Haem Flashcards
1
Q
only type of microcytic anaemia with increased reticulocyte count
A
3 gene deletion alpha thalassemia
2
Q
only type of microcytic anaemia with increased reticulocyte count
A
3 gene deletion alpha thalassemia
3
Q
new fact about sideroblastic anemia
A
it causes BOTH micro and MACROcytic anaemia
4
Q
when to transfuse in anaemia
A
- symptomatic
- Hct very low
- elderly
- heart disease
5
Q
Hct very low
A
6
Q
what transfusion to use in person with IgA deficiency
A
give IgA deficient FFP
7
Q
each unit of PRBC….
A
increases Hct by 3 points per unit, or 1g/dl of Hg
8
Q
what is NOT the answer to haemophilia A or B
A
FFP
9
Q
cryoprecipitate….
A
NEVER used first for anything
10
Q
whole blood….
A
NEVER correct, since divided into PRBC or FFP
11
Q
transfuse in asymptomatic young person with anaemia
A
NO NEED TO TRANSFUSE
12
Q
levels of hepcidin in anaemia of chronic disease
A
LOW levels
13
Q
PC= asymptomatic anaemia with NORMAL Fe studies
A
thalassemia
14
Q
target cells most commonly found in….
A
thalassemia
15
Q
ferritin in Fe deficiency anaemia
A
= LOW;
but……1 out of 3 patients: normal or increased
16
Q
new thing elevated in Fe deficiency anaemia
A
platelets
17
Q
new fact about sideroblastic anemia
A
it causes BOTH micro and MACROcytic anaemia
18
Q
when to transfuse in anaemia
A
- symptomatic
- Hct very low
- elderly
- heart disease
19
Q
Hct very low
A
less than 25-30
20
Q
what transfusion to use in person with IgA deficiency
A
give IgA deficient FFP
21
Q
each unit of PRBC….
A
increases Hct by 3 points per unit, or 1g/dl of Hg
22
Q
what is NOT the answer to haemophilia A or B
A
FFP
23
Q
cryoprecipitate….
A
NEVER used first for anything
24
Q
whole blood….
A
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
