HEME Flashcards
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
liver VOC and acute cholocystisis
if MORE unconjugated then LIVER
if MORE conjugated then cholecystitis
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
Low molecular weight heparin
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
autoimmune hemolytic anemia
AIHA
DAT
steroids
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
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!
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
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
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
ANSWER TEC
not microcytic
all feature of iron def anemia EXCEPT pica koilonychia cheilosis scleral icterus psychomotor reatradation
scleral icterus
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
VERIFY COMPLAINCE
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 - restrict milk 500ml/day
the IRON IS TID, which is too high
microcytic hypochromic anemia
iron deficiency you think
what else to on CBC is consistent
increased RDW
thrombocytosis
MCV/RBC >13 suggestive of iron def (<13 suggest thal trait) – MEISSNER index
pencil cells
most likely diagnosis
Hb A –> none, Hb A2 –> 2%
Hb F is 75% Hb S is 25%
sickle cell
sickle cell disease
alpha thal
sickle cell disease
as YOU HAVE NO Hb A
does not matter the Hb F
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
hgb100 MCV 60 RBC 4.8
HIGH RBC, and low MCV
and its minor
Meisner 12.5
want it be,low 13
number one cause of complications in beta thal major?
iron overload
megaoloblastic anemia
cardiomyopathy
hematopoeisis
iron overload
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
hm electophoresis
likely has thal
what is basophilic stippling
lead poisoining - esp coarse
common presentation of sphreocytosis in a NEWBORN
a) jaundice
b) splenomegaly
c) reticulocytosis
JAUNDICE
MOST COMMON complicationn of herediatry spherocytosis? frontal bossing gallstones reticulocytes aplastic anemia (can happen) splenic infarct
gallstones
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
g6pd - has basket CELL
more common in boys
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 -
ABO
Question: A child is currently on goat’s milk. What do you recommend supplementing with?
Folic acid
Iron
Multivitamin
Vitamin B12
folic acid
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
fetal-metarnal hem
as he is pale
The severity of Rh incompatability at the time of birth is best predicted by Cord hemoglobin Cord bilirubin Prematurity Splenomegaly Maternal anti-D antibody
CORD hemoglobin
sickler indications for transfusion
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%.
psychosocial issues with sicklers
pain
neurodevelopmental abnomralities
Transfusion risks
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.
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
cholecystiectomy
. 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
stroke
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
inc lethargy
recent poor school performance
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
ACS
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?
Tx: Admit, Fluids, Pain control, reassurance, physio, heat/relaxation, etc.
(NOT antibiotics, unless fever. Transfusions usually not necessary.)
Hydroxyurea (especially if recurrent episodes))
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?
Viral suppression.
Absolute neutrophil count.
Which two conditions cause pancreatic insufficiency?
CF
Shwachman-Diamond Syndrome
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
IVIG
mother platelet low so this is ITP
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…
PLA1 negative platelet
Question: In which of the following is a platelet transfusion indicated? Aplastic anemia Acute ITP Chronic ITP Hemolytic uremic syndrome ITP secondary to quinidinee
aplastic anemia
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 CNS bleed is the worst complication
: 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
ANC <1000
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?
Low white cell count Low hemoglobin Blasts on the peripheral smear Lymphadenopathy Hepatosplenomegaly
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
Antithrombin III deficiency
heparin binds to antithrombin and makes it come close to THROMBIN
Which of the following is most consistent with DIC?
Increased fibrinogen Increased PTT Decreased PT Decreased INR Decreased fibrin-split products
inc PTT
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
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
Which of the following predisposes to early hemorrhagic disease of the newborn?
Breastfeeding Prematurity Cystic fibrosis Maternal phenytoin Diarrhea
phenytoin
. A vWD patient presents with bleeding. What is the best treatment? Frozen plasma DDAVP Cryoprecipitate Platelets
cryo
most cases its DDAVP but highest concentrate of vWF is cryo
DDAVP ONLY WORKS with TYPE 1
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
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
. 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
hemophilia
. List 4 clinical presentations of hemophilia in the neonatal period.
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
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
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
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
Apply pressure for 10 minutes after immunization
Most cases it is DDAVP; highest vWF concentration found in cryo
Question. Transient fever in blood transfusions is most commonly a result of Sensitization to WBC antigens Acute GVHD Hepatitis C Hemolysis Bacterial contamination
acute gvhd
What is the advantage of using leukoreduced transfusions?
Decreases hemolytic reactions
Decreases infectious complications
Decreases febrile transfusion reactions
dec infection
esp CMV
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
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).
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%
50%
Child getting large volumes of PRBC transfusion. What ECG complication do you expect to see?
Peaked T-waves
U waves
Short PR interval
peak T
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
BMA
Baby with petichiae. Plt 12. After transfusion, Plt are 16. Mom’s CBC normal. What is best management?
PLA1 negative platelets
IVIg
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.
Newborn with trisomy 21. High WBC, thrombocytopenia, anemia, HSM, petechiae. What does he have? TORCH infection Sepsis Transient abnormal myelopoiesis HSV infection
TAM - transient abn myelpoieiss
could have torch
Kid with Burkitt’s and TLS. Urine pH 7.0. What’s next.
- Hemodialysis.
- Potassium
- ACE inhibitor
- Rasburicase
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.
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?
- Neuroblastoma
- Langerhans cell histiocytosis
- ALL
- Ewing Sarcoma
LCH
A Child has a distended abdomen. An ultrasound shows an adrenal mass and hepatomegaly. Which of the following tests will make the diagnosis?
- MIBG Scan
- Serum AFP
- Urine HVA/VMA
- Abdo/Chest CT
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.