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Heme Flashcards

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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
Aplastic anemia tx
50 ATG + cyclosporine Refractory to ATG- alemtuzumab
26
Burr and spur cells
Burr- renal | Spur- liver
27
Warm antibody
Ag: Rh Ab: IgG +/- c3 Etio: sle, cll, lymphoma, drugs Tx: steroids, danazol, splenectomy, rituximab
28
Cold antibody
Ag: iAg Ab: IgM, C3 Etio: quinidine, lymphoba, viral Tx: no role for steroids Cyclophosphamide Chlorambucil - rituximab
29
PNH dxtic
CD 55, 59
30
Tx for PNH
BM transplant | Eculizumab dec transfusion need
31
Dxtic for hereditary spherocytosis
Osmotic fragility test
32
Tx for hereditary spherocytosis
FA, splenectomy
33
Defect in spherocytosis
Spectrin membrane defect
34
Bite/ blister cells
G6PD
35
When to repeat G6PD levels after attack
2-3 months
36
TTP
Fever, renal, pancytopenia, autoimmune hemolytic anemia, neuro (headache, forgetfulness)
37
T/F Pt, ptt is abnormal in TTP
F
38
Tx for TTP
``` Plasmapheresis FFP (bec it has adamts 13) ```
39
Bloody diarrhea, renal failure, hemolytic anemia
HU Tx: supportive
40
Fever, chills, sob w/in one hr of BT
Major hemolytic rxn - ABO incompatibility
41
Hematuria, jaundice a week after BT
Late transfusion rxn- Rh incompatibility
42
Hx of Urticaria and allergies needs BT
Washed PRBC
43
Fever and chills every time transfused
Leukocyte reduced PRBC
44
Fever, chills, sob, angioedema minutes into BT
IgA deficiency Tx: washed PRBC
45
Fever, chills, hypotension, oliguria w/ no hematuria w/in 2 hrs of BT
Bacterial contamination
46
Defect in TRALI
Anti- Leukocyte ab fr donor
47
Post transfusion purpura
PLA-1 ab fr prior alloimmunization
48
Px with platelet 4 ab needing anticoagulation
Bivalirudin
49
Hx of HIIT, platelet 4 ab neg now, requires anticoagulation
Heparin for 4-6hrs then arixtra after
50
Universal donor
O-
51
Universal recipient
AB+
52
Labs in primary hemostasis (platelets)
Bleeding time inc, normal PT/PTT
53
Labs in secondary hemostasis (clotting factors)
Normal bleeding time, inc pt/ptt
54
Dxtic for vWd
Ristocetin cofactor assay (dec) Inc BT, PTT normal or inc, PT normal
55
Tx for vWd
Mild DDAVP Severe factor VIII concentrates (which contains vwF) or cryo NOT FFP
56
Giant platelets, inc BT
Bernard Soulier dse Etio: Jak2 mutation
57
Defect in platelet aggregation; g2b3a
Glanzmann's dse
58
Glycoprotein 2b3a inhibitor
Abciximab, tirofiban, eptifibatide
59
Direct thrombin inhibitor
Bivalirudin, argatroban, dabigatran
60
Which direct thrombin inhibitor can be used in renal pxs
Argatroban
61
Gestational thrombocytopenia target
Plt > 70000 Monitor 1-2x weekly
62
T/F ab are required for dx of ITP
F
63
ITP w/ plt > 30,000 tx
None
64
ITP w/ plt
Prednisone -- IV Ig --- cyclophosphamide/rituximab/ romiplostim/ splenectomy
65
ITP w/ plt > 50000 with bleeding or for surgery tx
IV Ig + plt
66
ITP w/ plt
Iv Ig + plt
67
What to do for pxs with HIT who need anticoagulation
Give direct thrombin inhibitors
68
What to do if px develops hyperK on heparin
Give fondaparinux
69
Inc ptt normal pt
``` Heparin No bleed factor 12 Mild bleed factor 11 Mod bleed factor 9 Severe bleed factor 8 ```
70
Ptt corrects w/ mixing
Factor def
71
Ptt does not correct w/ mixing
Factor inihibitor
72
Dxtic for lupus anticoagulant
Dilute russel viper venom
73
Dxtic for factor 13
Clot retraction test
74
Inc PT normal PTT
Warfarin Xa inhibitors Vit k def Factor 7 def
75
Tx for factor 13 def
Ffp
76
Tx for hemophilia a
For any surgery or bleeding, factor should have 50% activity for 48-72 hours (surg) or 2 wks (ICH)
77
Px with hemophilia developed ab to factor 8 conc
Give factor 7 conc
78
Inr 5 on warfarin, no bleeding
Hold one dose then dec by 5-20%
79
On warfarin inr 5-9 no bleeding
Give vit k 2.5 mg po. Recheck inr. Resume at lower dose once at 3
80
Warfarin inr > 9, or on warfarin w/ bleeding
4 factor prothrombin conc
81
Leading cause of dvt
Factor V leiden mutation
82
Second leading cause of dvt
Factor II 20210 defect
83
Is protein C or S dec in pregnancy?
Protein S
84
Duration of anticoagulation in px w/ factor v leiden or protein c def
6 mos
85
Duration of anticoagulation in px w/ reversible cause of dvt
3 mos
86
Duration of anticoagulation in px w/ lupus anticoagulant
Indefinitely
87
When to do work-up for hypercoagulable states
At least 2 wks off anticoagulation
88
Choice of anticoagulant in pregnancy
LMWH
89
Duration of anticoagulation in pregnancy
Until 6 wks postpartum
90
Duration of bridging
At least 5 days with target inr for 24hrs
91
Tx for warfarin skin necrosis
Ffp
92
Tear drop cells
Myelofibrosis
93
EPO level in polycythemia vera
Low
94
T/F all myeloproliferative syndromes can burn out in myelofibrosis
T
95
Dx of PV
Hct > 60, splenomegaly, jak2 mutation
96
Tx of PV
Phlebotomy to keep Hct
97
Tx for myelofibrosis
50 supportive with BT +/- ruxolitinib
98
Dx of essential thrombocytosis
Plt > 600,000, jak2 mutation
99
Tx of essential thrombocytosis
Low dose asa, Hydroxyurea, anagrelide | Urgent: plateletpheresis
100
Dec LAP score
CML PNH Wilson's dse
101
Dx of CML
FISH for t(9,22) philadelphia chromosome | PCR for Abl/bcr fusion gene
102
Tx of cml
50 gleevex
103
Auer rods
AML
104
Myeloperoxidase stain pos in
M2-M5
105
Which type of aml prone to dic
M3 T(15,17) Tx: ATRA
106
Tx of aml
50 cytarabine, daunorubicin
107
Tx for ATRA differentiation syndrome
Dexamethasone
108
Sweet syndrome
Fever, leukocytosis, painful erythematous nodules and plaque Biopsy: dense neutrophilic infiltrate Assoc with aml
109
Smudge cells
CML
110
Tx of cml
Asymptomatic : none Fludarabine, chlorambucil, rituximab IvIg for infections
111
Indication for tx of cll
Anemia
112
BRAF V600E mutation
Hairy cell leukemia Tx: cladribine

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