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Heme Flashcards

1
Q

Most sensitive assay for IDA

A

Transferrin/ ferritin receptor assay

> 2: IDA

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2
Q

Best screening for hemochromatosis

A

Transferrin sat

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3
Q 
Normal lab values:
MCV
TIBC
Ferritin
Iron
Transferrin sat
A 
Mcv: 80-100
Tibc: 220-240
Ferritin: > 20
Iron: 50-160
Transferrin sat: 20-40%
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4
Q

Dxtic for alpha thalassemia

A

DNA PCR analysis

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5
Q

Dxtic for beta thalassemia

A

Hgb electrophoresis

+ hgb A2

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6
Q

Clues on CBC for beta thalassemia

A

Looks like iron def (anemia, low mcv) but normal RDW and high RBC count

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7
Q

Sickle cell trait

A

Splenic sequestration

Hematuria

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8
Q

Sickle cell anemia

A

Vaso-occlusive dse

Infxn w/ encapsulated

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9
Q

Sickle cell dse

A

Vaso-occlusive
Avacular necrosis
Retinal infarcts

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10
Q

Leading cause of death in sickle

A

Acute chest crisis

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11
Q

Shown to produce cure in sickle

A

Peripheral blood stem cell transplant

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12
Q

Sickle cell w/ stroke in evolution

A

Exchange transfusion

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13
Q

Most common cause of osteomyelitis in sickle

A

Salmonella

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14
Q

Characteristics of megaloblastic anemia

A

Inc LDH and bilirubin
Dec retic count
Oval macrocytes, hypercellular marrow w/ megaloblasts

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15
Q

T/F Folic acid will inc platelets

A

T (falsely elevated)

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16
Q

T/F all chronic hemolytic syndromes should receive folic acid

A

T (falsely normal)

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17
Q

Causes of folic def

A

A folic drop

Alcohol/ folate antagonists/ OCP/ low intake/ lnfxn/ celiac sprue/ dilantin/ rel folic def/ old age/ pregnancy

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18
Q

How to differentiate b12 fr folic def

A

Folic: high homocysteine and normal methylmalonic acid levels

B12: high himocysteine and high methymalonic acid levels (MM acid levels rise before b12 goes down)

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19
Q

T/F vit b6 def will also have high homocysteine levels

A

T

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20
Q

Normal b12 level

A

200-800

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21
Q

Megaloblastic anemia w: hyposegmented PMNs

A

Myelodysplastic syndrome

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22
Q

MDS tx

A

55 - azacitidine

epo 500 - antithymocyte Ig + cyclosporine
Unable to tolerate ATG- lenalidomide
5q deletion- lenalidomide

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23
Q

Dxtics for hemochromatosis

A

Screening: Transferrin sat > 45, ferritin > 1000
Dxtic: HFE gene testing

To find out extent of iron overload:
Liver biopsy

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24
Q

Tx for hemochromatosis

A

Phlebotomy

Deferoxamine

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25
Q

Aplastic anemia tx

A

50 ATG + cyclosporine

Refractory to ATG- alemtuzumab

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26
Q

Burr and spur cells

A

Burr- renal

Spur- liver

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27
Q

Warm antibody

A

Ag: Rh
Ab: IgG +/- c3

Etio: sle, cll, lymphoma, drugs

Tx: steroids, danazol, splenectomy, rituximab

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28
Q

Cold antibody

A

Ag: iAg
Ab: IgM, C3

Etio: quinidine, lymphoba, viral

Tx: no role for steroids
Cyclophosphamide
Chlorambucil - rituximab

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29
Q

PNH dxtic

A

CD 55, 59

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30
Q

Tx for PNH

A

BM transplant

Eculizumab dec transfusion need

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31
Q

Dxtic for hereditary spherocytosis

A

Osmotic fragility test

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32
Q

Tx for hereditary spherocytosis

A

FA, splenectomy

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33
Q

Defect in spherocytosis

A

Spectrin membrane defect

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34
Q

Bite/ blister cells

A

G6PD

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35
Q

When to repeat G6PD levels after attack

A

2-3 months

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36
Q

TTP

A

Fever, renal, pancytopenia, autoimmune hemolytic anemia, neuro (headache, forgetfulness)

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37
Q

T/F Pt, ptt is abnormal in TTP

A

F

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38
Q

Tx for TTP

A 
Plasmapheresis
FFP (bec it has adamts 13)
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39
Q

Bloody diarrhea, renal failure, hemolytic anemia

A

HU

Tx: supportive

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40
Q

Fever, chills, sob w/in one hr of BT

A

Major hemolytic rxn - ABO incompatibility

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41
Q

Hematuria, jaundice a week after BT

A

Late transfusion rxn- Rh incompatibility

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42
Q

Hx of Urticaria and allergies needs BT

A

Washed PRBC

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43
Q

Fever and chills every time transfused

A

Leukocyte reduced PRBC

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44
Q

Fever, chills, sob, angioedema minutes into BT

A

IgA deficiency

Tx: washed PRBC

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45
Q

Fever, chills, hypotension, oliguria w/ no hematuria w/in 2 hrs of BT

A

Bacterial contamination

46
Q

Defect in TRALI

A

Anti- Leukocyte ab fr donor

47
Q

Post transfusion purpura

A

PLA-1 ab fr prior alloimmunization

48
Q

Px with platelet 4 ab needing anticoagulation

A

Bivalirudin

49
Q

Hx of HIIT, platelet 4 ab neg now, requires anticoagulation

A

Heparin for 4-6hrs then arixtra after

50
Q

Universal donor

51
Q

Universal recipient

52
Q

Labs in primary hemostasis (platelets)

A

Bleeding time inc, normal PT/PTT

53
Q

Labs in secondary hemostasis (clotting factors)

A

Normal bleeding time, inc pt/ptt

54
Q

Dxtic for vWd

A

Ristocetin cofactor assay (dec)

Inc BT, PTT normal or inc, PT normal

55
Q

Tx for vWd

A

Mild DDAVP
Severe factor VIII concentrates (which contains vwF) or cryo

NOT FFP

56
Q

Giant platelets, inc BT

A

Bernard Soulier dse

Etio: Jak2 mutation

57
Q

Defect in platelet aggregation; g2b3a

A

Glanzmann’s dse

58
Q

Glycoprotein 2b3a inhibitor

A

Abciximab, tirofiban, eptifibatide

59
Q

Direct thrombin inhibitor

A

Bivalirudin, argatroban, dabigatran

60
Q

Which direct thrombin inhibitor can be used in renal pxs

A

Argatroban

61
Q

Gestational thrombocytopenia target

A

Plt > 70000

Monitor 1-2x weekly

62
Q

T/F ab are required for dx of ITP

63
Q

ITP w/ plt > 30,000 tx

64
Q

ITP w/ plt

A

Prednisone – IV Ig — cyclophosphamide/rituximab/ romiplostim/ splenectomy

65
Q

ITP w/ plt > 50000 with bleeding or for surgery tx

A

IV Ig + plt

66
Q

ITP w/ plt

A

Iv Ig + plt

67
Q

What to do for pxs with HIT who need anticoagulation

A

Give direct thrombin inhibitors

68
Q

What to do if px develops hyperK on heparin

A

Give fondaparinux

69
Q

Inc ptt normal pt

A 
Heparin
 No bleed factor 12
Mild bleed factor 11
Mod bleed factor 9
Severe bleed factor 8
70
Q

Ptt corrects w/ mixing

A

Factor def

71
Q

Ptt does not correct w/ mixing

A

Factor inihibitor

72
Q

Dxtic for lupus anticoagulant

A

Dilute russel viper venom

73
Q

Dxtic for factor 13

A

Clot retraction test

74
Q

Inc PT normal PTT

A

Warfarin
Xa inhibitors
Vit k def
Factor 7 def

75
Q

Tx for factor 13 def

76
Q

Tx for hemophilia a

A

For any surgery or bleeding, factor should have 50% activity for 48-72 hours (surg) or 2 wks (ICH)

77
Q

Px with hemophilia developed ab to factor 8 conc

A

Give factor 7 conc

78
Q

Inr 5 on warfarin, no bleeding

A

Hold one dose then dec by 5-20%

79
Q

On warfarin inr 5-9 no bleeding

A

Give vit k 2.5 mg po. Recheck inr. Resume at lower dose once at 3

80
Q

Warfarin inr > 9, or on warfarin w/ bleeding

A

4 factor prothrombin conc

81
Q

Leading cause of dvt

A

Factor V leiden mutation

82
Q

Second leading cause of dvt

A

Factor II 20210 defect

83
Q

Is protein C or S dec in pregnancy?

84
Q

Duration of anticoagulation in px w/ factor v leiden or protein c def

85
Q

Duration of anticoagulation in px w/ reversible cause of dvt

86
Q

Duration of anticoagulation in px w/ lupus anticoagulant

A

Indefinitely

87
Q

When to do work-up for hypercoagulable states

A

At least 2 wks off anticoagulation

88
Q

Choice of anticoagulant in pregnancy

89
Q

Duration of anticoagulation in pregnancy

A

Until 6 wks postpartum

90
Q

Duration of bridging

A

At least 5 days with target inr for 24hrs

91
Q

Tx for warfarin skin necrosis

92
Q

Tear drop cells

A

Myelofibrosis

93
Q

EPO level in polycythemia vera

94
Q

T/F all myeloproliferative syndromes can burn out in myelofibrosis

95
Q

Dx of PV

A

Hct > 60, splenomegaly, jak2 mutation

96
Q

Tx of PV

A

Phlebotomy to keep Hct

97
Q

Tx for myelofibrosis

A

50 supportive with BT +/- ruxolitinib

98
Q

Dx of essential thrombocytosis

A

Plt > 600,000, jak2 mutation

99
Q

Tx of essential thrombocytosis

A

Low dose asa, Hydroxyurea, anagrelide

Urgent: plateletpheresis

100
Q

Dec LAP score

A

CML
PNH
Wilson’s dse

101
Q

Dx of CML

A

FISH for t(9,22) philadelphia chromosome

PCR for Abl/bcr fusion gene

102
Q

Tx of cml

A

50 gleevex

103
Q

Auer rods

104
Q

Myeloperoxidase stain pos in

105
Q

Which type of aml prone to dic

A

M3

T(15,17)

Tx: ATRA

106
Q

Tx of aml

A

50 cytarabine, daunorubicin

107
Q

Tx for ATRA differentiation syndrome

A

Dexamethasone

108
Q

Sweet syndrome

A

Fever, leukocytosis, painful erythematous nodules and plaque

Biopsy: dense neutrophilic infiltrate

Assoc with aml

109
Q

Smudge cells

110
Q

Tx of cml

A

Asymptomatic : none

Fludarabine, chlorambucil, rituximab
IvIg for infections

111
Q

Indication for tx of cll

112
Q

BRAF V600E mutation

A

Hairy cell leukemia

Tx: cladribine

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