoxic T cells

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25
Q

MC trigger for HLH

A

EBV infection

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26
Q
  • HSM
  • anemia
  • thrombocytopenia
  • high levels of plasma ferratin and IL-2 receptor
  • elevated LFTs
  • possible DIC
A

HLH

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27
Q

lymphotrophic viruses ass’d as causative agents of lymphomas

A
  • HTLV-1
  • EBV
  • HHV-8/KSHV
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28
Q

smokers have a 1.3-2 fold increased risk of

A

AML

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29
Q

ALLs are compose of

A

immature B or T cells (lymphoblasts)

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30
Q

B-ALLs MC present as

A

childhood “leukemias”

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31
Q

T-ALLs MC present at

A

adolescent male thymic “lymphomas”

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32
Q

MC cancer of children

A

ALL

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33
Q
  • myeloperoxidase negative

- periodic acid-Shiff-positive cytoplasmic material

A

lymphoblasts

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34
Q

TbT immunotyping

A

pre-B and pre-T lymphoblasts, + in more than 95% of B/T-ALLs

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35
Q
  • CD10
  • CD19
  • PAX5
A

B-ALL

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36
Q
  • CD1
  • CD2
  • CD5
  • CD7
A

T-ALL

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37
Q

pathognmonic fo CLL/SLL

A

proliferation centers in LNs

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38
Q
  • CD19
  • CD20
  • CD23
  • CD5
A

CLL/SLL

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39
Q

5-10% of CLL/SLL pts can progress to

A

Richter synd - diffuse large B-cell lymphoma

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40
Q

translocations involving BCL2

A

follicular lymphoma

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41
Q

t(14;18)

A

follicular lymphoma

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42
Q

small cells with irregular or cleaved nuclear contours and scant cytoplasm

A

centrocytes

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43
Q

larger cells with open nuclear chromtin, severla nucleoli, and modest amounts of cytoplasm

A

centroblasts

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44
Q

centrocytes and centroblasts seen in

A

follicular lymphoma

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45
Q

MC form of NHL

A

diffuse large B-cell lymphoma (DLBCL)

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46
Q

breakpoint in BCL6 at chromasome 3q27

A

DLBCL

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47
Q
  • DLBCL subtype
  • KSS/HHV-8 infection
  • clonal IgH gene rearrangements
A

primary effusion lymphoma

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48
Q

MYC gene translocation

A

Burkitt lymphoma

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49
Q

“starry sky”

A

Burkitt lmyphoma

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50
Q
  • CD19+
  • CD20+
  • CD10+
  • BCL6+
  • BCL2-
A

Burkitt lymphoma

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51
Q

mass involving the iliocecum

A

sporadic burkitt

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52
Q

mass involving the mandible

A

endemic burkitt

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53
Q

dyscrasias

A

plasma cell neoplasms

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54
Q

Bence Jones proteins

A

free light chains in the urine - plasma cell tumors

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55
Q

tumorous masses scattered throughout the skeletal system

A

multiple myeloma

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56
Q

single mass in bone or soft tissue (plasma cell neoplasm)

A

solitary myelomas

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57
Q

lack of symptoms, high plasma M component

A

smoldering myeloma

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58
Q
  • older adult
  • high levels of IgM
  • hyperviscosity of blood
  • MC ass’d with lymphoplasmacytic lymphoma
A

Waldenstrom macoglobulinemia

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59
Q

ass’d with lymphoplasmacytic lymphoma and small bowel marginal zone lymphoma

A

heavy chain disease

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60
Q
  • older pt
  • lack of s/s
  • moderately large M component in blood
A

monclonal gammopathy of undetermined significance (MGUS)

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61
Q
  • lytic bone lesions
  • hypercalcemia > bone fractures
  • renal failure
  • aquired immun abnormalities
A

multiple myeloma

62
Q
  • flame cells
  • Mott cells
  • cells with other inclusions
A

multiple myeloma

63
Q

globular inclusions in cytoplasm

A

Russell bodies

64
Q

globular inclusion in nucleus

A

Dutcher bodies

65
Q

high IgM leads to linear arrays of RBCs stuck together

A

rouleaux formation

66
Q

rouleaux formations seen in

A
  • multiple myeloma
  • lupus
  • early HIV inf
67
Q
  • CD138 +
  • CD56+
  • syndecan-1 +
68
Q

acquired mutations in MYD88

A

lymphoplasmocytic lymphoma

69
Q

periodic acid-shiff-positive inclusions

A

lymphoplasmocytic lymphoma

70
Q

t(11;14) involving IgH locus on Ch14 and cyclin D1 locus on Ch11 > overexpression of cyclin D1

A

mantle cell lymphoma

71
Q

polyp like lesions

A

mantle cell lymphoma

72
Q
  • high levels of cyclin D1
  • CD19+
  • CD20+
  • high levels of surface Ig - usually M or D
  • CD5+
  • CD23 -
A

mantle cell lymphoma

73
Q
  • CD19+
  • CD20+
  • surface Ig - usually IgG
  • CD11c+
  • CD25+
  • CD103+
  • annexin A1
A

hairy cell leukemias

74
Q

“massive” splenomegaly

A

hairy cell leukemia

75
Q

atypical mycobacterial infection

A

hairy cell leukemia (TB stains acid fast!)

76
Q

rearrangements in the ALK gene on Ch2p23

A

anaplastic large-cell lymphoma

77
Q

“horseshoe-like” or “embryoid” nuclei

A

anaplastic large-cell lymphoma

78
Q

neoplastic T cells with cerebriform appearance

A

mycosis fungoides

79
Q
  • CLA+
  • CCR4+
  • CCR10+
A

mycosis fungoides/sezary syndrome

80
Q

T cell variant of large granular lymphocytic leukemia

81
Q

NK cell variant of large granular lymphocytic leukemia

A

CD56+, CD3-

82
Q

RA, splenomegaly, neutopenia

A

Felty syndrome

83
Q

felty sydrome is ass’d with

A

large granular lymphocytic leukemia

84
Q
  • destructive nasoparyngeal mass
  • sometimes involves testis and skin
  • highly ass’d w/ EBV
  • seen in Asia
  • CD3 negative
A

extranodal NT/T-cell lymphoma

85
Q

mutations in BRAD serine/threonine kinase

A

hairy cell leukemia

86
Q
  • aggressive tumor of CD4+ T cells

- ass’d with HTLV-1 infection

A

adult T cell leukemia/lymphoma

87
Q

STAT3 mutations

A

large granular lymphocytic leukemia

88
Q

neoplastic giant cells found in hodgkin lymphoma

A

Reed Sternburg cells

89
Q

distinctive B-cell immnuophenotype of Reed-Sternburg cells

A

lyphocyte predominance Hodgkin lymphoma

90
Q

MC form of Hodgkin lymphoma

A

Nodular sclerosis type

91
Q
  • lacunar type reed-sternberg cells

- collagen deposition in bands that divide involved LNs into circumscribed nodules

A

nodular sclerosis type HL

92
Q
  • PAX5+
  • CD15+
  • CD30+
A

“classic” immunophenotype of RS cells in HL

93
Q

classic HL where 90% are EBV +

A

lymphocyte depletion HL

94
Q

least common classic HL

A

lymphocyte depletion HL

95
Q
  • nodular nucleus (“popcorn cell”)
  • CD20+
  • BCL6+
A

L&H variant RS cell

96
Q

L&H variant RS cells found in

A

lymphocyte predominant HL

97
Q

subtypes of HL that are ML to have constitutional symptoms

A

disseminated disease (stage III or IV), mixed cellularity type, lymphocyte depletion type (these are both the “classic” EBV+ HLs!)

98
Q

spread of HL

A

nodal dz > splenic dz > hepatic dz

99
Q

dx of AML is based on

A

presence of at least 20% blasts in the BM

100
Q
  • myeloperoxidase pos
  • aeur rods
  • 2-4 nucleoi
A

myeloblasts

101
Q
  • folded lobulated nuclei

- nonspecific esterase positive

A

monoblasts

102
Q

marker or mature myeloid cells

103
Q
  • CD34+
  • CD33+
  • CD15+ (a little more mature)
A

myeloblast markers

104
Q

erythroblasts with iron-laden mitochondria visible in perinucleear granules on prussian blue stain

A

ring sideroblasts

105
Q

neutrophils with only two nuclear lobes

A

pseudo-pelger-huit cells

106
Q

megakaryocytes with single nuclear lobes or multiple seperate nuclei

A

pawn ball megakaryocytes

107
Q
  • ring sideroblasts
  • pseudo-pelger-huit cells
  • pawn ball megakaryocytes
A

myelodysplastic syndrome (MDS)

108
Q

BCR-ABL fusion gene

A

chronic myelogenous leukemia

109
Q

scattered MPs with abundant wrinkled, green-blue cytoplasm (sea-blue histiocytes)

A

chronic myelogenous leukemia

110
Q

activating point mutations in JAK2

A

polycythemia vera

111
Q

progression of PCV to spent phase is characterized by

A

marrow fibrosis

112
Q

marked thrombocytosis and giant platelets (almost as big as RBCs) on peripheral blood smear

A

essential thrombocytosis

113
Q

erythromelalgia is seen in

A

essential thrombocytosis and PCV

114
Q
  • obliterative marrow fibrosis

- extensive deposition of collagen in marrow by non-neoplastic fibroblasts

A

primary myelfibrosis

115
Q

teardrop shaped RBCs

A

primary myelfibrosis

116
Q
  • abundant, vacuolated cytoplasm
  • vesicular nuclei with linear grooves or folds
  • bierbeck granules
A

langerhans cell

117
Q

pentilaminar tubules with dilated terminal forming a tennis racket like appearance

A

bierbeck granules

118
Q
  • seborrheic eruptions of the trunk and scalp
  • HSM
  • LAD
  • pulmonary lesions
  • destructive osteolytic bone lesions
A

multifocal multisystem langerhans cell histiocytosis

119
Q
  • calvarial bone defects
  • diabetes incipidus
  • exopthamos
A

hand-schuller-christian triad

120
Q

hand-schuller-christian triad

A

multifocal unisystem langerhans cell histiocytosis (hisiocytosis X)

121
Q
  • MC effects skeletal system in older children or adults
  • pathologic fractures
  • bierbeck granules
A

unifocal unisystem langerhans cell histiocytosis

122
Q
  • adult smoker

- BRAF mutation

A

pulmonary langerhans cell histiocytosis

123
Q

neoplastic langerhans cells are different from normal epi langerhans cells d/t

A

CCR7+ - both are CCR6+

124
Q
  • dragging senation in LUQ

- discomfort after eating

A

splenomegaly

125
Q

hypersplenism

A

-anemia
-leukopenia
-thrombocytopenai
(alone or in combo)

126
Q

hemolytic streptococcus can cause this in the spleen

A

white pulp follicle necrosis

127
Q

main cause of massive congestive splenomegaly (1-5 kg)

A

cirrhosis of the liver

128
Q

MC primary tumors of the spleen

A

lymphangiomas and hemangiomas

129
Q

MC tumors that cause splenomegaly

A

myeloid and lymphoid tumors

130
Q

other congenital defects ass’d with asplenia

A

situs inversus and cardiac malformations

131
Q

consideration of an accessory spleen is esp impt in

A

hereditary spherocytosis and immune thrombocytopenia purpura

132
Q

predisposing conditions for splenic rupture

A
  • mono
  • malaria
  • typhoid fever
  • lymphoid neoplasms
133
Q
  • hypoplasia or aplasia of the thymus

- part of 22q11 deletion syndrome

A

DiGeorge syndrome

134
Q

MC disorders ass’d with thymic hyperplasia

A
  • myasthenia gravis (up to 75%)
  • graves
  • SLE
  • scleroderma
  • RA
135
Q

tumor of thymic epi cells

136
Q
  • swirling arrangement of thymic epi cells
  • oval/elongated nuclei
  • inconspicuous nucleoi
A

benign thymoma, medullary type (MC)

137
Q
  • tumor penetrates though the capsule into the surrounding structures
  • MC of the cortical variety
  • polygonal epi cells with round, bland nuclei
A

invasive thymoma - malignant, type I

138
Q
  • sheets of cells with indistinct borders
  • bear close resemblance to NP carcinomas
  • most have monoclonal EBV genomes
A

lyphoepithelioma-like carcinoma of the thymus

139
Q

MC type of thymic carcinoma

A

sq cell carcinoma

140
Q
  • S-100 +

- CD1a +

A

langerhans cell

141
Q

decreased splenic function causes increased risk of infection with

A

encapsulated bacteria - pneumococcus, H flu, meningococcus

142
Q

medulla of thymus is derived from

A

third pharyngeal pouch

143
Q

cortex of thymus is derived from

A

third pharyngeal cleft

144
Q

cortical thymomas rich in thymocytes are more likely to be ass’d with

A

autoimmune disorders

145
Q

lymphoepithelioma type of thymic carcinoma can be ass’d with

146
Q

interconnecting pattern of keratin-immunoreactivity

147
Q

smudge cells

148
Q

M2 AML

A
  • AML w/ myelocytic maturation

- t(8;21)

149
Q

M4 AML

A
  • AML with myelomonocytic matureation (AMML)
  • inv(16)
  • nonspecific exterase positive
150
Q

can turn into DLBCL

A
  • CLL
  • follicular lymphoma
  • marginal cell lymphoma
  • lymphoplasmocytic lymphoma

Heme & Lymph (11 decks)

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