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Flashcards in White Blood Cell Disorders Deck (232)
1

What is the CD molecule for hematopoietic stem cells?

CD34

2

What is a normal leukocyte count?

5,000 - 10,000

3

What class of drugs can cause leukopenia?

alkylating agents

4

What cell type is the most susceptible during leukopenia?

neutrophils

5

Can gram-negative or gram-positive sepsis result in leukopenia?

gram-negative

6

What drug is used to treat leukopenia?

GM-CSG or G-CSF

7

What type of cell is most susceptible to radiation?

lymphocyte

8

What genetic disease can cause lymphopenia?

DiGeorge

9

What do corticosteroids do to lymphocytes?

induce apoptosis

10

What are the two causes of leukocytosis?

bacterial infection

tissue necrosis

11

What CD molecule if the Fc receptor?

CD16

12

Immature neutrophils are deficient in what molecule?

CD16 (Fc receptor)

13

What are the four downstream cells types of myeloid cells?

Megakaryoblast, Erythroblast, Monoblast, Myeloblast

14

What are the three types of myeloblasts?

neutrophils, eosinophils and basophils

15

What do steroids do to neutrophils? Why?

neutrophilia

releases marginated pool of neutrophils

16

What do steroids do to eosinophils? Why?

eosinopenia

sequestions eosinophils in lymph nodes

17

What do steroids do to lymphocytes? Why?

lymphopenia

induce apoptosis of lymphocytes

18

What two disease states can cause monocytosis?

chronic inflammation

malignancy

19

What type of Neoplasia can cause eosinophilia?

Hodgkin Lymphoma

20

What three cell types can increase during CML?

neutrophils, metemyelocytes, basophils

21

What type of lymphocyte can cause hyperplasia in response to virally infected cells?

CD8+

22

Which bacteria produces lymphocytosis promoting factor? How does this toxin work?

Bordatella pertussis

prevents lymphocytes from leaving the blood

23

What does lymphocytosis promoting factor do?

prevent lymphocytes from leaving the blood stream to enter the lymph nodes

24

What two viral infections can cause infectious mononucleosis?

EBV and CMV

25

What sort of leukocytosis can occur during Infectious Mononucleosis?

Lymphocytic Leukocytosis

26

What type of lymphocytes increase in the blood during Lymphocytic Leukoytosis?

CD8+

27

What three body parts can be infected during mono?

oropharynx

Liver

B-cells

28

Where in the lymph nodes do T-cells hypertrophy during mononucleosis?

Paracortex

29

What section of the spleen do T-cells hypertrophy during mononucleosis?

Periarterial Lymphatic Sheath (PALS)

30

Where in the lymph nodes do B-cells hypertrophy?

cortex

31

Lymphocytic leukocytosis features what type of CD8+ cell?

reactive

32

The monospot test detects what specifc type of immunoglobulin? What cell type can these AB's bind?

heterophile IgM

animal RBCs

33

A negative monospot test is indicative of what viral infection?

CMV

34

A definitive test for EBV is detection of the presence of what?

EBV viral capsid antigen

35

What is the largest complication of EBV?

splenic rupture

36

A person with Infectious Mononucleosis should not be treated with what drug? Why?

Ampicillin

rash

37

What type of cancer can develop in patients with dormant EBV?

B-cell lymphoma

38

What cell organelle is abnormally found during an acute leukemia? What cell type? What does it look like?

nucleolus

immature blasts

punched out

39

What blast expresses TdT? What two specific cells? Where in the cell is TdT located?

lymphoblasts

pre-B cells and pre-T cells

nucleus

40

What is more common, B-ALL or T-ALL?

B-ALL

41

What three CD molecules are often found on cancerous cells in B-ALL?

CD10, CD19 and CD20

42

What ALL translocation has a good prognosis and is often seen in children?

t(12:21)

43

What ALL translocation has a poor prognosis and is often seen in adults?

t(9:22)

44

Is CD10 seen on pre-B or pre-T cells?

pre-B

45

What range of CD molecules would T-ALL present with?

CD2 - CD8

46

What enzyme is a classic marker for myeloblasts? What structure can this marker crystallize into?

Myeloperoxidase

auer rods

47

What type of enzyme is TdT?

DNA polymerase

48

Where in the body does T-ALL usually form a mass?

mediastinum

49

What is the named for aggregates of MPO?

Auer rods

50

Are Auer rods seen in myeloblastic leukemia or lymphoblastic leukemia?

Myeloblastic

51

What translocation is most often seen in Acute Promyelocytic Leukemia? What gene is involved? What move?

t(15:17)

retinoic acid receptor from 17 to 15

52

WHat does retinoic acid receptor translocation prevent during Acute Promyelocytic Leukemia?

maturation of promyelocytes

53

What hematological abnormality can AML produce? Why? Which type?

DIC

promyelocytes can release primary granules

M3

54

What is the specific treatment for acute promyelocytic leukemia?

all-trans retinoic acid

55

How is acute monoblastic leukemia differentiated from acute myeloblastic leukemia?

monoblastic = lack myeloperoxidase

56

What part of the body does acute monoblastic leukemia often invade?

gums

57

What type of leukemia develops in pts with Downs Syndrome AFTER the age of 5?

ALL

58

What specific type of leukemia develops in pts with Downs Syndrome BEFORE the age of 5? After 5?

acute megakaryoblastic leukemia

ALL

59

Previous exposure to what two agents can result in AML?

alkylating agents or radiation

60

What is the difference between acute and chronic leukemias?

acute = neoplastic proliferation of immature cells

chronic = neoplastic proliferation of mature cells

61

In general, does CML effect younger or older individuals?

older

62

What are the two CD markers for CLL? Are these B-cells or T-cells?

CD5 and CD20

B-cells

63

What type of leukemia are Smudge Cells seen?

CLL

64

What is it called when CLL invades the lymph nodes?

Small Lymphocytic Lymphoma

65

What is the most common cause of death in CLL? Why?

infection

hypogammaglobulinemia

66

Hairy Cell Leukemia is a neoplastic proliferation of what type of cell?

Mature B-cells

67

What stain is used to identify Hairy Cell Leukemia?

TRAP

68

Where in the body do 'Hairy Cells' accumulate? Causing?

red pulp of spleen

splenomegaly

69

What type of leukemia produces bone marrow fibrosis?

Hairy Cell

70

What is the Tx of Hairy Cell Leukemia? What is the MOA of this drug?

cladribine

purine analogue inhibitor of adenosine deaminase

71

Does Adult T-cell Leukemia/Lymphoma feature a proliferation of CD4+ or CD8+ T-cells? Naive or mature?

CD4+

mature

72

What causes Adult T-cell Leukemia/Lymphoma? What two locations?

HTLV1

Japan and Caribbean

73

Does ATLL have hyer or hypo calcemia?

hyper

74

Mycosis fungoides features a proliferation of what cell type? Naive or mature?

CD4+

mature

75

What layer of the skin receives infiltrates during Mycosis Fungoides? What are these infiltrates called? What type of cells?

epidermis

Pautrier microabscesses

Neoplastic Tcells

76

What disease is it called if Mycosis Fungoides invades the blood?

Sezary Syndrome

77

What is the buzz word for what the neoplastic cells look like during Sezary Syndrome? Resembling?

cerebriform nuclei

brain

78

What metabolite can expand during a Myeloproliferative Disorder?

uric acid

79

What is Chronic Myeloid Leukemia?

neoplastic proliferation of mature myeloid cells

80

What cell type especially proliferates during CML? What specific cell?

granulocytes

basophils

81

What fusion protein drives CML?

Bcr-Abl

82

What is the first-line treatment of CML? What enzymatic activity does this treatment block?

imatinib

tyrosine kinase

83

Splenomegaly during CML suggests what?

transformation to AML or ALL

84

What test differentiates CML from a leukomoid rxn?

CML will not have increase leukocyte alkaline phos.

Leukomoid rxn will

85

What type of cell is present in a CML that is not present in a leukomoid rxn?

basophil

86

What three cell types increase during polycythemia vera?

RBCs

granulocytes (NEBM)

platelets

87

What type of mutation is present during PV?

JAK2 kinase

88

What is Budd-Chiari Syndrome? What condition most often causes Budd-Chiari Syndrome?

occlusion of hepatic veins leading to liver infarct

polycythemia vera

89

What is the characteristic sign of PV? Why does this happen?

itching after warm bathing

degranulation of mast cells release histamine

90

What drug is used to treat PV?

hydroxyurea

91

Does Polycythemia Vera have a high or low level of Epo?

low Epo

92

What type of neoplasia can produce high levels of Epo?

Renal Cell Carcinoma

93

Essential thrombocytopenia is caused by a mutation in what?

JAK2

94

Does Essentil thrombocytosis have an increased or decreased risk of bleeding? Why?

increased

platelets sequester too many clotting factors

95

Myelofibrosis features an over proliferation of what cell type? What cytokine is produced in excess that drives myelofibrosis?

megakaryocytes

PDGF

96

What mutation is present during myelofibrosis?

JAK2

97

What cytokine drives myelofibrosis?

PDGF

98

Why does splenomegaly occur during myelofibrosis?

extra-medullary hematopoiesis

99

Which lymphocyte lives in the follicle of a lymph node?

follicle = B-cell

100

What two (non-neoplastic) diseases can produce follicular hyperplasia?

Rheumatoid

HIV early stages

101

What type of lymphocyte lives in the paracortex? What type of infection would drive paracortex hyperplasia?

T-cell

viral

102

Hyperplasia of sinus histiocytes is seen during what disease?

lymph node draining a cancerous region

103

Which lymphoma spreads intranodally?

Hodgkins

104

Which lymphoma spreads extra-nodally?

non-Hodgkins

105

Which CD molecule is the key marker for B cells?

CD20

106

What translocation drives follicular cell lymphoma?

t(14:18)

107

Regarding follicular cell lymphoma, what is present on 14? 18?

14 = Ig heavy chain

18 = bcl-2

108

What is the function of bcl-2?

inhibit apoptosis

109

What is the Tx for follicular cell lymphoma? What molecule does rituximab attack?

Rituximab

CD20

110

If a patient with Follicular Cell Lymphoma begins to present with an enlarging lymph node, what disease should one suspect?

Diffure large B-cell lymphoma

111

What translocation drives mantle cell lymphoma?

t(11:14)

112

Regarding mantle cell lymphoma, what is on chromosome 14? Chromosome 18?

11 = cyclin D1

14 = Ig heavy chain

113

Which cell cycle transition is activated during mantle cell lymphoma?

G1/S

114

Marginal Zone lymphoma is associated with what three diseases?

H. pylori infection

Hashimoto's Thyroiditis

Sjogren syndrome

115

What virus is Burkitt's Lymphoma associated with?

EBV

116

What translocation is present in Burkitt's Lymphoma? What protein is over-produced?

t(8:14)

c-Myc

117

What is the key phrase for the histological appearance of Burkitts Lymphoma?

Starry Sky

118

What is the most common type of NHL? What is the prognosis of this disease?

Diffuse large B-cell lymphoma

poor

119

Which CD molecules are present on Reed-Sternberg cells?

CD15 and CD30

120

Why do reactive inflammatory cells make up the bulk of Hodgkin Lymphoma cells?

RS cells secretet cytokines that attract them

121

What are the four types of HL?

Nodular sclerosing

Lymphocyte rich

Mixed cellularity

Lymphocyte depleted

122

What is the most common type of HL?

Nodular Sclerosing

123

What two locations does nodular sclerosis often present?

cervical lymph node

mediastinal lymph node

124

What sex does nodular sclerosis most often present? Age?

female

teenager

125

Which type of Hodgkins Lymphoma has the best prognosis?

Lymphocyte rich

126

What interleukin can Reed Sternberg cells produce? What granulocyte does this attract?

IL5

eosinophil

127

What type of Hodgkins lymphome is the most aggressive? What two population groups is this most often found?

lymphocyte depleted

HIV+ pt's and the elderly

128

What type of cell proliferates during multiple myeloma? Where is this cell found?

Plasma

bone marrow

129

Which interleukin can drive Multiple Myeloma?

IL-6

130

What disease is associated with CRAB? What does CRAB stand for?

CRAB = multiple myeloma

elevated Calcium
real insufficiency
Anemia
bone lesions

131

Neoplastic plasma cells of Multiple Myeloma can activate with receptor?

RANK

132

What two locations of the skeleton are most susceptible to bone lesions during multiple myeloma?

skull and vertebrae

133

What two immunoglobulin isotypes are most often found during multiple myeloma M-spike?

IgA and IgG

134

What is the most common cause of death in multiple myeloma? Why?

infection

neoplastic plasma cells only produce monoclonal Ab;s

135

Do neoplastic plasma cells overproduce light chain or heavy chain? What can this lead to?

light chain

amyloidosis

136

Bence Jones protein is indicative of what disease? Where is it found? What composes it?

Multiple Myeloma

urine

Ig light chain

137

What is the only similarity between Multiple Myeloma and MGUS?

M-spike

138

What Ig isotype is produced during Waldenstroms Macroglobulinemia?

IgM

139

What two systems are primarily effected during Waldenstroms Macroglobulinemia? Such as?

vision and CNS

retinal damage and stroke

140

Does Waldenstroms Macroglobulinemia have an increased or decreased liklihood of bleeding? Why?

increased

viscous serum results in defective aggregation

141

What cells are Langerhans cells derived from? What type of cells are langerhans cells?

monocytes

dendritic cells

142

What type of protein is found in the urine of pt's with Langerhans Cell Histiocytosis?

Birbeck granules

143

What are the three immunohistochemical markers of Langerhans Cell Histiocytosis?

CD1a and S100 and CD207

144

Proliferation of what type of cell is seen during Letterer-Siwe disease?

Langerhans cells

145

What is the classic presentation of a patient with Letterer-Siwe Syndrome? What age group?

Skin rash and cystic skeletal lesions

Infant

146

What cell type proliferates during an Eosinophilic Granuloma? Where?

Langerhans

Bone

147

What cell type proliferates during Hand-Schuler-Christian Disease?

Langerhans

148

What is the triad of Hand-Schuler-Christian Disease?

diabetes insipidus

lytic bone lesions

exopthalmos

149

What does a high cortisol state due to neutrophil numbers? Why?

increases

causes neutrophils to be released from marginated pool (lungs)

150

Basophilis is clasically associated with what disease?

CML

151

What are the two main causes of lymphocytic leukocytosis?

Viral infection

Bordatella Pertussis Infection

152

Why does splenomegaly occur during mononucleosis?

T-cell hyperplasia in PALS

153

Infectious mononucleosis features what specific type of T-cell?

reactive CD8+ T-cell

154

Acute leukemia is defined as greater than what percent of blasts in the bone marrow?

20%

155

What two sites require ancillary chemotherapy during ALL? Why?

scrotum and CSF

BTB and BBB

156

Where does T-ALL usually manifest? What age group?

thymus

teenagers

157

What is another name for a T-ALL located in the thymus?

Acute Lymphoblastic Lymphoma

158

How is acute monocytic leukemia differentiated from acute promyelocytic leukemia?

monocytic leukemia = no myeloproxidase

159

Where does Acute Monoblastic Leukemia clasically infiltrate?

gums

160

Do megakaryocytes have MPO?

no

161

What are the three major complications of CLL?

Hypogammaglobulinemia

AI hemolytic anemia

Transformation into diffuse large B-cell lymphoma

162

What is the transformation of CLL to Diffuse large B-cell lymphoma characterized by?

growing lymph node

163

What are the three common features of Hairy Cell Leukemia?

Splenomegaly

Bone Marrow fibrosis (dry tap)

no lymphadenopathy

164

What cause the splenomegaly in Hairy Cell Leukemia?

hairy cells accumulating in red pulp of spleen

165

Lytic bone lesions is a knee-jerk rxn for what disease? Lytic bone lesions with a rash is indicative of what disease?

multiple myeloma

Human T-cell leukemia/lymphoma

166

During a myeloproliferative disorder, which cells increase in number?

all cell types increase in number

167

What is the definition of a myeloproliferative disorder?

neoplastic proliferation of mature cells of the myeloid lineage

168

What is a common complication of all myeloproliferative disorders?

hyperuricemia

169

What two states can a myeloproliferative disorder progress to?

marrow fibrosis

acute leukemia

170

What is the accelerated phase of CML marked by?

splenomegaly

171

What are the three physical symptoms of polycythemia vera?

blurry vision and headache

flushed face

itching after bathing

172

Other than RBCs, what other cell often expands in number during polycythemia vera that can cotribute to its diagnosis?

mast cells

173

What are the two treatments of polycythemia vera?

1. Phlebotomy

2. Hydroxyurea

174

What myelodysplastic syndrome does not have an increased risk of hyperuricemia?

Essential thrombocythemia

175

Why does myelofibrosis result in extramedullary hematopoiesis?

no bone marrow for hematopoiesis

176

What protein prevents immature blood cells from leaving the bone marrow? Is this protein present in the spleen?

reticulin

no

177

What non-metastatic and non-infective disorder can cause follicular lymphadenopathy?

Rheumatoid Arthritis

178

What virus is known to cause follicular lymphadenopathy?

HIV

179

Where is the lymph node do sinus histiocytes live?

medulla

180

What is a lymphoma?

neoplastic proliferation of lymphoid cells that form a mass

181

When does follicular lymphoma usually present?

late adulthood

182

What does a follicular lymphoma look like histologically?

dozens of follicles

183

What can a follicular lymphoma transform into?

Diffuse Large B-cell Lymphoma

184

Where in the lymph node do the follicles live?

cortex

185

What is the difference between follicular cell lymphoma and follicular hyperplasia?

lymphoma = follicles all over cell instead of just cortex

186

Which form, hyperplasia or lymphoma, results with tingible body macrophages?

hyperplasia due to infection

187

Which is monoclonal, follicular lymphoma or follicular hyperplasia?

lymphoma

188

What is the proliferation of kappa to lambda light chain in non-neoplastic B-cells?

3:1

189

When does mantle cell lymphoma present?

late adult hood

190

What condition produces a marginal zone of a lymph node?

chronic inflammation

191

What is the most distinguishing characteristic of a Reed-Sternberg cell?

prominent nucleoli

192

Why is a mass produced during Hodgkin lymphoma?

RS cells secrete cytokines that attract other cells

193

Why does Hodgkin Lymphoma present with typical B-cell symptoms?

RS secretion of cytokines

194

What is the specific cell type found in HL Nodular Sclerosis?

Lacunar cell

195

What type of HL produces IL5? What cell type does this attract

mixed-cellularity

eosinophil

196

Is the M-spike of multiple myeloma caused by monoclonal or polyclonal Ab's?

Monoclonal

197

What causes a Rouleaux formation? What disease?

high protein in serum decreases charge between RBCs

Multiple Myeloma

198

What protein deposits in the urine during Multiple Myeloma? Composed of?

bence jones

Ig light chains

199

What age group is MGUS most prevalent?

elderly

200

What type of specific neoplasia is Waldenstroms Macroglobulinemia?

B-cell lymphoma

201

Does Waldenstroms Macroglobulinemia present with lytic bone lesions?

no

202

Does Waldenstroms Macroglobulinemia have an M-spike?

yes

203

Why does Waldenstroms Macroglobulinemia present with CNS complications?

high IgM makes blood hyper-viscous

204

What is the specific function of Langerhans cells?

present antigen to naive T-cells

205

Is eosinophilic granuloma benign or pathological?

benign

206

What is the classic presentation for a patient with eosinophilic granuloma?

pathological bone fracture in adolescent

207

What type of aggressive neoplasm can follicular lymphoma transform into?

diffuse large B-cell lymphoma

208

Letterer-Siwe Disease is a malignant proliferation of what cell?

Langerhans cell

209

What is the classic presentation of Hand Schuler Christian Disease?

scalp rash and lytic bone defects

210

Do high cortisol levels cause eosinophilia or eosinopenia?

eosinopenia

211

Do megakaryocytes express MPO?

no

212

Are B-cells in CLL naive or mature?

Naive

213

Are myeloproliferative disorders an expansion of mature or immature cells?

mature

214

Does an Eosinophilic Granuloma involve a skin rash?

no

215

What virus can cause a nasopharyngeal carcinoma?

EBV

216

Where does EBV maintain dormancy?

B-cells

217

Which form of ALL usually presents in teenagers?

T-ALL

218

What is the average age of development of AML?

50-60

219

How can PV be distinguished from Essential Thrombocytosis?

PV runs a risk or hyperuricemia

ET does not

220

Which form of HL causes the lacunar type of RS cell?

Nodular Sclerosing

221

What two forms of Hodgkins Lymphoma is EBV infection associated with?

mixed-cellularity

lymphocyte depleted

222

How is Waldenstroms Treated?

plasmapheresis

223

What is the difference between an Eosinophilic Granuloma and Letterer-Siwe Disease?

Eosiniphilic granuloma doesnt involve a rash

224

Which cell is most often found in an Eosinophilic Granuloma?

Eosinophils

225

Greater than what percent of blasts have to be present in the blood to Dx an Acute Leukemia?

20%

226

What are the two translocations of ALL?

12:21

9:22

227

Which translocation of ALL has a poor prognosis?

9;22

228

Which translocation of ALL has a good prognosis?

12:21

229

What is a Richter Transformation?

CLL into large B-cell lymphoma

230

Is lymphocytic leukocytosis caused by viruses or bacteria?

virus

231

Which two leukemias can CML transform into?

AML or ALL

232

Where is the mantle located in the lymph node?

immediately adjacent to the follicle