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Flashcards in Haem Deck (136):
1

only type of microcytic anaemia with increased reticulocyte count

3 gene deletion alpha thalassemia

2

only type of microcytic anaemia with increased reticulocyte count

3 gene deletion alpha thalassemia

3

new fact about sideroblastic anemia

it causes BOTH micro and MACROcytic anaemia

4

when to transfuse in anaemia

1. symptomatic
2. Hct very low
- elderly
- heart disease

5

Hct very low

6

what transfusion to use in person with IgA deficiency

give IgA deficient FFP

7

each unit of PRBC....

increases Hct by 3 points per unit, or 1g/dl of Hg

8

what is NOT the answer to haemophilia A or B

FFP

9

cryoprecipitate....

NEVER used first for anything

10

whole blood....

NEVER correct, since divided into PRBC or FFP

11

transfuse in asymptomatic young person with anaemia

NO NEED TO TRANSFUSE

12

levels of hepcidin in anaemia of chronic disease

LOW levels

13

PC= asymptomatic anaemia with NORMAL Fe studies

thalassemia

14

target cells most commonly found in....

thalassemia

15

ferritin in Fe deficiency anaemia

= LOW;
but......1 out of 3 patients: normal or increased

16

new thing elevated in Fe deficiency anaemia

platelets

17

new fact about sideroblastic anemia

it causes BOTH micro and MACROcytic anaemia

18

when to transfuse in anaemia

1. symptomatic
2. Hct very low
- elderly
- heart disease

19

Hct very low

less than 25-30

20

what transfusion to use in person with IgA deficiency

give IgA deficient FFP

21

each unit of PRBC....

increases Hct by 3 points per unit, or 1g/dl of Hg

22

what is NOT the answer to haemophilia A or B

FFP

23

cryoprecipitate....

NEVER used first for anything

24

whole blood....

NEVER correct, since divided into PRBC or FFP

25

transfuse in asymptomatic young person with anaemia

NO NEED TO TRANSFUSE

26

levels of hepcidin in anaemia of chronic disease

LOW levels

27

PC= asymptomatic anaemia with NORMAL Fe studies

thalassemia

28

target cells most commonly found in....

thalassemia

29

ferritin in Fe deficiency anaemia

= LOW;
but......1 out of 3 patients: normal or increased

30

new thing elevated in Fe deficiency anaemia

platelets

31

most accurate test for Fe deficiency anaemia (But rarely done....)

bone marrow biopsy

32

most accurate test in thalassemia

electrophoresis

33

alpha thalassemia diagnosis

DNA analysis

34

only anemia a/w end stage renal failure responds to...

EPO replacement

35

tx of sideroblastic anaemia

B6

36

Cooley anemia

= beta thalassemia major

37

diagnosis of MACROcytic anaemia

peripheral blood smear (because looking for hyperhsegmented neutrophils)

38

increased methylmalonic acid

B12 deficiency

39

B12 deficiency

= high LDH + high bilirubin + low reticulocytes
----> ineffective erythropoiesis

40

schilling test

NEVER the correct answer

41

pernicious anaemia dx confirmation

Anti-if Ab, Anti-parietal cell Ab

42

B12 and folate deficiency cause what two things...

1. MACROcytic anaemia
2. PANCYTOPENIA!!!!!!!!!!!!!!!!!!!!!

43

improvement of neuro symptoms with B12 deficiency

- minor
- short duration

44

complication of B12 or folate replcaement

HYPOkalaemia, because so quickly new blood cells are made--> marrow packages up all the potassium

45

best initial test for sickle cell

blood smear

46

most accurate test for sickle cell

electrophoresis

47

2 things that lower mortality in sickle cell

1. hydroxyurea prevention
2. antibiotics with fever

48

morulae inside neutrophils

ehrlichia infections

49

what to do.... sickle cell patient with fever

ANTIBIOTICS ASAP-- do not wait for testing

50

exchange transfusion severe VOC:

- acute chest syndrome
- priapism
- stroke
- visual disturbance-- retinal infarct

51

parvovirus B19 most accurate test

PCR > IgM level

52

best initial tx of parvovirus B19

IVIG

53

bone marrow in parvovirus B19

rarely done, but shows...
GIANT PRONORMOBLASTS

54

treatment of warm agglutinin disease

1. steroids
2. splenectomy
3. IVIG
4. rituximab

55

alternate treatments of warm agglutinin disease, to diminish need for steroids:

1. cyclophosphamide
2. cyclosporine
3. azathioprine
4. mycophenolate mofetil

56

tx of cold agglutinin

1. stay warm
2. rituximab and sometimes plasmapheresis
3. cyclophosmaide, cyclosporine, other immunosuppressive agents, stop Ab production

57

steroids and splenectomy in cold agglutinin

NEVERRRRRRR NOOOOOOO WRONG ANSWER

58

cryoglobulins a/w

Hep C
joint pain
glomerulonephritis

59

DELAY plasmapharesis tx of HUS/TTP....

transfuse FFP

60

transfusion of platelets in HUS/TTP

NOOOOOOOOOOOOO
worsens the disease

61

most accurate test in PNH

FLOW CYTOMETRY-- CD55,CD59

62

treatment of PNH

1. prednisone-- best initial tx for haemolysisi
2. BMT-allogenic= CURE
3. Eculizumab: for haemolysis and thrombosis
4. folic acid and replacement with transfusions as needed

63

most common sites of thrombosis in PNH

1. hepatic vein thrombosis
2. mesentetric vein thrombosis

64

tx of aplastic anaemia

1. blood transfusion (anemia), antibiotics (infection), platelets (bleeding)
2. BMT allogenic-- young
3. too old for BMT

65

tx if too old for BMT in aplastic anaemia

1. antithymocyte globulin= ATG
2. cyclosporine (or Tarcolimus), since T cell mediated response

66

2 things that present with pruritus after bath in harm onc

1. PCV
2. CML

67

MCV in PCV

LOW

68

after spleen removal in PCV, what happens to platelets

transient INCREASE in platelets

69

new point of thing elevated in PCV

B12 INCREASE (unsure reason why)

70

most accurate test PCV

JAK2 mutation

71

ruzolitinib

inhibits JAK2

72

JAK2 in ET

50%

73

ET secondary in

- infection
- malignancy
- Fe deficiency

74

best initial tx of ET

HYDROXYUREA

75

Tx erythromelalgia

ASPIRIN

76

tx of myelofibrosis

thalidomide and lenalidomide

77

best initial test for acute leukaemia

BLOOD SMEAR-- BLASTS

78

best indicator of prognosis in acute leukaemia is

CYTOGENETICS

79

what tx must you add for ALL

intrathecal MTX

80

most accurate test in CML

FISH or PCR-- Bcr:Abl

81

best initial therapy for CML

Imatinib
Dasatinib
Nilotinib
= TK inhibitors

82

most effective cure of CML

BMT-- NEVER this first therapy

83

of all the myeloproliferative disorders-- which is most likely to transform into acute leukemia

CML

84

tx of leukostasis reaction

LEUKAPHERESIS

85

what deletion is associated with myelodysplastic syndrome

5q deletion

86

what 2 complications occur in MDS before development of acute leukaemia

bleeding and infection

87

diagnosis of MDS:

1. CBC: increased mcv, nucleated red cells, less than 20% blasts
2. hypercellular marrow
3. ringed sideroblasts
4. severity-- blast percentage dependent
5/ 5q deletion= better prognosis

88

5q deletion tx

LENALIDOMIDE

89

ringed sideroblasts not in sideroblastic anemia

myelodysplastic syndrome

90

what drug decreases transfusion dependence, and ultimately increases survival in MDS

AZACITIDINE

91

richter phenomenon

5% CLL--> acute leukemia

92

stage 3 CLL

anaemia

93

stage 4 CLL

thrombocytopenia

94

stage 0 CLL

elevated WBC

95

stage 1 CLL

LAD

96

stage 2 CLL

HSM

97

tx for stage 0-2 CLL

NO TX

98

tx for stage 3 and 4 CLL

- fludrabine
- cyclophosphamide
- rituximab

99

when fludrabine fails in CLL

alemtuzumab= anti-CD-52

100

refractory cases CLL

cyclophos

101

mild cases of CLL

chlorambucil (elderly)

102

severe infection CLL

IVIG

103

autoiummune thrombocytopenia or hemolysis in CLL

prednisone

104

what prophylaxis in CLL

PCP

105

which is less dangerous: thrombocytopenia and anemia from autoimmune effect, or from marrow infiltration with CLL cells?

autoimmune effect-- treated with prednisone (NOT the same as stage 3/4)

106

tx of hairy cell leukemia

cladribine or pentostatin

107

non-HL best initial test

excisional biopsy-- NOT NEEDLE ASIRATION

108

tx of non-HL stage Ia, IIa

local radiation and small course CTX

109

tx of non-HL stage III, IV, B symptoms

CHOP + rituximab
Cyclophosphamide
Hydroxydaunorubicin
Oncovin== vincristine
Prednisone

110

presentation of advanced non-hl

80 to 90%

111

tx of HL

ABVD
Adriamycin
Bleomycin
Vinblastine
Dacarbazine

112

complications off radiation

solid tumors: breast/thyroid/lung
increased chance of PREMATURE CORONARY ARTERY DISEASE

113

relapses after radiaiton....

tx= CTX

114

relapses after CTX.....

tx= INCREASE CTX

115

what scan before adriamycin

MUGA or nuclear ventriculogram

116

SPEP in MM

IgG= 60%
IgA= 25%

117

bence jones protein found on....

urine electrophoresis

118

most common causes of death in MM

INFECTION
RENAL FAILURE

119

what type of anion gap in MM

DECREASED anion gap (since IgG= positive, increase Cl-, HCO3-)

120

single most accurate test for MM

bone marrow biopsy: greater than 10% plasma cells

121

of those with M spike, how many have myeloma

1% have myeloma
99% DO NOT have myeloma

122

best initial therapy in waldenstroms

PLASMAPHARESIS

123

SIZE and ITP

spleen= normal
platelets= LARGE

124

no bleeding, count> 30,000 ITP

NO tx

125

mild bleeding, count

glucocorticoids

126

severe bleeding (GI or CNS), count

IVIG, anti-D

127

recurrent episodes, steroid dependence ITP

splenectomy

128

ineffective splenectomy or steroids in ITP

- romiplostim or eltrombopag
- rituximab
- azathioprine
- cyclosporine
- mycophenolate

129

romiplostim

synthetic thrombopoietin

130

eltrombopag

synthetic thrombopoietin

131

tx of HIT

STOP all heparin containing products
- administer direct thrombin inhibitors: argatroban, lepirudin, bivalrudin, fondaparinux

132

scenario where get one clot, but require lifelong warfarin

APLS

133

arterial or venous clots in APLS

VENOUS

134

confirmed dx of HIT

ELISA-- platelet factor 4 antibodies

135

best initial test for APLS

mixing study

136

transfuse platelets in those with HIT?

NOOOOOOO makes it worse