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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
Mechanism and lab values seen in Bernard-Soulier syndrome
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.
26
Mechanism and lab values seen in Glanzmann's Thrombasthenia
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.
27
Mechanism and lab values seen in Idiopathic Thrombocytopenic Purpura (ITP)
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.
28
Mechanism and lab values seen in Thrombotic Thrombocytopenia Purpura (TTP) --SX associated (hint: mneumonic)
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
29
Mechanism, triad of sx and most common cause of HUS
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.
30
2 locations of vWF synthesis
``` megakaryocytes (alpha granules inside platelets) endothelial cells (Weibel-Palade bodies) ```
31
Pathogenesis of von Willebrand's disease
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.
32
Dx of von Willebrand's disease uses ...
Ristocetin cofactor assay
33
Lifespan of a platelet
8-10 days
34
Pathogenesis of DIC
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.
35
4 neoplasms associated with AIDS
Kaposi sarcoma Invasive SCC CNS lymphoma NHL
36
4 diseases associated with Marginal Cell Lymphoma
Hashimoto Thyroiditis Sjogren disease (salivary gland) Stomach in H. pylori - gastritis GI tract in MALToma
37
Most common lymphoma in US
Diffuse large B cell lymphoma
38
2 lymphomas associated with EBV
Burkitt lymphoma | Hodgkin lymphoma
39
Lymphoma equivalent of CLL
Small lymphocytic lymphoma
40
STarry sky pattern due to phagocyotsis of apoptotic tumor cell
Burkitt lymphoma
41
Age distribution of Hodgkin's vs. Non-Hodgkin's lymphoma
Hodgkin's: bi-modal (under 20 or over 65) | NHL: widely variable
42
Most common type of NHL in adults; NHL in children
Adults: diffuse large B cell Children: lymphoblastic lymphoma
43
Epidemiology of ALL
White male children
44
Leukemia associated with the enzyme terminal deoxynucleotide transferase (TdT)
ALL
45
Leukemia that is PAS +
ALL
46
Characteristic auer rods
AML (M2, M3)
47
AML that are CD13 and CD33+
M0-M6 AML
48
AML associated with Down syndrome
M7 AML
49
Myelodysplastic and myeloproliferative d/o may both progress to
AML
50
Acute leukemia vs chronic leukemia blast count
Acute: >20% Chronic: <5%
51
Numerous basophils, splenomegaly, and negative for leukocyte alkaline phosphatase
CML
52
Always positive for philadelphia chromosome t9;22
CML
53
Only AML that is CD13 and CD33-
M7 AML
54
Solid sheets of lymphoblasts in marrow
ALL
55
Acute leukemia positive for peroxidase
AML
56
PAS - acute leukemia
AML
57
Always associated iwth the BCR-ABL genes
CML
58
Commonly presents with bone pain
ALL
59
Smudge cells and autoimmune hemolytic anemia
CLL
60
6 associated findings in Multiple Myeloma
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
Histological appearance of Multiple Myeloma
Numerous plasma cells with "clock face" chromatin and intracytoplasmic inclusions containg immunoglobulin
62
How to distinguish Multiple Myeloma from Waldenstrom's macroglobulinemia
M Spike is due to IgM, not IgG/IgA (seen in MM) | No lytic bone lesions
63
MGUS is a monoclonal expansion of?
Plasma cells M spike -- high levels of IgM in circulation Patients develop MM at a rate of 1-2% per year
64
Genetics of CML
t9;22 | bcr-abl fusion
65
Genetics of Burkitt's lymphoma
t8;14 causes c-myc activation
66
Genetics of Mantle cell lymphoma
t11;14 | cyclin D1 activation -->G1/S transition
67
Genetics of Follicular lymphoma
t14;18 | bcl-2 activation
68
Genetics of M3 type of AML
t15;17
69
Common presentation seen in M3 subtype of AML
DIC Also note that this subtype responds to all-trans retinoic acid (vitamin A) as this induces differentiation of myeloblasts
70
Risk factors for esophageal cancer
``` ABCDEFGH Achalasia/alcohol Barrett's esophagus Corrosive esophagitis/cigarettes Diverticuli Esophageal web Familial GERD Hotdogs (nitrosamine) ```
71
2 neoplasms associated with Down syndrome
ALL, AML (M7)
72
Ash-leaf spots, adenoma sabaceous, seizures, MR --associated with what 3 neoplasms?
Tuberous sclerosis Assoc with giant cell astrocytoma Renal angiomyolipoma Cardiac rhabdomyoma
73
Paget's disease of bone is assoc with what 2 neoplasms
Secondary osteosarcoma | Fibrosarcoma
74
Acanthosis nigricans is associated with what type of neoplasm?
Visceral malignancies (stomach, lung, uterus, breast)
75
3 neoplasms associated with radiation exposure
Leukemia | Papillary Thyroid Cancer
76
Sjogren's syndrome is associated with what type of neoplasm?
B cell lymphoma
77
Oncogene associated with CML and has what kind of gene product?
abl | Tyrosine kinase
78
Oncogene associated with Burkitt's lymphoma and what gene product?
c-myc | Transcription factor
79
Oncogene associated iwth follicular and undifferentiated lymphomas and has what kind of gene product?
bcl-2 | Anti-apoptotic molecule
80
Oncogene associated with colon CA and has what kind of gene product
ras | GTPase
81
Oncogene associated with lung tumors and has what kind of gene product?
L-myc | Transcription factor
82
Oncogene associated with neuroblastoma and has waht kind of gene product?
N-myc
83
Oncogene associated with MEN 2A and 2B and has what kind of gene product?
ret | Tyrosine kinase
84
Oncogene associated with gastrointestinal stromal tumors and has what kind of gene product?
c-kit | Cytokine receptor
85
In order for an oncogene to cause cancer, you must damage..?
only 1 allele | Gain of function --> increased cancer risk
86
In order for a tumor suppressor gene to cause cancer, you must damage...?
Both alleles | Loss of function --> increased cancer risk
87
Tumor suppressor gene associated with retinoblastoma and osteosarcoma and what is its gene product?
Rb | Inhibits E2F therefore blocks G1-->S phase
88
p53 is the tumor suppressor gene that acts as the transcription factor for?
p21 | Blocks G1--> S phase
89
BRCA1/BRCA2 gene mutations result in damage to what kind of proteins?
DNA repair proteins
90
2 tumor suppressor genes associated with melanoma
p16 | BRAF
91
Tumor suppressor gene associated with NF type I and its gene product
NF1 | RAS GTPase activating protein (RAS-GAP)
92
Tumor suppressor gene associated with NF Type 2 and its gene product
NF2 | Merlin (schwannomin) protein
93
Which type of neurofibromatosis is associated with bilateral acoustic schwannomas?
NF-2
94
Tumor suppressor gene associated with pancreatic cancer
DPC4
95
Tumor suppressor gene associated with colon cancer
DCC
96
Patient eats a ton of corn and comes in with RUQ pain. Waht is it?
Hepatocellular CA from aflatoxins which are from Aspergillus and cause a mutation in p53
97
Vinyl chloride is associated with what neoplasm?
Angiosarcoma of liver
98
Carbon tetrachloride is associated with what disease process?
Centrilobular necrosis and fatty change of liver
99
Smoked foods are associated with what type of neoplasm?
Nitrosamines --> gastric cancer
100
3 neoplasms (other than laryngeal and lung) that can be caused by smoking
Renal cell CA Transitional cell CA of bladder Pancreatic adenocarcinoma
101
2 neoplasms associated with asbestos exposure
Bronchogenic CA | Mesothelioma
102
3 organs affected by arsenic exposure
Skin (SCC) Liver (angiosarcoma) Lung
103
Moth balls are associated with what neoplasm?
These contain naphthalene (aniline) dyes | Transitional cell CA of bladder
104
K-RAS mutation is associated with which 3 types off tumors?
Colon Lung Pancreatic
105
H-RAS mutation associated with which 2 types of tumors?
Bladder | Kidney
106
N-RAS mutations associated with which 2 types of neoplasms?
Melanomas | Hematologic malignancies
107
Describe the MOA of p-53.
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
NF-1 is located on what chromosome?
17
109
NF-2 is located on what chromosome?
22
110
Some guy ingested a ton of batteries. What 2 types of neoplasms is he at higher risk of?
Cadmium is associated with prostate and lung cancer.
111
Patient worked for NASA his whole life and now presents with a neoplasm. Which kind does he most likely have?
Lung cancer d/t beryllium.
112
Chromium compounds put you at increased risk for developing what kind of cancer?
Lung cancer
113
Nickel sulfide increases your risk for what 2 types of cancers?
Lung | Upper airway
114
What are the most common cancers associated with ionizing radiation?
``` Myeloid leukemias (AML, CML) Thyroid cancer in young ```
115
Hepatitis C is associated with what neoplasm?
Papillary thyroid CA
116
Ataxia-Telangiectasias are associated with which 2 neoplasms?
Leukemias | Lymphomas
117
Unique enzyme normally absent in somatic cells but active in stem cells and cancer cells?
Telomerase
118
2 proangiogenic cytokines
Basic fibroblast growth factor (bFGF) | Vascular endothelial growth factor (VEGF)
119
CEA tumor marker associated with
Colroectal and pancreatic cancers Also produced by gastric, breast, and medullary thyroid CAs. CarcinoEmbryogenic Antigen Non-specific
120
alpha-fetoprotein tumor marker associated with
Hepatocellular CA | Non-seminomatous germ cell tumors of testis and ovary (e.g. endodermal sinus)
121
CA-125 tumor marker associated with
Ovarian cancer
122
3 cancers that S-100 tumor marker is associated with
Melanoma Neural tumors Schwannomas
123
3 cancers Alkaline phosphatase tumor marker is associated with
Mets to bone Mets to liver Paget's disease of bone
124
Bombesin tumor marker is associated with what 3 cancers?
Neuroblastoma Lung cancer Gastric cancer
125
Cancer associated with TRAP tumor marker
Tartrate-Resistant-Acid Phosphatase | Hairy cell leukemia -- TRAP the hairy animal!
126
CA-19-9 tumor marker associated with
Pancreatic adenocarcinoma
127
4 cancers associated with the paraneoplastic syndrome affecting erythropoietin (causes polycythemia)
Renal cell CA Hemangioblastoma Hepatocellular CA Pheochromocytoma
128
6 cancers that metastasize to bone
``` Prostate Thyroid Testes Breast Lung Kidney ```
129
5 cancers that metastasize to the brain
``` Lots of Bad Stuff Kills Glia Lung Breast Skin (melanoma) Kidney (renal cell CA) GI tract ```
130
5 cancers that metastasize to the liver
``` Cancer Sometimes Penetrates Benign Liver Colon Stomach Pancreas Breast Lung ```
131
6 cancers that may cause hypercalcemia
``` Squamous cell lung CA Squamous cell CA of head and neck Multiple myeloma Breast cancer Renal cell CA Metastatic disease to bones ```
132
"Rings on a tree" buzzword associated with?
Classic description of psammoma bodies (laminated, concentric and calcific spherules) -- PSaMMoma Papillary adenocarcinoma of thyroid Serous papillary cystadenocarcinoma of ovary Meningioma Malignant mesothelioma

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