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Master the Boards > Haem > Flashcards

Haem Flashcards

1
Q

only type of microcytic anaemia with increased reticulocyte count

A

3 gene deletion alpha thalassemia

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2
Q

only type of microcytic anaemia with increased reticulocyte count

A

3 gene deletion alpha thalassemia

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3
Q

new fact about sideroblastic anemia

A

it causes BOTH micro and MACROcytic anaemia

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4
Q

when to transfuse in anaemia

A
  1. symptomatic
  2. Hct very low
    - elderly
    - heart disease
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5
Q

Hct very low

A
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6
Q

what transfusion to use in person with IgA deficiency

A

give IgA deficient FFP

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7
Q

each unit of PRBC….

A

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

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8
Q

what is NOT the answer to haemophilia A or B

A

FFP

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9
Q

cryoprecipitate….

A

NEVER used first for anything

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10
Q

whole blood….

A

NEVER correct, since divided into PRBC or FFP

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11
Q

transfuse in asymptomatic young person with anaemia

A

NO NEED TO TRANSFUSE

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12
Q

levels of hepcidin in anaemia of chronic disease

A

LOW levels

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13
Q

PC= asymptomatic anaemia with NORMAL Fe studies

A

thalassemia

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14
Q

target cells most commonly found in….

A

thalassemia

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15
Q

ferritin in Fe deficiency anaemia

A

= LOW;

but……1 out of 3 patients: normal or increased

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16
Q

new thing elevated in Fe deficiency anaemia

A

platelets

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17
Q

new fact about sideroblastic anemia

A

it causes BOTH micro and MACROcytic anaemia

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18
Q

when to transfuse in anaemia

A
  1. symptomatic
  2. Hct very low
    - elderly
    - heart disease
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19
Q

Hct very low

A

less than 25-30

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20
Q

what transfusion to use in person with IgA deficiency

A

give IgA deficient FFP

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21
Q

each unit of PRBC….

A

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

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22
Q

what is NOT the answer to haemophilia A or B

A

FFP

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23
Q

cryoprecipitate….

A

NEVER used first for anything

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24
Q

whole blood….

A

NEVER correct, since divided into PRBC or FFP

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25
Q

transfuse in asymptomatic young person with anaemia

A

NO NEED TO TRANSFUSE

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26
Q

levels of hepcidin in anaemia of chronic disease

A

LOW levels

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27
Q

PC= asymptomatic anaemia with NORMAL Fe studies

A

thalassemia

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28
Q

target cells most commonly found in….

A

thalassemia

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29
Q

ferritin in Fe deficiency anaemia

A

= LOW;

but……1 out of 3 patients: normal or increased

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30
Q

new thing elevated in Fe deficiency anaemia

A

platelets

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31
Q

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

A

bone marrow biopsy

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32
Q

most accurate test in thalassemia

A

electrophoresis

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33
Q

alpha thalassemia diagnosis

A

DNA analysis

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34
Q

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

A

EPO replacement

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35
Q

tx of sideroblastic anaemia

A

B6

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36
Q

Cooley anemia

A

= beta thalassemia major

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37
Q

diagnosis of MACROcytic anaemia

A

peripheral blood smear (because looking for hyperhsegmented neutrophils)

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38
Q

increased methylmalonic acid

A

B12 deficiency

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39
Q

B12 deficiency

A

= high LDH + high bilirubin + low reticulocytes

—-> ineffective erythropoiesis

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40
Q

schilling test

A

NEVER the correct answer

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41
Q

pernicious anaemia dx confirmation

A

Anti-if Ab, Anti-parietal cell Ab

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42
Q

B12 and folate deficiency cause what two things…

A
  1. MACROcytic anaemia

2. PANCYTOPENIA!!!!!!!!!!!!!!!!!!!!!

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43
Q

improvement of neuro symptoms with B12 deficiency

A
  • minor

- short duration

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44
Q

complication of B12 or folate replcaement

A

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

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45
Q

best initial test for sickle cell

A

blood smear

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46
Q

most accurate test for sickle cell

A

electrophoresis

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47
Q

2 things that lower mortality in sickle cell

A
  1. hydroxyurea prevention

2. antibiotics with fever

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48
Q

morulae inside neutrophils

A

ehrlichia infections

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49
Q

what to do…. sickle cell patient with fever

A

ANTIBIOTICS ASAP– do not wait for testing

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50
Q

exchange transfusion severe VOC:

A
  • acute chest syndrome
  • priapism
  • stroke
  • visual disturbance– retinal infarct
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51
Q

parvovirus B19 most accurate test

A

PCR > IgM level

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52
Q

best initial tx of parvovirus B19

A

IVIG

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53
Q

bone marrow in parvovirus B19

A

rarely done, but shows…

GIANT PRONORMOBLASTS

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54
Q

treatment of warm agglutinin disease

A
  1. steroids
  2. splenectomy
  3. IVIG
  4. rituximab
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55
Q

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

A
  1. cyclophosphamide
  2. cyclosporine
  3. azathioprine
  4. mycophenolate mofetil
56
Q

tx of cold agglutinin

A
  1. stay warm
  2. rituximab and sometimes plasmapheresis
  3. cyclophosmaide, cyclosporine, other immunosuppressive agents, stop Ab production
57
Q

steroids and splenectomy in cold agglutinin

A

NEVERRRRRRR NOOOOOOO WRONG ANSWER

58
Q

cryoglobulins a/w

A

Hep C
joint pain
glomerulonephritis

59
Q

DELAY plasmapharesis tx of HUS/TTP….

A

transfuse FFP

60
Q

transfusion of platelets in HUS/TTP

A

NOOOOOOOOOOOOO

worsens the disease

61
Q

most accurate test in PNH

A

FLOW CYTOMETRY– CD55,CD59

62
Q

treatment of PNH

A
  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
Q

most common sites of thrombosis in PNH

A
  1. hepatic vein thrombosis

2. mesentetric vein thrombosis

64
Q

tx of aplastic anaemia

A
  1. blood transfusion (anemia), antibiotics (infection), platelets (bleeding)
  2. BMT allogenic– young
  3. too old for BMT
65
Q

tx if too old for BMT in aplastic anaemia

A
  1. antithymocyte globulin= ATG

2. cyclosporine (or Tarcolimus), since T cell mediated response

66
Q

2 things that present with pruritus after bath in harm onc

A
  1. PCV

2. CML

67
Q

MCV in PCV

A

LOW

68
Q

after spleen removal in PCV, what happens to platelets

A

transient INCREASE in platelets

69
Q

new point of thing elevated in PCV

A

B12 INCREASE (unsure reason why)

70
Q

most accurate test PCV

A

JAK2 mutation

71
Q

ruzolitinib

A

inhibits JAK2

72
Q

JAK2 in ET

A

50%

73
Q

ET secondary in

A
  • infection
  • malignancy
  • Fe deficiency
74
Q

best initial tx of ET

A

HYDROXYUREA

75
Q

Tx erythromelalgia

A

ASPIRIN

76
Q

tx of myelofibrosis

A

thalidomide and lenalidomide

77
Q

best initial test for acute leukaemia

A

BLOOD SMEAR– BLASTS

78
Q

best indicator of prognosis in acute leukaemia is

A

CYTOGENETICS

79
Q

what tx must you add for ALL

A

intrathecal MTX

80
Q

most accurate test in CML

A

FISH or PCR– Bcr:Abl

81
Q

best initial therapy for CML

A

Imatinib
Dasatinib
Nilotinib
= TK inhibitors

82
Q

most effective cure of CML

A

BMT– NEVER this first therapy

83
Q

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

A

CML

84
Q

tx of leukostasis reaction

A

LEUKAPHERESIS

85
Q

what deletion is associated with myelodysplastic syndrome

A

5q deletion

86
Q

what 2 complications occur in MDS before development of acute leukaemia

A

bleeding and infection

87
Q

diagnosis of MDS:

A
  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
Q

5q deletion tx

A

LENALIDOMIDE

89
Q

ringed sideroblasts not in sideroblastic anemia

A

myelodysplastic syndrome

90
Q

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

A

AZACITIDINE

91
Q

richter phenomenon

A

5% CLL–> acute leukemia

92
Q

stage 3 CLL

A

anaemia

93
Q

stage 4 CLL

A

thrombocytopenia

94
Q

stage 0 CLL

A

elevated WBC

95
Q

stage 1 CLL

A

LAD

96
Q

stage 2 CLL

A

HSM

97
Q

tx for stage 0-2 CLL

A

NO TX

98
Q

tx for stage 3 and 4 CLL

A
  • fludrabine
  • cyclophosphamide
  • rituximab
99
Q

when fludrabine fails in CLL

A

alemtuzumab= anti-CD-52

100
Q

refractory cases CLL

A

cyclophos

101
Q

mild cases of CLL

A

chlorambucil (elderly)

102
Q

severe infection CLL

A

IVIG

103
Q

autoiummune thrombocytopenia or hemolysis in CLL

A

prednisone

104
Q

what prophylaxis in CLL

A

PCP

105
Q

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

A

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

106
Q

tx of hairy cell leukemia

A

cladribine or pentostatin

107
Q

non-HL best initial test

A

excisional biopsy– NOT NEEDLE ASIRATION

108
Q

tx of non-HL stage Ia, IIa

A

local radiation and small course CTX

109
Q

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

A 
CHOP + rituximab
Cyclophosphamide
Hydroxydaunorubicin
Oncovin== vincristine
Prednisone
110
Q

presentation of advanced non-hl

A

80 to 90%

111
Q

tx of HL

A 
ABVD
Adriamycin
Bleomycin
Vinblastine
Dacarbazine
112
Q

complications off radiation

A

solid tumors: breast/thyroid/lung

increased chance of PREMATURE CORONARY ARTERY DISEASE

113
Q

relapses after radiaiton….

A

tx= CTX

114
Q

relapses after CTX…..

A

tx= INCREASE CTX

115
Q

what scan before adriamycin

A

MUGA or nuclear ventriculogram

116
Q

SPEP in MM

A 
IgG= 60%
IgA= 25%
117
Q

bence jones protein found on….

A

urine electrophoresis

118
Q

most common causes of death in MM

A

INFECTION

RENAL FAILURE

119
Q

what type of anion gap in MM

A

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

120
Q

single most accurate test for MM

A

bone marrow biopsy: greater than 10% plasma cells

121
Q

of those with M spike, how many have myeloma

A

1% have myeloma

99% DO NOT have myeloma

122
Q

best initial therapy in waldenstroms

A

PLASMAPHARESIS

123
Q

SIZE and ITP

A 
spleen= normal
platelets= LARGE
124
Q

no bleeding, count> 30,000 ITP

A

NO tx

125
Q

mild bleeding, count

A

glucocorticoids

126
Q

severe bleeding (GI or CNS), count

A

IVIG, anti-D

127
Q

recurrent episodes, steroid dependence ITP

A

splenectomy

128
Q

ineffective splenectomy or steroids in ITP

A
  • romiplostim or eltrombopag
  • rituximab
  • azathioprine
  • cyclosporine
  • mycophenolate
129
Q

romiplostim

A

synthetic thrombopoietin

130
Q

eltrombopag

A

synthetic thrombopoietin

131
Q

tx of HIT

A

STOP all heparin containing products

- administer direct thrombin inhibitors: argatroban, lepirudin, bivalrudin, fondaparinux

132
Q

scenario where get one clot, but require lifelong warfarin

A

APLS

133
Q

arterial or venous clots in APLS

A

VENOUS

134
Q

confirmed dx of HIT

A

ELISA– platelet factor 4 antibodies

135
Q

best initial test for APLS

A

mixing study

136
Q

transfuse platelets in those with HIT?

A

NOOOOOOO makes it worse

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