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Lecom pathology year 2 > chapter 13- WBCs > Flashcards

chapter 13- WBCs Flashcards

1
Q

what is the CD34+ cell

A

hematopoietic stem cell

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2
Q

what is the term for low WBC count

A

leukopenia

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3
Q

what is the term for high WBC count

A

leukocytosis

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4
Q

what is neutropenia classically seen with

A

drug toxicity and severe infection

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5
Q

what can cause the eft shift with neutrophilic leukocytosis

A

bacterial infection and tissue necrosis because slightly immature neutrophils may be released (decreased Fc receptors)

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6
Q

how does high cortisol state cause neutrophilic leukocytosis

A

because it causes demargination

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7
Q

what is produced in hodgkin lymphoma that can lead to eosinophilia

A

increase production of IL-5

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8
Q

what is the lymphocytic leukocytosis associated with infectious mononucleosis comprised of

A

reactive CD8+ T cells

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9
Q

what is the presentation result of neoplastic proliferation of blasts

A

acute presentation with anemia, thrombocytopenia or neutropenia

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10
Q

what are lymphoblasts positive for in the nucleus

A

tDt

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11
Q

what are myeloblasts positive for

A

MPO

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12
Q

what is the condition classified as neoplastic accumulation of lymphoblasts

A

ALL

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13
Q

who does ALL most commonly arise in

A

kids

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14
Q

at what age is ALL associated with down syndrome

A

after age 5

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15
Q

what is the most common ALL and what does it express

A

B-ALL; CD10, CD19, CD20

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16
Q

what is the most common translocation in kids with B-ALL

A

t (12:21)

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17
Q

what is the most common translocation in B-ALL

A

t (9:22)

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18
Q

how does T-ALL present

A

mediastinal mass in teenagers

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19
Q

what is the term for neoplastic accumulation of myeloblasts

A

AML

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20
Q

who does AML typically present in

A

older adults (50-60yo)

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21
Q

what translocation is acute promyelocytic leukemia characterized by

A

t(15:17)

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22
Q

what receptor is disrupted in acute promyelocytic leukemia

A

RAR receptor

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23
Q

what do the promyelocytes in acute promyelocytic leukemia contain numerous of and what does this pose a risk for

A

numerous Auer rods; risk for DIC

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24
Q

what do the blasts in acute monocytic leukemia characteristically infiltrate

A

gums

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25
Q

what disorder is acute megakaryoblastic leukemia associated with

A

down syndrome before the age of 5

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26
Q

what is the term for neoplastic proliferative of naive B cells that coexpres CD5 and CD20

A

CLL

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27
Q

what should you think of when you see smudge cells

A

CLL

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28
Q

what are some complications of CLL

A

hypogammaglobulinemia, autoimmune hemolytic anemia, transformation to diffuse large B cell lymphoma

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29
Q

what is the term for neoplastic proliferation of mature B cells

A

hairy cell leukemia

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30
Q

what are the cells in hairy cell leukemia positive for

A

TRAP

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31
Q

what does hairy cell leukemia have excellent response to

A

2-CDA

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32
Q

what typically causes the death in individuals with hair cell leukemia

A

adenosine accumulates to toxin levels in neoplastic B cells

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33
Q

what is the term for neoplastic proliferation of mature CD4+ T cells

A

ATLL

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34
Q

what is ATLL associated with

A

HTLV-1 (japan and caribbean)

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35
Q

what is the clinical presentation associated with ATLL

A

rash, generalized LAD with HSM, lytic bone lesions with hypercalcemia

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36
Q

what is the term for aggregates of neoplastic T cells in the epidermis and what disease is it associated with

A

paltrier micrabscesses; mycosis fungoides

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37
Q

what is it called when cells of mycosis fungicides spread to the blood

A

sezary syndrome

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38
Q

what are the characteristic lymphocytes in sezary syndrome

A

cerebriform nuclei

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39
Q

what is chronic myeloid leukemia driven by

A

t (9:22)

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40
Q

are CML granulocytes LAP positive or negative

A

negative

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41
Q

what is the term for neoplastic proliferation of mature myeloid cells, especially RBCs

A

polycythemia Vera

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42
Q

what is also increased besides RBCs in my polycythemia vera

A

granulocytes and platelets

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43
Q

if someone is itching after bathing, have blurry vision and headache and a flushed face, what disorder might you consider

A

polycythemia vera

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44
Q

what mutation in polycythemia associated with

A

JAK2 kinase mutation

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45
Q

what is the term for neoplastic proliferation of mature myeloid cells, especially platelets

A

essential thrombocythemia

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46
Q

what cells are increased in essential thrombocythemia

A

RBC and granulocytes

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47
Q

what mutation is associated with essential thrombocythemia

A

JAK2 kinase

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48
Q

what is the term for neoplastic proliferation of mature myeloid cells, especially megakaryocytic

A

myelofibrosis

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49
Q

what mutation is associated with myelofibrosis

A

JAK2 kinase

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50
Q

what is produced in excess in myelofibrosis

A

PDGF

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51
Q

what is the clinical presentation of follicular lymphoma

A

late adulthood with painless LAD

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52
Q

what is follicular lymphoma driven by

A

t (14:18)

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53
Q

what can follicular lymphoma progress to as a complication

A

diffuse large B cell lymphoma

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54
Q

how does large B cell lymphoma present

A

enlarging LN

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55
Q

what should you think of with follicular hyperplasia

A

tangible body macrophages in GC

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56
Q

how is mantle cell lymphoma presented clinically

A

late adulthood with painless LAD

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57
Q

what is mantel cell lymphoma driven by

A

t(11:14)

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58
Q

what is highly over expressed in mantle cell lymphoma

A

cyclin D1

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59
Q

what is the clinical presentation of Burkitt lymphoma

A

extra nodal mass in kids or young adults

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60
Q

what does the african form of burkitt lymphoma typically involves

A

jaw

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61
Q

what does the sporadic form of burkitt lymphoma typically involve

A

abdomen

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62
Q

what translocation is common in burkitt lymphoma

A

c-myc (chr 8); t(8:14)

63
Q

what lymphoma has starry sky appearance

A

Burkitt lymphoma

64
Q

what is the most common form of NHL

A

DLBCL

65
Q

what should you think of with reed-sternberg cells

A

hodgkin lymphoma

66
Q

describe the nucleus in reed sternberg cells

A

CD15+, CD30+; owl-eyed nucleus

67
Q

what subtype of hodgkin lymphoma has the best prognosis

A

lymphocyte rich

68
Q

what subtype of hodgkin lymphoma is associated with abundant eosinophils

A

mixed cellularity type

69
Q

what subtype of hodgkin lymphoma has the worst prognosis and is seen in the elderly or HIV+

A

lymphocyte depleted

70
Q

how do the RS cells present in nodular sclerosis type of hodgkin lymphoma

A

lake-like spaces (lacunar cells)

71
Q

what is the term for malignant proliferation of plasma cells in bone marrow

A

multiple myeloma

72
Q

what is the most common primary malignancy of bone

A

multiple myeloma

73
Q

describe the Xray seen in multiple myeloma

A

lytic punched out lesions seen on X-ray, especially in vertebrae and skull

74
Q

what is the most common cause of death in multiple myeloma

A

infection

75
Q

what should you think of with rouleaux formation on blood smear

A

multiple myeloma

76
Q

what is primary AL amyloidosis a consequence of

A

multiple myeloma

77
Q

how is the proteinuria seen in primary AL amyloidosis excreted

A

as Bence-jones proteins

78
Q

what is the term or B cell lymphoma with monoclonal IgM production

A

waldenstrom macroglobulinemia

79
Q

what is the clinical presentation of waldenstrom macroglobulinemia

A

generalized LAD; lytic bone lesions are NOT present; increased serum protein with M spike; visual and neurological deficits

80
Q

what should you think of with birbeck/tennis racket lesions on EM

A

langerhans cell histiocytosis

81
Q

how is letterer-siwe disease presented

A

skin rash and cystic skeletal defects in an infant

82
Q

what is the term for benign proliferation of langerhans cells in bone

A

eosinophilic granuloma

83
Q

what is the presentation of eosinophilic grandma

A

pathologic fracture in adolescents; skin NOT involved

84
Q

what is the presentation of Hand-schuller-christian disease

A

scalp rash, lytic skull defects, DI, exophthalmos in a kid

85
Q

What is the chief site of blood formation until shortly before birth

A

Liver

86
Q

What is the term for abnormal release of immature precursors into the peripheral blood

A

Leukoerythroblastosis

87
Q

What is the normal range for adult granulocytes

A

40-70%

88
Q

What is the most common cause of agranolocytosis in neutropenia

A

Drug toxicity

89
Q

What congenital condition has impairment of granulocytic differentiation

A

Kostmann syndrome

90
Q

What is the most common consequence of agranulocytosis

A

Infections

91
Q

What is the term for patches of dilated ER that appear as sky-blue cytoplasmic puddles seen with leukocytosis

A

Dohle bodies

92
Q

What is the term for coarser and darker than normal neutrophilic granules seen in leukocytosis

A

Toxic granules

93
Q

What can cause leukocytosis due to increased release from marrow stores

A

Endotoxemia, infection, hypoxia

94
Q

What can cause decreased margin action leading to leukocytosis

A

Exercise, catecholamines

95
Q

How does acute nonspecific lymphadenitis present

A

Painful red abscess

96
Q

What is acute non-specific lymphadenitis in the axillary or inguinal regions most often caused by

A

Infections in extremities

97
Q

What are some causes of follicular hyperplasia

A

Rheumatoid arthritis, toxoplasmosis, early stages of HIV

98
Q

What is another name for reticular hyperplasia

A

Sinus histiocytosis

99
Q

What are myodysplastic myeloid neoplasms associated with

A

Ineffective hematopoiesis and resultant peripheral blood cytopenias

100
Q

What do Bloom and Fanconi increase the risk of

A

Acute leukemia

101
Q

What is the origin of most lymphoid neoplasms

A

B cell origin

102
Q

What is the primary T cell associated with CD1

A

Thymocytes and langerhans cells

103
Q

What are the primary T cells associated with CD3

A

Thymocytes, mature T cells

104
Q

What are the primary B cells associated with CD10

A

Pre-B cells and germinal center B cells

105
Q

What are the primary B cells associated with CD19

A

Pre B cells and mature B cells but not plasma cells

106
Q

What is the EBC receptor

A

CD21

107
Q

What B cells is CD23 primarily associated with

A

Activated mature B cells

108
Q

What is the primary cell associated with CD14

A

Monocytes

109
Q

What are the primary cells associated with CD15

A

Granulocytes’ Reed-Sternberg cells and variants

110
Q

What cells are associated with CD64

A

Mature myeloid cells

111
Q

What is the most common cancer of childhood

A

ALL

112
Q

What is the peak incidence for B-ALL

A

Age 3

113
Q

What is mutation in T-ALLs

A

NOTCH1

114
Q

Are lymphoblasts myeloperoxidase positive or negative

A

Negative

115
Q

What is the specialized DNA polyermase that is expressed only in pre-B and pre-T lymphoblasts and is positive in 95% of classes in ALL

A

TdT

116
Q

What do pre-B cells usually epxress

A

CD19, PAX5, CD10

117
Q

What is the most common leukemia of adults in western world

A

CLL/SLL

118
Q

What are human CLL cells dependent on

A

BTK pathway

119
Q

What is the immunophenotype in CLL,SLL

A

CD19+, CD20+, CD23+, CD5+

120
Q

What is the most common form of indolent NHL

A

Follicular lymphoma

121
Q

What is follicular lymphoma strongly associated with chromosomal translocations involving

A

BCL2

122
Q

What is disregulated in diffuse large B cell lymphoma

A

BCL6

123
Q

What is primary effusion lymphoma associated with

A

KSHV/HHV-8

124
Q

What translocation is involved in Burkitt lymphoma

A

MYC gene on chromosome 8

125
Q

What is the immunophenotype of Burkitt lymphoma

A

Express IgM, CD19, CD20, CD10, BCL6; almost always FAILS to express BCL2

126
Q

What is the most common and deadly plasma neoplasm

A

Multiple myeloma

127
Q

What heavy chain gene rearrangement is common in multiple myeloma

A

Chromosome 14q32

128
Q

What is the most common plasma cell dyscrasia

A

MGUS

129
Q

What is the genotype on large granular lymphocytic leukemia

A

Point mutations in STAT3

130
Q

What mutations are associated with lymphoplasmacytic lymphoma

A

Acquired mutations in MYD88

131
Q

When you see Russell bodies or ditched bodies, what should you think of

A

Lymphoplasmacytic lymphoma

132
Q

What infectio. Must be present to get adult T-cell leukemia/lymphoma

A

HTLV-1

133
Q

What does HTLV-1 encode

A

Tax

134
Q

What should you think of with cloverleaf or flower cells

A

Adult T cell leukemia or lymphoma

135
Q

What is the triad associated with felty syndrome

A

Rheumatoid arthritis, splenomegaly, neutropenia

136
Q

What presentation is most common with extranodal NKT-cell lymphoma

A

Destructive nasopharyngeal mass

137
Q

What are the classic type of Hodgkin lymphoma positive for

A

PAX5, CD15, CD30

138
Q

What type of Hodgkin lymphoma is NOT associated with EMV

A

Lymphocyte predominance

139
Q

What disease should you think of with Auer rods

A

AML

140
Q

What type of syndromes should you think of with pseudo-pelger-Huet cells and pawn ball megakaryocytes

A

Disordered differentiation in myelodysplastic syndrome

141
Q

What is the common mutation in chronic myelogenous leukemia

A

BCR-ABL fusion gene

142
Q

What is the mutations in essential thrombocytopenia

A

JAK2 and MPL point mutations

143
Q

What is the mutation in systemic mastocytosis

A

KIT point mutations

144
Q

What should you think of with sea-blue histiocytes

A

CML

145
Q

How is essential thrombocytosis separated from PCV and primary myelofibrosis

A

Based on absence of polycythemia and marrow fibrosis, respectively

146
Q

What disorder is erthromelalgia seen in

A

Essential thrombocytosis

147
Q

What is the term for throbbing and burning of hands and feet caused by occlusion of small arterioles by platelet aggregation

A

Essential thrombocytosis

148
Q

What is the chief pathology feature of primary myelofibrosis

A

Extensive deposition of collagen in marrow by non-neoplasticism fibroblasts

149
Q

How does primary myelofibrosis come to attention

A

Because of progressive anemia and splenomegaly, which produces sensation of fullness in LUQ

150
Q

What is the hand-Schiller-Christian triad associated with

A

Eosinophilic granuloma

151
Q

What is pulmonary langerhans cell histiocytosis most common in

A

Adult smokers

152
Q

What do neoplastic langerhans cells express

A

Both CCR6 and CCR7

153
Q

Where do most thymomas arise

A

Anterior superior mediastinum

Lecom pathology year 2 (18 decks)

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