HEME Flashcards

1
Q
Teenage sickler, sexually active with prev transfusions
pale, icteric with abdo pain
RUQ tender no guard, HGB 70. AST 60 ALt 55 and bili elevated
Diagnosis
a) liver VOC
b) acute cholecystititis
c) hemochromatosis
d) viral hepatitis
e) fitz hugh curtis
A

liver VOC and acute cholocystisis

if MORE unconjugated then LIVER
if MORE conjugated then cholecystitis

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2
Q

An adolescent female presents to the ED with history of left leg swelling and pain. She was recently started on an OCP for her acne. In the ED she develops sudden chest pain and respiratory distress. What is your next step in management?

a) Warfarin
b) Surgical thrombolectomy
c) TPA
d) Low molecular weight heparin

A

Low molecular weight heparin

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3
Q

7 yo with dec energy and pallor
viral illness 2 weeks ago with yellow sclerae
CBC HGB 70 WBC 8.6 PLT 245 and high retic
Anemia type
Test to confirm
tx

A

autoimmune hemolytic anemia
AIHA
DAT
steroids

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4
Q

7 yo boy, previously well. Post tonsillectomy bleeding. Increased PTT, normal platelets, normal factor levels. What do you do?

a. Amicar (aminocaproic acid)
b. DDAVP
c. factor VIII
d. Cryoprecipitate

A

AMICAR

a. Amicar (aminocaproic acid) - similar to tranexemic acid (MILD BLEEDS) - best as per lecture
b. DDAVP (only if you know type 1 and a responder)
c. factor VIII
d. Cryoprecipitate (IF SEVERE BLEEDING)

*If you know diagnosis is definitely vWD and option for vWF concentrate is there, choose that!

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5
Q

You have a kid with spherocytosis, you get the results and the kid is slightly anemic and evidence of spherocytes on peripheral smear
a. What are two tests you can do to confirm hereditary spherocytosis

b. What are two indications for splenectomy in this patient

A

flow cytometric EMA (eosin-5-maleimide) binding test
cryohemolysis test
*Osmotic fragility is not specific for HS

Severe HS
Moderate HS and frequent hypoplastic or aplastic crises, poor growth, or cardiomegaly

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6
Q

2yo boy wiht pallor
1L milk
Hgb 49, MCV 80, RDW 14%, smear normal
HbA and HbS whats the condition

A) TEC
B) Iron def
C) sickle cell

A

ANSWER TEC

not microcytic

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7
Q
all feature of iron def anemia EXCEPT
pica
koilonychia
cheilosis
scleral icterus
psychomotor reatradation
A

scleral icterus

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8
Q

9 month ex 32 weeker, 40 ounces homo milk
hb 60, MCV 50, tx with iron x 1 month and rpt hb 52, MCV 50 and retic 1% - what do you do
BMA
inc dose
verify complioance
hgb elecrtophoresis
jejenual biopsy

A

VERIFY COMPLAINCE

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9
Q
infant get 5 bottles of cow per milk
hgb 40, wiegh 13kg, vital stable
a_ restrict milk to 500ml/day
b_ start iron 60 mg po TID
c_ blood transfusion
A

A - restrict milk 500ml/day

the IRON IS TID, which is too high

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10
Q

microcytic hypochromic anemia
iron deficiency you think
what else to on CBC is consistent

A

increased RDW
thrombocytosis
MCV/RBC >13 suggestive of iron def (<13 suggest thal trait) – MEISSNER index
pencil cells

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11
Q

most likely diagnosis
Hb A –> none, Hb A2 –> 2%
Hb F is 75% Hb S is 25%

sickle cell
sickle cell disease
alpha thal

A

sickle cell disease
as YOU HAVE NO Hb A

does not matter the Hb F

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12
Q
which following most liekly to be seen with thal MINOR
a hgb 100, mcv 75 RBC 4.25
b  hgb 100 MCV 60 RBC 4.8
c hgb 60 MCV 75 RBC 2.5
d hgb 60 MCV 60 RBC 2.5
A

hgb100 MCV 60 RBC 4.8

HIGH RBC, and low MCV
and its minor
Meisner 12.5
want it be,low 13

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13
Q

number one cause of complications in beta thal major?

iron overload
megaoloblastic anemia
cardiomyopathy
hematopoeisis

A

iron overload

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14
Q

8yo fatigue. bloodwork shows hgb 80 MCV 50, ferittin 150ug/l, serum irn 50umol/l
what is next step

barium meal
osmotic gragility test
abdo Tc99 scin
hgb electophoresis
BMA
A

hm electophoresis

likely has thal

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15
Q

what is basophilic stippling

A

lead poisoining - esp coarse

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16
Q

common presentation of sphreocytosis in a NEWBORN

a) jaundice
b) splenomegaly
c) reticulocytosis

A

JAUNDICE

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17
Q
MOST COMMON complicationn of herediatry spherocytosis?
frontal bossing
gallstones
reticulocytes
aplastic anemia (can happen)
splenic infarct
A

gallstones

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18
Q

1 w old chinese boy, 1 day jaundice, bili indirect 270, hgb 95,
mom is A+ and he is B+ and he looks well

sepsis
thalasemia
g6pd
ABO incompat

A

g6pd - has basket CELL

more common in boys

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19
Q

n Asian baby presents at 12 hours of life with jaundice. Mother is O+, the baby is A+. The bilirubin is 200. What is the diagnosis?

a. ABO incompatibility
b. G6PD
c. Rh incompatibility -
d. Physiological jaundice -

A

ABO

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20
Q

Question: A child is currently on goat’s milk. What do you recommend supplementing with?

Folic acid

Iron

Multivitamin

Vitamin B12

A

folic acid

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21
Q

Question. A fullterm baby is delivered to an O+ mom. He looks well but is pale. Hb is 70. He is hemodynamically stable. What is the most likely diagnosis?

ABO incompatability

Chronic fetal-maternal hemorrhage

Rh incompatability

G6PD Deficiency

A

fetal-metarnal hem

as he is pale

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22
Q
The severity of Rh incompatability at the time of birth is best predicted by
Cord hemoglobin
Cord bilirubin
Prematurity
Splenomegaly
Maternal anti-D antibody
A

CORD hemoglobin

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23
Q

sickler indications for transfusion

A
Aplastic crisis
Splenic sequestration
Pre-op (high risk surgeries)
Stroke – if low Hb, awaiting exchange
ACS – if low Hb, awaiting exchange
Also used chronically to prevent stroke by keeping Hb S under 30%.
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24
Q

psychosocial issues with sicklers

A

pain

neurodevelopmental abnomralities

25
Q

Transfusion risks

A
Transfusion risks:
Iron overload
Alloimmunization
Potential infections
Final Hct after a transfusion should not exceed 30%, or else the blood can become too viscous. Blood should have extended crossmatching (C,D, E, Kell).  

After 1 year of monthly transfusions, iron overload usually starts. At that time, regular iron chelation with deferoxamine (Desferal) or deferasirox (Exjade) should be started.

26
Q

A 13 year old, obese patient with Hb SS presents with RUQ pain, tenderness, guarding, fever and jaundice. Ultrasound shows multiple gallstones, dilated CBD, and inflamed walls. You diagnose acute cholecystitis and admit for hydration and triple antibiotics. After the acute process is treated, what do you suggest?

Transfusion therapy
Ursodiol
Cholecystectomy
Nothing

A

cholecystiectomy

27
Q

. A 10 year old boy with sickle cell anemia has multiple episodes of acute chest crises. Which of the following complications does this place him at increased risk for?

Stroke
Gall stones
Nephropathy

A

stroke

28
Q

In which of the following situations should you investigate a child with sickle cell anemia for possible stroke?

Increasing lethargy
Recent poor school performance
Acute worsening of anemia

A

inc lethargy

recent poor school performance

29
Q

A 3 year old child with sickle cell disease presents with fever, cough, tachypnea, and looking unwell. What do you need to rule out first?

Acute chest crisis
Pulmonary Embolus
Pneumonia
Asthma

A

ACS

30
Q

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?

A

Tx: Admit, Fluids, Pain control, reassurance, physio, heat/relaxation, etc.
(NOT antibiotics, unless fever. Transfusions usually not necessary.)
Hydroxyurea (especially if recurrent episodes))

31
Q

A 4 year old child comes to your Emergency department with a history of a fever for 3 days. You do a CBC and find that the WBC count is low at 3.2. What is the most common reason for this clinical scenario?
On a differential, which cell line, if low, increases the risk of serious infection?

A

Viral suppression.

Absolute neutrophil count.

32
Q

Which two conditions cause pancreatic insufficiency?

A

CF

Shwachman-Diamond Syndrome

33
Q

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?

Random donor platelets
PLA-1 negative platelets
IVIG
Washed maternal platelets

A

IVIG

mother platelet low so this is ITP

34
Q

Question. baby with plts 16 at birth, mother 280. plts still 16 after plt transfusion, next best step:

IVIG
Observe
PLA-1-negative plts
DIC work-up…

A

PLA1 negative platelet

35
Q
Question: In which of the following is a platelet transfusion indicated?
Aplastic anemia
Acute ITP
Chronic ITP
Hemolytic uremic syndrome
ITP secondary to quinidinee
A

aplastic anemia

36
Q

Which of the following is true about ITP?
There is often splenomegaly
There is no proven link to a preceding viral infection
A CNS bleed is the worst complication

A

A CNS bleed is the worst complication

37
Q

: Which of the following is an indication for a bone marrow aspiration or biopsy in a child with ITP who has platelets of 12,000?

Absolute neutrophil count under 1000
Previous use of steroids
Hemoglobin under 115
Fever over 39

A

ANC <1000

38
Q

You are working in an Emergency department and a mother brings in a 2 year old girl with a petechial rash noted that morning while changing her diaper. She is otherwise well-appearing. You suspect a diagnosis of ITP. What are 4 indications for a bone marrow aspiration in the setting of a probable diagnosis of ITP?

A
Low white cell count
Low hemoglobin
Blasts on the peripheral smear
Lymphadenopathy
Hepatosplenomegaly
39
Q
You are treating a 7-year-old girl with ALL for a central line-associated thrombus. Despite regular increases in her heparin dose you can’t achieve a therapeutic level. What is a possible cause?
Antithrombin III deficiency
Protein C deficiency
Factor V Leiden
Factor VIII deficiency
A

Antithrombin III deficiency

heparin binds to antithrombin and makes it come close to THROMBIN

40
Q

Which of the following is most consistent with DIC?

Increased fibrinogen
Increased PTT
Decreased PT
Decreased INR
Decreased fibrin-split products
A

inc PTT

41
Q
A patient is being assessed for a bleeding diathesis. Labs show PT 12 s, PTT 50 s, Bleeding time 5 min, and plts of 250. What is the most likely diagnosis?
Hemophilia
von Willebrand disease
Vitamin K deficiency
Factor V deficiency
Factor X deficiency
A

Hemophilia
von Willebrand disease (Marta thinks it’s this)

PT normal 10-14
Bleeding time is usually a marker of primary hemostasis – plt function (but has been documented to be prolonged in hemophilia patients)
B - vWD is most likely
FV and FX deficiency very rare

42
Q

Which of the following predisposes to early hemorrhagic disease of the newborn?

Breastfeeding
Prematurity
Cystic fibrosis
Maternal phenytoin
Diarrhea
A

phenytoin

43
Q
.   A vWD patient presents with bleeding. What is the best treatment?
Frozen plasma
DDAVP
Cryoprecipitate
Platelets
A

cryo

most cases its DDAVP but highest concentrate of vWF is cryo

DDAVP ONLY WORKS with TYPE 1

44
Q
A baby is born precipitously at home to parents who both have type 3 von Willebrand disease. The baby has an intraventricular hemorrhage. Which of the following is the most appropriate treatment?
Cryoprecipitate
FFP
Factor VIII/vWF concentrate
DDAVP
A

Factor VIII/vWF concentrate

type 3 (AR): absolute deficiency of vWF (rare), severe bleeding, treat with vWF concentrates

cryo - has fibrinogen, factor 8, factr 18, vwF - usuaslly for hemophilia

FFP has less of these

45
Q

. A 6-year-old Chinese boy fell off his bike 24 hours ago. He is brought into the ER with right knee swelling and a large yellow bruise on his anterior chest wall. What do you worry most about?

Hemophilia    
Thrombocytopenia
Child abuse     
Herbal remedies
But should we al
A

hemophilia

46
Q

. List 4 clinical presentations of hemophilia in the neonatal period.

A
Intraventricular hemorrhage
Circumcision bleeding 
IM hematoma after vit K injection 
Bleeding at the umbilical stumb 
Large caput or subgaleal bleed
Excessive bleeding with phlebotomy
(Hemarthrosis and deep muscle bleeding are
47
Q

You are seeing a pregnant woman during her first pregnancy. Her father has hemophilia A. Regarding the risk of her transmitting the disorder to her own children, you tell her:
None of her children will have it
All of her sons will have it
50% of her sons will have it, and all of her daughters will be carriers
50% of her sons will have it, and 50% of her daughters will be carriers

A

50% of her sons will have it, and 50% of her daughters will be carriers

Father is XY – had to transmit abnormal X allele hence mother is obligate carrier; 50% chance her sons will have it and 50% of daughters are carriers

48
Q

What would you advise a child with hemophilia regarding immunization?
Delay Immunization
Give factor VIII before immunization
Apply pressure for 10 minutes after immunization
Have factor VIII readily available in office to use if bleed occurs

A

Apply pressure for 10 minutes after immunization

Most cases it is DDAVP; highest vWF concentration found in cryo

49
Q
Question.   Transient fever in blood transfusions is most commonly a result of
Sensitization to WBC antigens
Acute GVHD
Hepatitis C
Hemolysis
Bacterial contamination
A

acute gvhd

50
Q

What is the advantage of using leukoreduced transfusions?
Decreases hemolytic reactions
Decreases infectious complications
Decreases febrile transfusion reactions

A

dec infection

esp CMV

51
Q

A child is receiving a blood transfusion. She becomes febrile and develops chills. What is your first step in management?
Continue with transfusion, give methylprednisolone
Stop transfusion, give steroids
Stop transfusion, run IV TKVO
Continue transfusion and slow rate

A

stop transfusion and IV TKVO

Management of febrile transfusion reactions:
Stop the transfusion, maintain IV access, evaluate the situation, examine, notify blood bank.
If not serious, give tylenol, continue cautiously.
If unwell, do not restart transfusion. Check ABO and cultures. Supportive care as required (benadryl, steroids, epi, fluids, antibiotics, demerol, oxygen, diuresis, etc).

52
Q
A dad has Hemophilia A. The mom is a carrier. If they have a daughter, what are the chances she will have Hemophilia A disease?
0%
25%
50%
100%
A

50%

53
Q

Child getting large volumes of PRBC transfusion. What ECG complication do you expect to see?
Peaked T-waves
U waves
Short PR interval

A

peak T

54
Q
A 3 year old girl presents with 7 day history of fever. She has bony pain but no arthritis on exam, but she has HSM and diffuse generalized lymphadenopathy. Her bloodwork shows WBC 18, Plt 110 and anemia. What is the next best step?
Bone Marrow Aspirate
EBV Serology 
Work up for systemic JIA
Lymph node biopsy
A

BMA

55
Q

Baby with petichiae. Plt 12. After transfusion, Plt are 16. Mom’s CBC normal. What is best management?
PLA1 negative platelets
IVIg

A

PLA
best answer is PLA1- platelets. The “textbook” answer would be washed maternal platelets but those are practically very hard to get a hold of. IVIG is also effective but much less so and wouldn’t be your first choice on an exam.

56
Q
Newborn with trisomy 21. High WBC, thrombocytopenia, anemia, HSM, petechiae. What does he have?
TORCH infection
Sepsis
Transient abnormal myelopoiesis
HSV infection
A

TAM - transient abn myelpoieiss

could have torch

57
Q

Kid with Burkitt’s and TLS. Urine pH 7.0. What’s next.

  1. Hemodialysis.
  2. Potassium
  3. ACE inhibitor
  4. Rasburicase
A

rasbirucase

I imagine rasburicase is the right answer. However, that only works for increased urate so if the main issue is hyperphosphatemia or runaway hyperkalemia then you will need hemodialysis. ACE inhibitor plays no role and potassium is definitely not good in most TLS cases.

58
Q

An 11 month old presents with a scaly rash all over, especially in the diaper area. He also has exopthalmos and HSM. Xrays show bony lucencies on the scalp. What is the likely diagnosis?

  1. Neuroblastoma
  2. Langerhans cell histiocytosis
  3. ALL
  4. Ewing Sarcoma
A

LCH

59
Q

A Child has a distended abdomen. An ultrasound shows an adrenal mass and hepatomegaly. Which of the following tests will make the diagnosis?

  1. MIBG Scan
  2. Serum AFP
  3. Urine HVA/VMA
  4. Abdo/Chest CT
A

urine HVA/VMA

MIBG scan will help for staging but is not the diagnostic test of choice. Ditto for CT abdo/pelvis. AFP is diagnostic for hepatoblastoma but the adrenal mass points to neuroblastoma.