HEME/ONC Flashcards

1
Q

Lab value used to monitor Heparin

A

PTT

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2
Q

Lab value used to monitor Warfarin

A

PT/INR

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3
Q

Lab value used to monitor Enoxaparin

A

Factor Xa activity

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4
Q

Test to dx beta-thalassemia minor

A

Hemoglobin electrophoresis – would find increased HbA2

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5
Q

Should be ruled out in male over 50 with new onset iron deficiency anemia

A

Colon cancer

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6
Q

8 causes of aplastic anemia

A
Radiation*
Benzene
Chloramphenicol
Alkylating agents
Anti-metabolites
Viruses (e.g. parvo B19, EBV, HIV, HCV)
Fanconi's anemia
Immune-related
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7
Q

Main pathogenesis of aplastic anemia as well as distinguishing bone marrow findings.

A

Failure or destruction of myeloid stem cells – usually due to radiation.
Pancytopenia characterized by severe anemia, leukopenia, and thrombocytopenia. Normal cell morphology but hypocellular bone marrow with fatty infiltration (dry bone marrow tap).

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8
Q

5 etiologies of B12 deficiency

A
Insufficient intake (e.g. strict vegans)
Malabsorption (e.g. Crohn's disease)
Pernicious anemia 
Diphyllobothrium latum (fish tapeworm)
PPIs
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9
Q

3 drugs that can cause folate deficiency

A

Methotrexate
Trimethoprim
Phenytoin

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10
Q

Lab findings in iron deficiency anemia

A

Decreased serum iron (PRIMARY)
Increased transferrin/TIBC
Decreased ferritin (iron stores depleted)
REALLY decreased % transferrin saturation (serum iron/TIBC)

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11
Q

Lab findings in anemia of chronic disease

A

Decreased serum iron
Decreased transferrin/TIBC because the body has adapted a system in which iron is stored within the cells of the body and prevents pathogens from acquiring circulating iron.
Increased ferritin (primary)

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12
Q

Lab findings in hemochromatosis

A

Increased serum iron (primary)
Decreased transferrin/TIBC
Increased ferritin
REALLY increased % transferrin saturation (serum iron/TIBC)

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13
Q

Lab findings in pregnancy/OCP use

A

Increased transferrin/TIBC (primary)

Decreased % transferrin saturation (serum iron/TIBC)

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14
Q

Findings in Hereditary Spherocytosis

A
Anemia
Jaundice
Pigmented gallstones*
Splenomegaly
\+ Osmotic fragility
- Coomb's (Abs are not causing hemolysis)
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15
Q

Difference between HbS defect and HbC defect

A

HbS: subsitution of valine for glutamic acid at position 6 on beta globin
HbC: substitution of lysine for glutamic acid

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16
Q

Difference between Direct Coomb’s and Indirect Coomb’s

A

The Coombs test evaluates for RBC agglutination with the addition of antihuman antibody because RBCs are coated with Ig or complement proteins.
In a Direct Coomb’s, prepared Abs are added to a patient’s washed RBC to detect the presence of immunoglobulins already present on the RBC (using an Ab to detect an Ab).
In an Indirect Coomb’s, the patient’s serum is incubate with normal RBC to detect for the presence of Abs.

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17
Q

3 situations with a positive Direct Coomb’s

A

Hemolytic disease of the newborn
Drug-induced autoimmune hemolytic anemia
Hemolytic transfusion reactions

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18
Q

2 situations which use an Indirect Coomb’s

A

Tests blood prior to transfusion
Screening for maternal Abs to a fetus’ blood

It’s positive when there are Abs present to foreign blood.

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19
Q

Pathogenesis of Cold Agglutinins and 3 infections associated

A

Antibodies against RBCs that interact more strongly at low temps than at body temp.
Nearly always IgM.
Occur regularly in infections with Mycoplasma pneumonia, EBV, malignancies (CLL)
Problems/disease occurs when there is circulation to a cold extremity –> IgM binds RBC antigen –> complement fixation –> MAC lysis, opsonization –> phagocytosis

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20
Q

Pathogenesis of Warm Agglutinins and 4 disease associations

A

Antibodies that react against RBC protein Ags at body temperature.
Nearly always IgG.
Seen in viruses (EBV, HIV), SLE, malignancies (CLL), and congenital immune abnormalities.

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21
Q

2 protozoal diseases that can cause hemolytic anemia

A

Malaria

Babesiosis

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22
Q

4 diseases that cause basophilic stippling

A

Thalassemia
Anemia of chronic disease
Iron deficiency
Lead poisoning*

TAIL

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23
Q

4 conditions associated with target cells

A
HALT says the hunter to its TARGET.
HbC disease
Asplenia
Liver disease
Thalassemias
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24
Q

Anemia resulting from mechanical destruction of erythrocytes due to aortic stenosis or prosthetic heart valves

A

Macroangiopathic anemia

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25
Q

Mechanism and lab values seen in Bernard-Soulier syndrome

A

Defect in platelet plug formation due to decreased GpIb, causing a defect in platelet-to-vWF adhesion.

Platelet count is decreased, bleeding time is increased.

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26
Q

Mechanism and lab values seen in Glanzmann’s Thrombasthenia

A

Defect in platelet plug formation due to decreased GpIIb/IIIa, causing a defect in platelet-to-platelet aggregation.
On labs, there is no platelet clumping on blood smear.

This causes increased bleeding time with no change in platelet count.

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27
Q

Mechanism and lab values seen in Idiopathic Thrombocytopenic Purpura (ITP)

A

Anti-GpIIb/IIIa Abs attached to platelets cause the splenic macrophages to sequester and destroy these complexes. This obviously decreases platelet survival, causing a decrease in platelet count and an increase in bleeding time.

Labs show increased megakaryocytes.

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28
Q

Mechanism and lab values seen in Thrombotic Thrombocytopenia Purpura (TTP) –SX associated (hint: mneumonic)

A

A deficiency of ADAMTS 13 (vWF metalloprotease) causes decreased degradation of vWF multimers. These huge multimers cause increased platelet aggregation and thrombosis, causing decreased platelet survival with schistocytes and increased LDH present.

SX are HUS + neurologic sx and fever.
FAT RN = Fever, Anemia (hemolytic), Thrombocytopenia, Renal failure (uremia), Neurologic sx

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29
Q

Mechanism, triad of sx and most common cause of HUS

A

Hemolytic anemia + thrombocytopenia + renal failure
Preceded by E. coli 0:157 H:7
Endothelial cells are damaged predomianntly in the kidney causing paltelets to aggregate, forming microthrombi. This causes renal injury leading to uremia.
The clot formation consumes platelets, causing thrombocytopenia.
The thrombi shear RBCs causing schistocytes and anemia.

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30
Q

2 locations of vWF synthesis

A
megakaryocytes (alpha granules inside platelets) 
endothelial cells (Weibel-Palade bodies)
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31
Q

Pathogenesis of von Willebrand’s disease

A

Qualitative or quantitative deficiency in vWF. In its absence, stability of factor VIII is lost causing an intrinsic pathway coagulation defect and increasing PTT. vWF is also involved in platelet adhesion to vessel wall and other platelets. Thus, its deficiency leads to an increased bleeding time due to the defect in platelet-to-vWF adhesion.

AUTOSOMAL DOMINANT.

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32
Q

Dx of von Willebrand’s disease uses …

A

Ristocetin cofactor assay

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33
Q

Lifespan of a platelet

A

8-10 days

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34
Q

Pathogenesis of DIC

A

Pathological activation of coagulation mechanisms in response to a variety of diseases. DIC leads to the formation of small blood clots inside the blood vessels throughout the body. As the small clots consume coagulation proteins and platelets, normal coagulation is disrupted and abnormal bleeding occurs from the skin (e.g. from sites where blood samples were taken), the GI tract, the respiratory tract and surgical wounds. The small clots also disrupt normal blood flow to organs (such as the kidneys), which may malfunction as a result.

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35
Q

4 neoplasms associated with AIDS

A

Kaposi sarcoma
Invasive SCC
CNS lymphoma
NHL

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36
Q

4 diseases associated with Marginal Cell Lymphoma

A

Hashimoto Thyroiditis
Sjogren disease (salivary gland)
Stomach in H. pylori - gastritis
GI tract in MALToma

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37
Q

Most common lymphoma in US

A

Diffuse large B cell lymphoma

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38
Q

2 lymphomas associated with EBV

A

Burkitt lymphoma

Hodgkin lymphoma

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39
Q

Lymphoma equivalent of CLL

A

Small lymphocytic lymphoma

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40
Q

STarry sky pattern due to phagocyotsis of apoptotic tumor cell

A

Burkitt lymphoma

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41
Q

Age distribution of Hodgkin’s vs. Non-Hodgkin’s lymphoma

A

Hodgkin’s: bi-modal (under 20 or over 65)

NHL: widely variable

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42
Q

Most common type of NHL in adults; NHL in children

A

Adults: diffuse large B cell
Children: lymphoblastic lymphoma

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43
Q

Epidemiology of ALL

A

White male children

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44
Q

Leukemia associated with the enzyme terminal deoxynucleotide transferase (TdT)

A

ALL

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45
Q

Leukemia that is PAS +

A

ALL

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46
Q

Characteristic auer rods

A

AML (M2, M3)

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47
Q

AML that are CD13 and CD33+

A

M0-M6 AML

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48
Q

AML associated with Down syndrome

A

M7 AML

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49
Q

Myelodysplastic and myeloproliferative d/o may both progress to

A

AML

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50
Q

Acute leukemia vs chronic leukemia blast count

A

Acute: >20%
Chronic: <5%

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51
Q

Numerous basophils, splenomegaly, and negative for leukocyte alkaline phosphatase

A

CML

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52
Q

Always positive for philadelphia chromosome t9;22

A

CML

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53
Q

Only AML that is CD13 and CD33-

A

M7 AML

54
Q

Solid sheets of lymphoblasts in marrow

A

ALL

55
Q

Acute leukemia positive for peroxidase

A

AML

56
Q

PAS - acute leukemia

A

AML

57
Q

Always associated iwth the BCR-ABL genes

A

CML

58
Q

Commonly presents with bone pain

A

ALL

59
Q

Smudge cells and autoimmune hemolytic anemia

A

CLL

60
Q

6 associated findings in Multiple Myeloma

A

Increased susceptibility to infection due to impaired Ab synthesis
Primary amyloidosis (AL)
Punched out lytic bone lesions on x-ray
M-spike on protein electrophoresis
Ig light chains in urine (Bence Jones protein)
Rouleaux formation (RBCs look like poker chips)

61
Q

Histological appearance of Multiple Myeloma

A

Numerous plasma cells with “clock face” chromatin and intracytoplasmic inclusions containg immunoglobulin

62
Q

How to distinguish Multiple Myeloma from Waldenstrom’s macroglobulinemia

A

M Spike is due to IgM, not IgG/IgA (seen in MM)

No lytic bone lesions

63
Q

MGUS is a monoclonal expansion of?

A

Plasma cells
M spike – high levels of IgM in circulation
Patients develop MM at a rate of 1-2% per year

64
Q

Genetics of CML

A

t9;22

bcr-abl fusion

65
Q

Genetics of Burkitt’s lymphoma

A

t8;14 causes c-myc activation

66
Q

Genetics of Mantle cell lymphoma

A

t11;14

cyclin D1 activation –>G1/S transition

67
Q

Genetics of Follicular lymphoma

A

t14;18

bcl-2 activation

68
Q

Genetics of M3 type of AML

A

t15;17

69
Q

Common presentation seen in M3 subtype of AML

A

DIC
Also note that this subtype responds to all-trans retinoic acid (vitamin A) as this induces differentiation of myeloblasts

70
Q

Risk factors for esophageal cancer

A
ABCDEFGH
Achalasia/alcohol
Barrett's esophagus
Corrosive esophagitis/cigarettes
Diverticuli
Esophageal web
Familial
GERD
Hotdogs (nitrosamine)
71
Q

2 neoplasms associated with Down syndrome

A

ALL, AML (M7)

72
Q

Ash-leaf spots, adenoma sabaceous, seizures, MR –associated with what 3 neoplasms?

A

Tuberous sclerosis
Assoc with giant cell astrocytoma
Renal angiomyolipoma
Cardiac rhabdomyoma

73
Q

Paget’s disease of bone is assoc with what 2 neoplasms

A

Secondary osteosarcoma

Fibrosarcoma

74
Q

Acanthosis nigricans is associated with what type of neoplasm?

A

Visceral malignancies (stomach, lung, uterus, breast)

75
Q

3 neoplasms associated with radiation exposure

A

Leukemia

Papillary Thyroid Cancer

76
Q

Sjogren’s syndrome is associated with what type of neoplasm?

A

B cell lymphoma

77
Q

Oncogene associated with CML and has what kind of gene product?

A

abl

Tyrosine kinase

78
Q

Oncogene associated with Burkitt’s lymphoma and what gene product?

A

c-myc

Transcription factor

79
Q

Oncogene associated iwth follicular and undifferentiated lymphomas and has what kind of gene product?

A

bcl-2

Anti-apoptotic molecule

80
Q

Oncogene associated with colon CA and has what kind of gene product

A

ras

GTPase

81
Q

Oncogene associated with lung tumors and has what kind of gene product?

A

L-myc

Transcription factor

82
Q

Oncogene associated with neuroblastoma and has waht kind of gene product?

A

N-myc

83
Q

Oncogene associated with MEN 2A and 2B and has what kind of gene product?

A

ret

Tyrosine kinase

84
Q

Oncogene associated with gastrointestinal stromal tumors and has what kind of gene product?

A

c-kit

Cytokine receptor

85
Q

In order for an oncogene to cause cancer, you must damage..?

A

only 1 allele

Gain of function –> increased cancer risk

86
Q

In order for a tumor suppressor gene to cause cancer, you must damage…?

A

Both alleles

Loss of function –> increased cancer risk

87
Q

Tumor suppressor gene associated with retinoblastoma and osteosarcoma and what is its gene product?

A

Rb

Inhibits E2F therefore blocks G1–>S phase

88
Q

p53 is the tumor suppressor gene that acts as the transcription factor for?

A

p21

Blocks G1–> S phase

89
Q

BRCA1/BRCA2 gene mutations result in damage to what kind of proteins?

A

DNA repair proteins

90
Q

2 tumor suppressor genes associated with melanoma

A

p16

BRAF

91
Q

Tumor suppressor gene associated with NF type I and its gene product

A

NF1

RAS GTPase activating protein (RAS-GAP)

92
Q

Tumor suppressor gene associated with NF Type 2 and its gene product

A

NF2

Merlin (schwannomin) protein

93
Q

Which type of neurofibromatosis is associated with bilateral acoustic schwannomas?

A

NF-2

94
Q

Tumor suppressor gene associated with pancreatic cancer

A

DPC4

95
Q

Tumor suppressor gene associated with colon cancer

A

DCC

96
Q

Patient eats a ton of corn and comes in with RUQ pain. Waht is it?

A

Hepatocellular CA from aflatoxins which are from Aspergillus and cause a mutation in p53

97
Q

Vinyl chloride is associated with what neoplasm?

A

Angiosarcoma of liver

98
Q

Carbon tetrachloride is associated with what disease process?

A

Centrilobular necrosis and fatty change of liver

99
Q

Smoked foods are associated with what type of neoplasm?

A

Nitrosamines –> gastric cancer

100
Q

3 neoplasms (other than laryngeal and lung) that can be caused by smoking

A

Renal cell CA
Transitional cell CA of bladder
Pancreatic adenocarcinoma

101
Q

2 neoplasms associated with asbestos exposure

A

Bronchogenic CA

Mesothelioma

102
Q

3 organs affected by arsenic exposure

A

Skin (SCC)
Liver (angiosarcoma)
Lung

103
Q

Moth balls are associated with what neoplasm?

A

These contain naphthalene (aniline) dyes

Transitional cell CA of bladder

104
Q

K-RAS mutation is associated with which 3 types off tumors?

A

Colon
Lung
Pancreatic

105
Q

H-RAS mutation associated with which 2 types of tumors?

A

Bladder

Kidney

106
Q

N-RAS mutations associated with which 2 types of neoplasms?

A

Melanomas

Hematologic malignancies

107
Q

Describe the MOA of p-53.

A

p53 acts through p21 to cause cell cycle arrest and is involved at the G1/S checkpoint and G2/M checkpoint. It causes apoptosis by inducing the transcription of pro-apoptotic genes such as BAX. Thus, mutations in this gene allow the cell to progress through the checkpoint despite the presence of DNA damage/mutations.

108
Q

NF-1 is located on what chromosome?

A

17

109
Q

NF-2 is located on what chromosome?

A

22

110
Q

Some guy ingested a ton of batteries. What 2 types of neoplasms is he at higher risk of?

A

Cadmium is associated with prostate and lung cancer.

111
Q

Patient worked for NASA his whole life and now presents with a neoplasm. Which kind does he most likely have?

A

Lung cancer d/t beryllium.

112
Q

Chromium compounds put you at increased risk for developing what kind of cancer?

A

Lung cancer

113
Q

Nickel sulfide increases your risk for what 2 types of cancers?

A

Lung

Upper airway

114
Q

What are the most common cancers associated with ionizing radiation?

A
Myeloid leukemias (AML, CML)
Thyroid cancer in young
115
Q

Hepatitis C is associated with what neoplasm?

A

Papillary thyroid CA

116
Q

Ataxia-Telangiectasias are associated with which 2 neoplasms?

A

Leukemias

Lymphomas

117
Q

Unique enzyme normally absent in somatic cells but active in stem cells and cancer cells?

A

Telomerase

118
Q

2 proangiogenic cytokines

A

Basic fibroblast growth factor (bFGF)

Vascular endothelial growth factor (VEGF)

119
Q

CEA tumor marker associated with

A

Colroectal and pancreatic cancers
Also produced by gastric, breast, and medullary thyroid CAs.

CarcinoEmbryogenic Antigen
Non-specific

120
Q

alpha-fetoprotein tumor marker associated with

A

Hepatocellular CA

Non-seminomatous germ cell tumors of testis and ovary (e.g. endodermal sinus)

121
Q

CA-125 tumor marker associated with

A

Ovarian cancer

122
Q

3 cancers that S-100 tumor marker is associated with

A

Melanoma
Neural tumors
Schwannomas

123
Q

3 cancers Alkaline phosphatase tumor marker is associated with

A

Mets to bone
Mets to liver
Paget’s disease of bone

124
Q

Bombesin tumor marker is associated with what 3 cancers?

A

Neuroblastoma
Lung cancer
Gastric cancer

125
Q

Cancer associated with TRAP tumor marker

A

Tartrate-Resistant-Acid Phosphatase

Hairy cell leukemia – TRAP the hairy animal!

126
Q

CA-19-9 tumor marker associated with

A

Pancreatic adenocarcinoma

127
Q

4 cancers associated with the paraneoplastic syndrome affecting erythropoietin (causes polycythemia)

A

Renal cell CA
Hemangioblastoma
Hepatocellular CA
Pheochromocytoma

128
Q

6 cancers that metastasize to bone

A
Prostate
Thyroid
Testes
Breast
Lung
Kidney
129
Q

5 cancers that metastasize to the brain

A
Lots of Bad Stuff Kills Glia
Lung
Breast
Skin (melanoma)
Kidney (renal cell CA)
GI tract
130
Q

5 cancers that metastasize to the liver

A
Cancer Sometimes Penetrates Benign Liver
Colon
Stomach
Pancreas
Breast
Lung
131
Q

6 cancers that may cause hypercalcemia

A
Squamous cell lung CA
Squamous cell CA of head and neck
Multiple myeloma
Breast cancer
Renal cell CA
Metastatic disease to bones
132
Q

“Rings on a tree” buzzword associated with?

A

Classic description of psammoma bodies (laminated, concentric and calcific spherules) – PSaMMoma

Papillary adenocarcinoma of thyroid
Serous papillary cystadenocarcinoma of ovary
Meningioma
Malignant mesothelioma