Hem/ Onc Pathology High Yield Flashcards Preview

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Flashcards in Hem/ Onc Pathology High Yield Deck (93):
1

Acanthocyte/ "spur cells" are seen in...

abetalipoproteinemia and liver disease

2

Basophilic stippling of RBCs are seen in...

Lead poisoning

3

What causes ringed sideroblasts?

excess Fe in mitochondira (sideroblastic anemia)

4

What can induce cycling?

dehydration, deoxygenation, high altitudes

5

When are dacrocyte (tear drop cells) seen?

bone marrow infiltration

6

What are the 4 pathologies associated with target cells?

HbC disease
Asplenia
Liver disease
Thalassemia

7

What causes heinz bodies?

Oxidation of Hb SH gropus to S---S

Leads to Hb precipitation

8

What pathology is associated with heinz bodies?

G6pD deficiency

9

What pathology is associated with heinz bodies?

G6pD deficiency

10

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

reticulocyte count

11

Transporter for Fe in the small intestine?

DMT1 transporter

12

Fe storage protein?

ferritin

13

"Fe deficiency anemia, esophageal webs, atrophic glossitis"

Plummer Vinson Syndrome

14

Is alpha thalassemia a mutation or a deletion?

It is a deletion of alpha globin genes

15

HbH disease?

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

16

Is beta thalassemia a mutation or a deletion?

It is a mutation in splice site and romotor sequences

17

Difference in presentation beta thalassemia major and minor?

Minor: usually asymptomatic, increased HbA2

Major: Beta chain is absent, severe, extramedulary hematopoesis

18

What are the 2 enzymes affected by lead poisoning?

ALA dehydrastase and ferrochetalase

19

Treatment for lead poisoning?

Dimercaprol, EDTA, succimer

20

What enzyme is deficiency in sideroblastic anemia? Inheritance pattern?

ALA synthase

X linked

21

What cofactor does ALA synthase require?

Pyridoxine (B6)

22

Ferretin and TIBC levels in sideroblastic anemia?

decreased TIBC and increased ferritin

23

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

B12 deficiency

24

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

B12 deficiency anemia

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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