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Pathoma > White Blood Cell Disorders > Flashcards

White Blood Cell Disorders Flashcards

1
Q

What is the CD molecule for hematopoietic stem cells?

A

CD34

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2
Q

What is a normal leukocyte count?

A

5,000 - 10,000

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3
Q

What class of drugs can cause leukopenia?

A

alkylating agents

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4
Q

What cell type is the most susceptible during leukopenia?

A

neutrophils

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5
Q

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

A

gram-negative

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6
Q

What drug is used to treat leukopenia?

A

GM-CSG or G-CSF

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7
Q

What type of cell is most susceptible to radiation?

A

lymphocyte

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8
Q

What genetic disease can cause lymphopenia?

A

DiGeorge

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9
Q

What do corticosteroids do to lymphocytes?

A

induce apoptosis

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10
Q

What are the two causes of leukocytosis?

A

bacterial infection

tissue necrosis

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11
Q

What CD molecule if the Fc receptor?

A

CD16

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12
Q

Immature neutrophils are deficient in what molecule?

A

CD16 (Fc receptor)

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13
Q

What are the four downstream cells types of myeloid cells?

A

Megakaryoblast, Erythroblast, Monoblast, Myeloblast

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14
Q

What are the three types of myeloblasts?

A

neutrophils, eosinophils and basophils

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15
Q

What do steroids do to neutrophils? Why?

A

neutrophilia

releases marginated pool of neutrophils

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16
Q

What do steroids do to eosinophils? Why?

A

eosinopenia

sequestions eosinophils in lymph nodes

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17
Q

What do steroids do to lymphocytes? Why?

A

lymphopenia

induce apoptosis of lymphocytes

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18
Q

What two disease states can cause monocytosis?

A

chronic inflammation

malignancy

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19
Q

What type of Neoplasia can cause eosinophilia?

A

Hodgkin Lymphoma

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20
Q

What three cell types can increase during CML?

A

neutrophils, metemyelocytes, basophils

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21
Q

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

A

CD8+

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22
Q

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

A

Bordatella pertussis

prevents lymphocytes from leaving the blood

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23
Q

What does lymphocytosis promoting factor do?

A

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

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24
Q

What two viral infections can cause infectious mononucleosis?

A

EBV and CMV

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25
Q

What sort of leukocytosis can occur during Infectious Mononucleosis?

A

Lymphocytic Leukocytosis

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26
Q

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

A

CD8+

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27
Q

What three body parts can be infected during mono?

A

oropharynx

Liver

B-cells

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28
Q

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

A

Paracortex

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29
Q

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

A

Periarterial Lymphatic Sheath (PALS)

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30
Q

Where in the lymph nodes do B-cells hypertrophy?

A

cortex

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31
Q

Lymphocytic leukocytosis features what type of CD8+ cell?

A

reactive

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32
Q

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

A

heterophile IgM

animal RBCs

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33
Q

A negative monospot test is indicative of what viral infection?

A

CMV

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34
Q

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

A

EBV viral capsid antigen

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35
Q

What is the largest complication of EBV?

A

splenic rupture

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36
Q

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

A

Ampicillin

rash

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37
Q

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

A

B-cell lymphoma

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38
Q

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

A

nucleolus

immature blasts

punched out

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39
Q

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

A

lymphoblasts

pre-B cells and pre-T cells

nucleus

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40
Q

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

A

B-ALL

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41
Q

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

A

CD10, CD19 and CD20

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42
Q

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

A

t(12:21)

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43
Q

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

A

t(9:22)

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44
Q

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

A

pre-B

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45
Q

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

A

CD2 - CD8

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46
Q

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

A

Myeloperoxidase

auer rods

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47
Q

What type of enzyme is TdT?

A

DNA polymerase

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48
Q

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

A

mediastinum

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49
Q

What is the named for aggregates of MPO?

A

Auer rods

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50
Q

Are Auer rods seen in myeloblastic leukemia or lymphoblastic leukemia?

A

Myeloblastic

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51
Q

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

A

t(15:17)

retinoic acid receptor from 17 to 15

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52
Q

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

A

maturation of promyelocytes

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53
Q

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

A

DIC

promyelocytes can release primary granules

M3

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54
Q

What is the specific treatment for acute promyelocytic leukemia?

A

all-trans retinoic acid

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55
Q

How is acute monoblastic leukemia differentiated from acute myeloblastic leukemia?

A

monoblastic = lack myeloperoxidase

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56
Q

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

A

gums

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57
Q

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

A

ALL

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58
Q

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

A

acute megakaryoblastic leukemia

ALL

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59
Q

Previous exposure to what two agents can result in AML?

A

alkylating agents or radiation

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60
Q

What is the difference between acute and chronic leukemias?

A

acute = neoplastic proliferation of immature cells

chronic = neoplastic proliferation of mature cells

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61
Q

In general, does CML effect younger or older individuals?

A

older

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62
Q

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

A

CD5 and CD20

B-cells

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63
Q

What type of leukemia are Smudge Cells seen?

A

CLL

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64
Q

What is it called when CLL invades the lymph nodes?

A

Small Lymphocytic Lymphoma

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65
Q

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

A

infection

hypogammaglobulinemia

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66
Q

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

A

Mature B-cells

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67
Q

What stain is used to identify Hairy Cell Leukemia?

A

TRAP

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68
Q

Where in the body do ‘Hairy Cells’ accumulate? Causing?

A

red pulp of spleen

splenomegaly

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69
Q

What type of leukemia produces bone marrow fibrosis?

A

Hairy Cell

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70
Q

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

A

cladribine

purine analogue inhibitor of adenosine deaminase

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71
Q

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

A

CD4+

mature

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72
Q

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

A

HTLV1

Japan and Caribbean

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73
Q

Does ATLL have hyer or hypo calcemia?

A

hyper

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74
Q

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

A

CD4+

mature

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75
Q

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

A

epidermis

Pautrier microabscesses

Neoplastic Tcells

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76
Q

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

A

Sezary Syndrome

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77
Q

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

A

cerebriform nuclei

brain

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78
Q

What metabolite can expand during a Myeloproliferative Disorder?

A

uric acid

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79
Q

What is Chronic Myeloid Leukemia?

A

neoplastic proliferation of mature myeloid cells

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80
Q

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

A

granulocytes

basophils

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81
Q

What fusion protein drives CML?

A

Bcr-Abl

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82
Q

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

A

imatinib

tyrosine kinase

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83
Q

Splenomegaly during CML suggests what?

A

transformation to AML or ALL

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84
Q

What test differentiates CML from a leukomoid rxn?

A

CML will not have increase leukocyte alkaline phos.

Leukomoid rxn will

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85
Q

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

A

basophil

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86
Q

What three cell types increase during polycythemia vera?

A

RBCs

granulocytes (NEBM)

platelets

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87
Q

What type of mutation is present during PV?

A

JAK2 kinase

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88
Q

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

A

occlusion of hepatic veins leading to liver infarct

polycythemia vera

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89
Q

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

A

itching after warm bathing

degranulation of mast cells release histamine

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90
Q

What drug is used to treat PV?

A

hydroxyurea

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91
Q

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

A

low Epo

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92
Q

What type of neoplasia can produce high levels of Epo?

A

Renal Cell Carcinoma

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93
Q

Essential thrombocytopenia is caused by a mutation in what?

A

JAK2

94
Q

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

A

increased

platelets sequester too many clotting factors

95
Q

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

A

megakaryocytes

PDGF

96
Q

What mutation is present during myelofibrosis?

A

JAK2

97
Q

What cytokine drives myelofibrosis?

A

PDGF

98
Q

Why does splenomegaly occur during myelofibrosis?

A

extra-medullary hematopoiesis

99
Q

Which lymphocyte lives in the follicle of a lymph node?

A

follicle = B-cell

100
Q

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

A

Rheumatoid

HIV early stages

101
Q

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

A

T-cell

viral

102
Q

Hyperplasia of sinus histiocytes is seen during what disease?

A

lymph node draining a cancerous region

103
Q

Which lymphoma spreads intranodally?

A

Hodgkins

104
Q

Which lymphoma spreads extra-nodally?

A

non-Hodgkins

105
Q

Which CD molecule is the key marker for B cells?

A

CD20

106
Q

What translocation drives follicular cell lymphoma?

A

t(14:18)

107
Q

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

A

14 = Ig heavy chain

18 = bcl-2

108
Q

What is the function of bcl-2?

A

inhibit apoptosis

109
Q

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

A

Rituximab

CD20

110
Q

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

A

Diffure large B-cell lymphoma

111
Q

What translocation drives mantle cell lymphoma?

A

t(11:14)

112
Q

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

A

11 = cyclin D1

14 = Ig heavy chain

113
Q

Which cell cycle transition is activated during mantle cell lymphoma?

A

G1/S

114
Q

Marginal Zone lymphoma is associated with what three diseases?

A

H. pylori infection

Hashimoto’s Thyroiditis

Sjogren syndrome

115
Q

What virus is Burkitt’s Lymphoma associated with?

A

EBV

116
Q

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

A

t(8:14)

c-Myc

117
Q

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

A

Starry Sky

118
Q

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

A

Diffuse large B-cell lymphoma

poor

119
Q

Which CD molecules are present on Reed-Sternberg cells?

A

CD15 and CD30

120
Q

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

A

RS cells secretet cytokines that attract them

121
Q

What are the four types of HL?

A

Nodular sclerosing

Lymphocyte rich

Mixed cellularity

Lymphocyte depleted

122
Q

What is the most common type of HL?

A

Nodular Sclerosing

123
Q

What two locations does nodular sclerosis often present?

A

cervical lymph node

mediastinal lymph node

124
Q

What sex does nodular sclerosis most often present? Age?

A

female

teenager

125
Q

Which type of Hodgkins Lymphoma has the best prognosis?

A

Lymphocyte rich

126
Q

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

A

IL5

eosinophil

127
Q

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

A

lymphocyte depleted

HIV+ pt’s and the elderly

128
Q

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

A

Plasma

bone marrow

129
Q

Which interleukin can drive Multiple Myeloma?

A

IL-6

130
Q

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

A

CRAB = multiple myeloma

elevated Calcium
real insufficiency
Anemia
bone lesions

131
Q

Neoplastic plasma cells of Multiple Myeloma can activate with receptor?

A

RANK

132
Q

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

A

skull and vertebrae

133
Q

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

A

IgA and IgG

134
Q

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

A

infection

neoplastic plasma cells only produce monoclonal Ab;s

135
Q

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

A

light chain

amyloidosis

136
Q

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

A

Multiple Myeloma

urine

Ig light chain

137
Q

What is the only similarity between Multiple Myeloma and MGUS?

A

M-spike

138
Q

What Ig isotype is produced during Waldenstroms Macroglobulinemia?

A

IgM

139
Q

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

A

vision and CNS

retinal damage and stroke

140
Q

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

A

increased

viscous serum results in defective aggregation

141
Q

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

A

monocytes

dendritic cells

142
Q

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

A

Birbeck granules

143
Q

What are the three immunohistochemical markers of Langerhans Cell Histiocytosis?

A

CD1a and S100 and CD207

144
Q

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

A

Langerhans cells

145
Q

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

A

Skin rash and cystic skeletal lesions

Infant

146
Q

What cell type proliferates during an Eosinophilic Granuloma? Where?

A

Langerhans

Bone

147
Q

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

A

Langerhans

148
Q

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

A

diabetes insipidus

lytic bone lesions

exopthalmos

149
Q

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

A

increases

causes neutrophils to be released from marginated pool (lungs)

150
Q

Basophilis is clasically associated with what disease?

A

CML

151
Q

What are the two main causes of lymphocytic leukocytosis?

A

Viral infection

Bordatella Pertussis Infection

152
Q

Why does splenomegaly occur during mononucleosis?

A

T-cell hyperplasia in PALS

153
Q

Infectious mononucleosis features what specific type of T-cell?

A

reactive CD8+ T-cell

154
Q

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

A

20%

155
Q

What two sites require ancillary chemotherapy during ALL? Why?

A

scrotum and CSF

BTB and BBB

156
Q

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

A

thymus

teenagers

157
Q

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

A

Acute Lymphoblastic Lymphoma

158
Q

How is acute monocytic leukemia differentiated from acute promyelocytic leukemia?

A

monocytic leukemia = no myeloproxidase

159
Q

Where does Acute Monoblastic Leukemia clasically infiltrate?

A

gums

160
Q

Do megakaryocytes have MPO?

A

no

161
Q

What are the three major complications of CLL?

A

Hypogammaglobulinemia

AI hemolytic anemia

Transformation into diffuse large B-cell lymphoma

162
Q

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

A

growing lymph node

163
Q

What are the three common features of Hairy Cell Leukemia?

A

Splenomegaly

Bone Marrow fibrosis (dry tap)

no lymphadenopathy

164
Q

What cause the splenomegaly in Hairy Cell Leukemia?

A

hairy cells accumulating in red pulp of spleen

165
Q

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

A

multiple myeloma

Human T-cell leukemia/lymphoma

166
Q

During a myeloproliferative disorder, which cells increase in number?

A

all cell types increase in number

167
Q

What is the definition of a myeloproliferative disorder?

A

neoplastic proliferation of mature cells of the myeloid lineage

168
Q

What is a common complication of all myeloproliferative disorders?

A

hyperuricemia

169
Q

What two states can a myeloproliferative disorder progress to?

A

marrow fibrosis

acute leukemia

170
Q

What is the accelerated phase of CML marked by?

A

splenomegaly

171
Q

What are the three physical symptoms of polycythemia vera?

A

blurry vision and headache

flushed face

itching after bathing

172
Q

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

A

mast cells

173
Q

What are the two treatments of polycythemia vera?

A
  1. Phlebotomy

2. Hydroxyurea

174
Q

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

A

Essential thrombocythemia

175
Q

Why does myelofibrosis result in extramedullary hematopoiesis?

A

no bone marrow for hematopoiesis

176
Q

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

A

reticulin

no

177
Q

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

A

Rheumatoid Arthritis

178
Q

What virus is known to cause follicular lymphadenopathy?

A

HIV

179
Q

Where is the lymph node do sinus histiocytes live?

A

medulla

180
Q

What is a lymphoma?

A

neoplastic proliferation of lymphoid cells that form a mass

181
Q

When does follicular lymphoma usually present?

A

late adulthood

182
Q

What does a follicular lymphoma look like histologically?

A

dozens of follicles

183
Q

What can a follicular lymphoma transform into?

A

Diffuse Large B-cell Lymphoma

184
Q

Where in the lymph node do the follicles live?

A

cortex

185
Q

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

A

lymphoma = follicles all over cell instead of just cortex

186
Q

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

A

hyperplasia due to infection

187
Q

Which is monoclonal, follicular lymphoma or follicular hyperplasia?

A

lymphoma

188
Q

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

A

3:1

189
Q

When does mantle cell lymphoma present?

A

late adult hood

190
Q

What condition produces a marginal zone of a lymph node?

A

chronic inflammation

191
Q

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

A

prominent nucleoli

192
Q

Why is a mass produced during Hodgkin lymphoma?

A

RS cells secrete cytokines that attract other cells

193
Q

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

A

RS secretion of cytokines

194
Q

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

A

Lacunar cell

195
Q

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

A

mixed-cellularity

eosinophil

196
Q

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

A

Monoclonal

197
Q

What causes a Rouleaux formation? What disease?

A

high protein in serum decreases charge between RBCs

Multiple Myeloma

198
Q

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

A

bence jones

Ig light chains

199
Q

What age group is MGUS most prevalent?

A

elderly

200
Q

What type of specific neoplasia is Waldenstroms Macroglobulinemia?

A

B-cell lymphoma

201
Q

Does Waldenstroms Macroglobulinemia present with lytic bone lesions?

A

no

202
Q

Does Waldenstroms Macroglobulinemia have an M-spike?

A

yes

203
Q

Why does Waldenstroms Macroglobulinemia present with CNS complications?

A

high IgM makes blood hyper-viscous

204
Q

What is the specific function of Langerhans cells?

A

present antigen to naive T-cells

205
Q

Is eosinophilic granuloma benign or pathological?

A

benign

206
Q

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

A

pathological bone fracture in adolescent

207
Q

What type of aggressive neoplasm can follicular lymphoma transform into?

A

diffuse large B-cell lymphoma

208
Q

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

A

Langerhans cell

209
Q

What is the classic presentation of Hand Schuler Christian Disease?

A

scalp rash and lytic bone defects

210
Q

Do high cortisol levels cause eosinophilia or eosinopenia?

A

eosinopenia

211
Q

Do megakaryocytes express MPO?

A

no

212
Q

Are B-cells in CLL naive or mature?

A

Naive

213
Q

Are myeloproliferative disorders an expansion of mature or immature cells?

A

mature

214
Q

Does an Eosinophilic Granuloma involve a skin rash?

A

no

215
Q

What virus can cause a nasopharyngeal carcinoma?

A

EBV

216
Q

Where does EBV maintain dormancy?

A

B-cells

217
Q

Which form of ALL usually presents in teenagers?

A

T-ALL

218
Q

What is the average age of development of AML?

A

50-60

219
Q

How can PV be distinguished from Essential Thrombocytosis?

A

PV runs a risk or hyperuricemia

ET does not

220
Q

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

A

Nodular Sclerosing

221
Q

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

A

mixed-cellularity

lymphocyte depleted

222
Q

How is Waldenstroms Treated?

A

plasmapheresis

223
Q

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

A

Eosiniphilic granuloma doesnt involve a rash

224
Q

Which cell is most often found in an Eosinophilic Granuloma?

A

Eosinophils

225
Q

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

A

20%

226
Q

What are the two translocations of ALL?

A

12: 21
9: 22

227
Q

Which translocation of ALL has a poor prognosis?

A

9;22

228
Q

Which translocation of ALL has a good prognosis?

A

12:21

229
Q

What is a Richter Transformation?

A

CLL into large B-cell lymphoma

230
Q

Is lymphocytic leukocytosis caused by viruses or bacteria?

A

virus

231
Q

Which two leukemias can CML transform into?

A

AML or ALL

232
Q

Where is the mantle located in the lymph node?

A

immediately adjacent to the follicle

Pathoma (19 decks)

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