Hematology Flashcards
1
Q
what is the most common transfusion related infection?
A
CMV
2
Q
what proteins make up hemoglobin?
A
alpha
beta
delta
gamma
3
Q
what proteins make up a fetus’s hemoglobin?
A
alpha
gamma
4
Q
when does HgbA overtake HgbF?
A
HgA overtakes hemoglobin F by 3 months
by 6 months there is very little HbF
5
Q
what is HbA
A
a2b2
6
Q
what is HbA2
A
alpha 2 delta 2 (normally up to 3% of adult hgb)
7
Q
what is HbH
Hb Bart
A
HbH: 4 beta
Hb Bart: 4 delta
8
Q
are most mutations in alpha or beta??
A
beta!!
9
Q
when do we see physiologic nadir of Hb in term babies?
A
8-10 weeks
after birth oxygen saturation increases and therefore causes decreased EPO, then EPO production is resumed with an increase in the retic count
10
Q
when do we see physiologic nadir of Hb in preterm babies?
A
6-8 weeks
nadir is earlier and deeper (>70)
11
Q
what is the definition of anemia
A
Hb 2 standard deviations below mean value for age, gender and race
12
Q
how should you classify anemia?
A
decreased production
- microcytic
- macrocytic
- normocytic
increased destruction
- intrinsic- membrane, hemoglobin enzyme
- extrinsic- immune, non-immune
13
Q
where is your blood made?
A
bone marrow
14
Q
where is fetus’s blood made?
A
liver
15
Q
where is embryo’s blood made?
A
yolk sac
16
Q
what is normal hb for men? women? newborn?
A
men: 140-180
women: 120-160
newborn: up to 200/220
17
Q
what are markers of RBC destruction?
A
icterus dark urine increased reticulocytes increased unconjugated bilirubin increased LDH hemoglobin (not rbc's) in urine urobilinogen decreased haptoglobin
18
Q
Ddx of microcytic anemia
A
T- thalassemia A- anemia of chronic disease (usually normocytic but can be microcytic) I- iron deficiency L- lead S- sideroblastic
19
Q
Ddx macrocytic anemia
A
megaloblastic:
folate
B12
marrow failure:
myelodysplasia, diamond-blackfan, falcon anemia, aplastic anemia
other: hypothyroidism, Down syndrome, chronic liver disease, drugs (AZT, alcohol)
20
Q
Ddx of normocytic anemia
A
chronic disease chronic renal failure transient erythroblastopenia of childhood malignancy/marrow infiltration HIV HLH
Most common: TEC, malignancy or anemia of chronic disease
normocytic anemia with inadequate reticulocyte response should usually lead to consideration of a bone marrow exam
21
Q
Ddx of intrinsic hemolytic disorders
A
membrane: hereditary spherocytosis, elliptocytosis
enzyme: G6PD deficiency, PK deficiency
hemoglobinopathy: HbSS, SC, S-B thal
22
Q
Ddx of extrinsic hemolytic disorders
A
immune- autoimmune, iso-immune, drug-induced
non-immune- HUS, TTP, DIC, burns, Wilson, vit E deficiency
23
Q
what are the 3 most important lab tests for evaluating anemia?
A
CBC: MCV, WBC, RBC, Plts
reticulocyte count
peripheral blood smear
24
Q
what are some optional tests for iron deficiency anemia? (5)
A
serum iron transferrin/TIBC ferritin soluble transferrin receptor bone marrow biopsy
25
when do you see pencil cell on smear?
iron deficiency
26
when do you see basket cell on smear?
pathognomonic for G6PD deficiency
27
what is the most common cause of worldwide anemia?
iron deficiency anemia
| 10% of infants and adolescent girls are iron deficient of which one third are anemic
28
what are some complications of iron deficiency anemia
```
irritability
poor concentration
GI dysfunction
reduced immunity
pica
poor school performance
lead poisoning
```
29
what are 4 mechanisms causing iron deficiency anemia?
inadequate iron endowment at birth (prematurity)
insufficient iron in the diet
blood loss (menstruation)
malabsorption of iron (celiac disease, IBD, giardiasis)
30
why does excessive cows milk lead to iron deficiency anemia?
iron is absorbed at 50% efficiency from breast milk and 10% from cows milk
excessive cow's milk interferes with balanced nutrition and causes GI blood loss
31
what is the treatment for iron deficiency anemia?
limit cows milk to 16-24 ounces/day
start 4-6mg/kg/day of elemental iron (Taking vit C helps with absorption)
check CBC and retics within 1-2 weeks of treatment
therapy should continue for at least 3-6 months to replenish iron stores
if hemoglobin is not increased within 2 weeks then consider non-compliance
32
```
which of the following is not a feature of iron deficiency
pica
koilonychia
cheilosis
scleral icterus
psychomotor retardation
```
scleral icterus is NOT
koilonychia- groove in nails
cheilosis- cracking at corner of mouth
33
you are seeing a child with microcytic hypo chromic anemia. You believe it is iron deficiency. What other features of the CBC would be consistent with this?
1. increased RDW
2. thrombocytosis
3. MCV/RBC >13 (<13 is suggestive of thal trait)
4. pencil cells
34
what is seen if a patient has 3 alpha genes (1 alpha gene missing)
silent carrier state
slightly reduced hemoglobin and MCV
Hb electrophoresis pattern is the same as normal
35
what is seen if a patient has 2 alpha genes missing
anemia (100)
microcytosis
not picked up on Hb electrophoresis
36
what are the differences between iron deficiency anemia and alpha thal trait?
in alpha thal, iron studies are normal
in alpha thal, RDW is normal. in IDA RDW is high.
in alpha thal, RBC is increased, usually around 5 and the MCV is very low
in IDA the platelets are often increased
Hb electrophoresis is normal in both
37
what is the Mentzer's index?
MCV/RBC is >13 in IDA
| <13 in thal trait
38
what is seen if a patient has 3 alpha genes missing
```
Hb H disease
moderate anemia (70-100)
hypochromia
microcytosis
target cells
heinz bodies
splenomegaly
jaundice
HbBart may be present
```
39
what does Bo mean?
absent production (more severe thalassemia)
40
what does B+ mean?
reduced production
41
what does B thalassemia minor mean?
one defective gene
| either BBo or BB+
42
how do you diagnose B thalassemia minor? what is seen on hemoglobin electrophoresis? is treatment required?
based on Hb electrophoresis pattern
increased HbA2 and HbF
they do not need any treatment at all other than genetic counselling
there is no increased incidence of complications
43
what is B thalassemia major?
either BoB+ or BoBo
severe anemia
abnormal growth
iron overload and the need for transfusion
on electrophoresis: HbF (95%)
No HbA for BoBo
44
what is the most common complication seen with B thalassemia major?
iron overload
| mostly in the liver then the heart and endocrine glands
45
what is the treatment for thalassemia major?
regular transfusions and iron chelation
iron chelation: parenteral desferoxamine or oral deferasirox
bone marrow transplant can be curative
46
```
what is the most likely diagnosis with the following
HbA- none
HbA2- 2%
HbF- 75%
HbS- 25%
```
sickle cell trait
sickle cell disease
beta thalassemia
alpha thalassemia
sickle cell disease
47
Ddx of normocytic anemia
anemia of chronic disease
malignant infiltration
diamond-blackfan anemia
transient erythroblastopenia of childhood
48
what is transient erythroblastopenia of childhood
normocytic anemia
usually appears in otherwise health children age 1-3
often after a viral trigger (not necessarily Parvo)
onset is gradual, anemia can be severe
recovery is spontaneous within 1-2 months
the main ddx is diamond-blackfan anemia
key is that it is transient!!
HbF normal whereas HbF increased with diamond blackfan
49
what causes an aplastic crisis
parvovirus B19
50
what test can help confirm the diagnosis of hereditary spherocytosis
osmotic fragility (although nonspecific)
51
what is the treatment for hereditary spherocytosis
splenectomy- corrects the anemia and normalizes the RBC survival even though the spherocytes persist
52
what is the most common presentation of spherocytosis in a newborn?
jaundice
53
what is the most common complication of hereditary spherocytosis?
gall stones
54
how is G6PD inherited
X chromosome enzyme
55
what are some oxidants that can trigger G6PD
```
sulfa drugs
nitrofurantoin
dapsone
anti-malarial
fava beans
infection
rasburicase
```
56
what is the treatment for G6PD
supportive
| avoidance of triggers
57
what are the 3 types of autoimmune hemolytic anemia
warm (IgG)
cold (IgM)
biphasic
58
what is the treatment for warm autoimmune hemolytic anemia?
supportive care
transfusions
steroids
59
What is given to moms to prevent hemolytic disease of the newborn
rhogam is given at 28 weeks if Rh-
| also at delivery or if any invasive procedures
60
the severity of Rh incompatibility at the time of birth is best predicted by what?
cord hemoglobin
61
what is the triad for HUS
microwngiopathic hemolytic anemia
thrombocytopenia
renal impairment
caused by Ecoli O157:H7 toxin
62
what is primary hemostasis
platelet plug
63
what is secondary hemostasis
coagulation cascade
64
what factors are involved in the intrinsic pathway
12, 11, 9 and 8
| **primarily 8 and 9
65
what factors are involved in the extrinsic pathway
factor 7
66
what factors are involved in the common pathway
factor 10
factor 2
fibrin (factor 13)
= clot
67
what does plasmin do?
degrades your fibrin clot
68
what can activate plasminogen to plasmin
tPA
| urokinase
69
what are vit k dependent factors
2, 7, 9, 10
| pn c and s
70
what factors do protein c and s work on
5 and 8
71
what does heparin do to antithrombin
binds to antithrombin and forces it to come into close proximity with thrombin
72
what factors is affected by LMWH? UFH?
LMWH: factor 10
UFH: factor 10 and thrombin
73
differences between UFH and LMWH
```
UFH
fast onset and offset
not affected by renal function
can be given IV
cheap
monitor with PTT
```
```
LMWH
more predictable
can be given at home
less frequent monitoring
monitor with anti-Xa
```
74
why do you need bridging when you start warfarin?
because protein c and s have shorter half life so they go down first and you are at increased risk of clotting for the first couple of days
therefore always need bridging with heparin
75
what test should you do if you have an abnormal INR or PTT
mixing study
76
what test should you do if you have a normal platelet count but primary hemostasis problem suspected?
PFA-100 to assess platelet function
77
Ddx for normal INR with elevated PTT
```
factor 8
factor 9
factor 11
vWD
heparin
```
78
Ddx for elevated INR with normal PTT
factor 7
early vit k deficiency
warfarin
early liver disease
79
Ddx for elevated INR and PTT
liver disease
late vit k deficiency
DIC
80
does factor 13 affect INR or PTT
NO!!
therefore if bleeding disorder is strongly suspected in the face of normal coags a facto 13 level should be measured
delayed bleeding, umbilical stump separation
81
what factor affects PTT but is not a bleeding disorder
factor 12
82
what is the dose for oral vitamin k and when should it be given?
2mg PO
| repeated at 2-4 weeks and 6-8 weeks
83
what is the most common inherited bleeding disorder
vonwillebrands disease
84
what does ristocetin test?
vWF function
85
what is the biggest side effect from DDAVP? how does it work?
hyponatremia
therefore hold free water
DDAVP appears to act by causing the release of vWF from the endothelial cells
86
what is the treatment for vWD
```
DDAVP (for type 1 and 2 vWD)
factor VIII-vWF concentrate
OCP and treatment for anemia if needed for menorrhagia
supportive care: avoid NSAIDs, helmet
TXA is useful for mucocutaneous bleeds
```
87
what is type 3 vWD
absence of vWF
88
what is type 1 vWD
quantitative deficiency of normal vWF
89
what is type 2 vWD
qualitative problems with vWF (abnormal function)
90
what is hemophilia A
factor 8 deficiency
91
what is hemophilia B
factor 9 deficiency
92
how is hemophilia inherited
x-linked
| women are carriers but can have the disease with turner,s lyonizaition or homozygosity
93
how is hemophilia classified into mild, moderate and severe
severe <1% factor activity level
moderate: 1-5%
mild: 5-30%
94
what is the most common chronic sequelae of hemophilia
arthritis
95
what is the treatment for hemophilia
prophylactic factor after delivery can be considered
don't forget to give vitamin K- lots of pressure on site
acute treatment- factor should be given
DDAVP can be used for mild/moderate hemophilia A but must have a DDAVP challenge first
96
list 4 clinical presentations of hemophilia in the neonatal period
```
IVH
circumcision bleeding
IM hematoma after IM vit k
large caput or smbgaleal hemorrage
excessive bleeding with phlebotomy
```
97
name one indication for transfusion of FFP
reversal of warfarin
98
when would you transfuse cryoprecipitate
for low fibrinogen (<0.8-1.0)
| for bleeding with vWD or hemophilia ONLY if factor or DDAVP is unavailable
99
what does leukoreduction do?
reduced febrile non-hemolytic reactions
reduces Allo-immunization
reduces viral transmission
100
what does irradiation do
reduced GVHD for immunosuppressed recipients
101
what is the most common fatal reaction associated with transfusion
TRALI and sepsis
102
what is the most common acute reaction
allergy
| then fever
103
what is the most common pathogenic infection associated with transfusions
parvovirus
104
what should you do if there is a transfusion reaction
stop the transfusion
keep IV
send blood and samples to lab
provide supportive care
105
what is the treatment for massive hemorrhage
1 unit of plts and 1 unit of FFP for every 4 units of pRBCs
106
what are some complications following splenectomy
infection- pneumococcal/h influenza, meningococcal
thrombo-embolic complications
pulmonary hypertension
107
List 3 long term effects of cyclophosphamide
pulmonary fibrosis
bladder cancer
Reproductive effects: Gonadal dysfunction, delayed or arrested puberty, premature menopause, germ cell dysfunction or failure, infertility
108
List 4 indications for blood transfusion in sickle cell disease
Acute chest syndrome
Abnormal transcranial dopplers, or acute stroke
Acute splenic sequestration (but only give ½ volume BECAUSE of autotransfusion)
Aplastic crisis
Prior to surgery (for Hb around 100)
Exchange transfusion is reserved for stroke, severe ACS, and pre-op some major surgeries.
Generally, transfusion NOT indicated for vasoocclusive
crisis.
109
Black kid from Kenya very sick with chest symptoms. Hgb is 48- what is underlying dx and 4 things to do (ie. symptoms of acute chest)
1. Pain management
2. supplemental O2
3. antibiotics (ceftriaxone and azithromycin)
4. transfusion
110
Young kid with behaviour change, microcytic, hypochromic anemia with basophilic stippling. Physician has already done iron studies, which were normal. What would you test?
lead levels (basophilic stippling)
111
Newborn with non-hemorrhagic stroke – 4 inheritable etiologies for clot
1. factor V Leiden
2. Pn C deficiency
3. Protein S deficiency
4. Antithrombin deficiency
5. Prothrombin mutation
112
```
A full term newborn develops petechiae and bruising. The baby is otherwise well appearing. On bloodwork his platelets are 12, WBCs are 18 and his Hgb is 140. He is given a platelet transfusion and a repeat platelet count is 16. The mother’s CBC shows platelets of 80. What is the best treatment:
PLA-1 negative platelets
IVIG
Washed maternal platelets
Steroids
```
IVIG
| due to maternal ITP
113
Give 4 mechanisms of anemia in a child with chronic renal disease.
Decreased erythropoietin production (major cause)
- Iron deficiency
- Anemia of chronic disease
- Increased blood loss (dialysis, hematuria, blood tests)
- secondary hyperparathyroidism - can suppress RBC production
114
You are treating a 7-year-old girl with ALL for a
central line line-associated thrombus. Despite
regular increases in her heparin dose you can’t
achieve a therapeutic level. What is a possible
cause?
1. Antithrombin III deficiency
The way heparin works is by binding to thrombin and antithrombin and bringing them together
Antithrombin then antis-the thrombin
2. Protein C deficiency
3. Factor V Leiden
4. Factor VIII deficiency
Antithrombin III deficiency
115
3 year old new Canadian with sickle cell disease comes to you for the first time. List 5 things that are important to do for this child right now.
Immunizations - encapsulated organisms
Penicillin prophylaxis
CBC + smear
Hgb electrophoresis
Educate for pain crisis/risk of infection (management of fever and spleen palp)
Transcranial doppler starting at 2yoa - if high flow may need blood transfusions to keep HbS <30% (decreases stroke risk)
116
what is the main risk from partial exchange transfusion for polycythemia
NEC
117
What is seen on peripheral smear for HUS
Schistocytes
118
10 year old girl with Sickle Cell Disease and a history of stroke. Which treatment is most recommended given her history of stroke?
Hydroxyurea
Transfusion with PRBCs
Folic acid
transfusion with pRBCs
119
what are 3 management strategies for tumor lysis syndrome
1. IVF
2. Rasburicase/ allopurinol
3. Monitor electrolytes q4-6h
120
A 7 year old girl comes to see you with
decreased energy and pallor for the past week.
She had a viral illness 1-2 weeks ago. You notice
that her sclerae are “a bit yellow”. Her CBC show
a Hb 70, WBC 8.6, Platelets 245, 24% retics.
1. What type of anemia is this?
2. What test would you do to confirm?
3. What treatment could you offer?
hemolytic anemia (autoimmune)
DAT
Steroids
121
when do you see Howell-Jolly bodies on smear
asplenia
122
what are two causes of osteomyelitis in sickle cell disease
salmonella and staph aureus
123
what is the treatment for stroke in SCD? How can you prevent a second stroke?
exchange transfusion
| After a stroke, chronic transfusion to maintain Hb S below 30% can prevent a second stroke by 80%.
124
when should transcranial dopplers be performed on patients with sickle cell disease
starting at age 2, annually
| if abnormal then indefinite transfusion
125
what are some risk factors for stroke in sickle cell patients
1. rate of and recent episode of acute chest crisis
2. low baseline hemoglobin
3. previous TIA
4. high blood pressure
5. SS genotype
126
what is required for a diagnosis of acute chest crisis
fever and new infiltrate
| hypoxia is not necessary for the diagnosis
127
what is the treatment for an acute chest crisis
Tx: Antibiotics (including cephalosporin and macrolide), oxygen, hydration, incentive
spirometry, bronchodilators.
Simple transfusion for hypoxia needing oxygen or a Hct<18%.
Exchange transfusion for progressive multilobe infarcts and hypoxia.
Recurrent ACS should have referral to respirology for PFTs.
128
how does hydroxyurea work
Hydroxyurea: raises fetal Hb levels
129
Question. A 14 yo girl with sickle cell disease presents
with a red, swollen, painful hand. (No vitals
mentioned).
What are three things you would do for management?
After leaving the hospital, what is one medication that can
be taken for prevention of these episodes?
1. Admit
2. Pain management
3. IVF
4. antibiotics
5. imaging
6. physio
1. hydroxyurea
130
what are 3 inherited bone marrow failure syndrome
1. dyskeratosis congenita
2. fanconi anemia
3. shwachman-diamond
131
List 5 causes of neonatal thrombocytopenia
1. TAR
2. sepsis
3. TORCH
4. Maternal ITP
5. NAIT
6. NEC
7. RDS
132
what is a treatment option for a baby with low platelets secondary to maternal ITP
IVIG
133
what is the treatment for a baby with NAIT
PLA-1 negative platelets
134
```
Question. What would you advise a child with
hemophilia regarding immunization?
A. Delay Immunization
B. Give factor VIII before immunization
C. Apply pressure for 10 minutes after
immunization
D. Have factor VIII readily available in office to
use if bleed occurs
```
Apply pressure for 10 minutes after immunization
135
What is the probable diagnosis of a 6-year-old child with pancytopenia, short
stature, abnormal thumbs, and areas of hyperpigmentation?
Fanconi anemia
| Diagnosed with chromosomal breakage analysis
