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Hem/ Onc Pathology High Yield Flashcards

1
Q

Acanthocyte/ “spur cells” are seen in…

A

abetalipoproteinemia and liver disease

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2
Q

Basophilic stippling of RBCs are seen in…

A

Lead poisoning

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3
Q

What causes ringed sideroblasts?

A

excess Fe in mitochondira (sideroblastic anemia)

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4
Q

What can induce cycling?

A

dehydration, deoxygenation, high altitudes

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5
Q

When are dacrocyte (tear drop cells) seen?

A

bone marrow infiltration

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6
Q

What are the 4 pathologies associated with target cells?

A

HbC disease
Asplenia
Liver disease
Thalassemia

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7
Q

What causes heinz bodies?

A

Oxidation of Hb SH gropus to S—S

Leads to Hb precipitation

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8
Q

What pathology is associated with heinz bodies?

A

G6pD deficiency

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9
Q

What pathology is associated with heinz bodies?

A

G6pD deficiency

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10
Q

How can you differentiate between hemolytic normocytic anemia and non hemolytic normocytic anemia?

A

reticulocyte count

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11
Q

Transporter for Fe in the small intestine?

A

DMT1 transporter

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12
Q

Fe storage protein?

A

ferritin

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13
Q

“Fe deficiency anemia, esophageal webs, atrophic glossitis”

A

Plummer Vinson Syndrome

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14
Q

Is alpha thalassemia a mutation or a deletion?

A

It is a deletion of alpha globin genes

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15
Q

HbH disease?

A

2 allele deletion of alpha globin gene…leading to very little alpha globin and beta chain tetromers (HbH)

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16
Q

Is beta thalassemia a mutation or a deletion?

A

It is a mutation in splice site and romotor sequences

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17
Q

Difference in presentation beta thalassemia major and minor?

A

Minor: usually asymptomatic, increased HbA2

Major: Beta chain is absent, severe, extramedulary hematopoesis

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18
Q

What are the 2 enzymes affected by lead poisoning?

A

ALA dehydrastase and ferrochetalase

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19
Q

Treatment for lead poisoning?

A

Dimercaprol, EDTA, succimer

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20
Q

What enzyme is deficiency in sideroblastic anemia? Inheritance pattern?

A

ALA synthase

X linked

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21
Q

What cofactor does ALA synthase require?

A

Pyridoxine (B6)

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22
Q

Ferretin and TIBC levels in sideroblastic anemia?

A

decreased TIBC and increased ferritin

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23
Q

What type of anemia is homocysteine AND methylamalonic acid increased in?

A

B12 deficiency

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24
Q

Which has neurological symptoms…folate deficiency anemia or B12 deficiecy anemia

A

B12 deficiency anemia

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25
What causes the neurological symptoms in B12 deficiency anemia?
the incrreased methymalonic acid!
26
Where is folate absorbed?
Jejunum
27
Where is folate absorbed?
Jejunum
28
Enzyme defective in orotic aciduria?
UMP synthase (de novo pyrimidine synthesis pathway
29
How do you differentiate ornithine transcarbamylase deficiency and orotic aciduria?
Both have increased orotic acid but only OTC deficiency has hyperammonemia
30
What is hepcidin?
released by liver to bind ferroportin on intestinal mucosal cells and macrophages to inhibit Fe transport Upregulated in anemia of chronic disease
31
What is the treatment for anemia of chronic disease?
EPO
32
What is the treatment for hereditary spherocytosis?
Splenectomy
33
What 3 proteins can be mutated in hereditary spherocytosis?
Ankyrin Band 3 4.2 spectrin
34
Which anemia is osmotic fragility test (+)?
Hereditary spherocytosis
35
Most commonenzymatic disorder of RBCs?
G6PD deficiency (X linked)
36
G6PD deficiecny leads to a decrease in what supstance?
decreased glutathione
37
"rigid red blood cells due to decreaesd ATP"
Pyruvate kinase deficency
38
Glu---> lys? in beta globin gene?
HbC defect
39
"complement mediated RBC lysis due to impaired synthesis for decay accelerating factor that pretects RBC membrane from complement"
Paroxysmal nocturnal hemoglobinuria
40
Coomb (-) hemolytic anemia?
Paroxysmal nocturnal hemoglobinuria
41
What cell markers are missing on the RBCs of patients with paroxysmal nocturnal hemoglobinuria?
CD 55/59
42
Glu--> val substitution on beta chain?
sickle cell anemia
43
Why are newborns with HbS and beta thalassemia usually asymptomatic?
because fetal Hb is around until 6mo old
44
What drug can increase HbF?
hydroxyurea
45
Warm agglutinin is IgG or IgM? What about cold agglutinin?
IgG- Warm IgM- Cold
46
Which anemia is coombs (+)?
Autoimmune hemplytc anemia
47
What type of anemia do mechanical heart valves lead to?
macroangiopathic anemia
48
What type of anemia do mechanical heart valves lead to?
macroangiopathic anemia
49
What enzyme is deficiency in acute intermittent porphyria?
Porphobilinogen deaminase
50
"painful abdomen, port wine colored urine, polyneuropathy, psychological disturbances, precipitated by drugs (P450 inducers)"
Acute intermittent porphyria
51
Enzyme defective in porphyria cutaea tarda?
Uroporphyrinogen decarboxylase?
52
Most common porphyria?
porphyria cutanea tarda
53
What cell damage results in Fe poisoning?
peroxidation of membrane lipids
54
What is increased in hemophelia...PT or PTT?
PTT
55
"decrease in GpIb leading to defective platelet adhesion"
Bernard- Soulier Syndrome
56
"decrease in GpIIb/ IIIa leading to defective platelet aggregation"
Glanzman thrombasthenia
57
"anti- GpIIb/IIIa antibodies commonly due to viral illness"
Immune thrombocytopenia
58
"inhibition/ deficiency of ADAMTS 12 leading to decreased degredation of vWF multimers--> thrombosis"
TTP
59
"Most common inherited coagulation disorder"
von Willebrand disease
60
What is the inheritance pattern of vonWillebrand disease?
Autosomal Dominent
61
"no increase in PTT following heparin administration"
antithrombin deficiency
62
Most common cause of inherited hypercoagulability in whites?
Factor V leiden
63
"increased risk of thrombotic skin necoriss with hemorrhage following administration of warfarin"
Protein C or S deficiecny
64
What are 2 ways to differentiate leukemoid reaction from leukemia/ lymphoma?
Increased LAP and Left shift
65
Which has "B" signs (constitutional symptoms)...Hodgkin or Non-Hodgkins?
Hodgkins
66
Which has common extranodal involvement... Hodgkin or Non-Hodgkins?
Non- Hodgkins
67
Reed- Sternberg cells?
Hodgkin lymphoma
68
Markers of reed Sternberg cells?
CD15 + CD30 (B cell origin)
69
Most common form of hodgkin lymphoma?
Nodular sclerosing form
70
Name the cancer and the genes involved: t(8;14)
Burkitt lymphoma c-myc (8) heavy chain Ig (14)
71
Most common NHL in adults?
diffuse large B cell lymphoma
72
Name the cancer and the genes involved: t(14;18)
Follicular lymphoma Heavy chain Ig (14) BCL-2 (18)
73
Name the cancer and the genes involved: t(11;14)
Mantle cell lymphoma ``` cyclin D1 (11) Heavy chain Ig (14) ```
74
"cutaneous lesions and lytic bone lesions caused by a virus"
Adult T cell lymphoma
75
Most common primary tumor arising within bone of people >40-50yo?
Multiple myeloma
76
Symptoms of Multiple myeloma? CRAB
hyerCalcemia Renal involvement (bence jones) Anemia Bone lytic lesions/ Back pain
77
Which Ig is increased in multiple myeloma?
IgG and IgA
78
"monoclonal expansion of plasma cells, asymptomatic"
Monoclonal gammopathy of undetermined dignificance
79
Which plasma cell disorder has increased IgM?
Waldenstrom macroglobulinemia
80
Which cytokine stimulates plasma cell growth?
IL-6
81
Which cytokine stimulates plasma cell growth?
IL-6
82
Which leukemia is TdT+?
ALL
83
Most common adult leukemia?
CLL/ small cell lymphoma
84
Leukemia associated with marrow fibrosis in the elderly?
Hairy cell leukemia
85
Which leukemia is TRAP (+)?
Hairy cell leukemia
86
Auer rods are associated with...
AML
87
Name the cancer and the genes involved: t(15;17)
AML/ PML RAR alpha translocation
88
Which leukemia is associated with basophelia?
CML
89
Name the cancer and the genes involved: t(9; 22)
CML BCR- ABL fusion gene with tyrosine kinase activity
90
"lytic bone lesions, skin rash, recurrent otitis media, mass of mastoid bone"
langerhans cell histiocytosis
91
"tennis racket" cells?
Langerhans cell histiocytosis
92
A mutation in what protein is associated with PV, ET and Myelofibrosis?
JAK2 mutation
93
"intense itching after hot shower"
PV becuase the basophelia

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