Heme-Onc Flashcards
A boy with Hemophilia A who is managed at home with recombinant Factor VIII presents with increasing episodes of bleeding and hemarthrosis of the ankle despite medication compliance. Your next step:
a. Check FVIII and inhibitor levels
b. Add DDAVP to the current regime
c. Try another FVIII product
d. Manage ankle with NSAIDs and rest
Check FVIII and inhibitor levels
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? a) 0% b) 25% c) 50% d) 100% -------- A man with hemophilia A marries a women who is a carrier of hemophilia A. Assuming they have a daughter, what is the likelihood of having a child with hemophilia A? a) 0% b) 25% c) 50% d) 100%
50%
X-linked recessive (think all the inbreeding in monarchy)
A baby is born precipitously at home to parents who both have type 3 von Willebrand disease. The baby is found to have an intraventricular hemorrhage. Which of the following would be the most appropriate treatment?
a) Cryoprecipitate
b) FFP
c) Factor VIII/vWF concentrate
d) DdAVP
Factor VIII + vWF concentrates
Type 3 vWD: no vWF vWF:Ag absent vWF:RCo absent F8: low Multimer distribution: absent
FFP: contains all coagulation factors
- Good for deficiencies in Facto 2, 5, 10, 11
- Not recommended if known severe hemo A/B, vWD, or factor 7 deficiency b/c safer factor specific concentrates are available
Cryoprecipitate: Factor 1 (fibrinogen), 8, 13, vWF
- Not recommended for vWF b/c risk of viral transmission
DDAVP is used for type 1 vWF
type 2+3 vWF need vWF containing concentrates
A newborn baby is bleeding. How do you differentiate DIC from hemorrhagic disease of the newborn due to vitamin K deficiency?
a) Low platelets
b) Low PTT
c) High PT
d) High fibrinogen
Low plts only in DIC
High PT in both
Fibrinogen low in DIC, normal in HDN
DIC
- consumption of coagulation factors
- high INR + PTT
- low pts, low fibrinogen, elevated D dimer
- blood smear: fragments, schistocytes
- Tx:
1) Treat underlying cause
2) Correct shock, acidosis, hypoxia
3) If severe bleeding, consider - plts for thrombocytopenia,
- cryo for hypofibrinogenemia,
- FFP for loss of factors
HDN - Vit K dependent factors 10, 9, 7, 2 C+S - 7 is extrinsic = high PT/INR - 9 is intrinsic = high PTT - 10, 2 are common - Normal plts - Tx: give vit K IM
You are treating a 7 year old girl with ALL for a clot from her central line. Despite increasing doses of heparin you are not getting a therapeutic level. What is the cause for this?
a) Antithrombin III deficiency
b) Protein C deficiency
c) Factor V Leiden
d) Factor VIII deficiency
Antithrombin III deficiency
Heparin binds to ATIII, which is an enzyme inhibitor -> causes a conformational change -> activated AT -> inactivates FIIa (thrombin) + FXa -> prevents fibrin clot
If ATIII is low, then heparin is not effective
- inherited or acquired (impaired proD’n (liver disease, warfarin), prot loss (nephrotic syndrome), consumption (DIC, thrombosis)
- Increased thrombotic risk
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 thrombectomy
c) TPA
d) Low molecular weight heparin
LMWH
Anticoagulation is first line therapy for PE/DVT
For acute phase, use LMWH or UFH
A child with known hemophilia comes for routine immunizations. What would you advise with immunizations?
a) Delay them
b) Give Factor VIII before immunizations
c) Apply pressure for 10 minutes
———————–
2 month old baby had IVH bleed, diagnosed with hemophilia A. What is best management/advice regarding immunizations?
a) Postpone 2 month vaccination
b) Give IM vaccine and put pressure x 10 minutes
c) Give factor VIII before giving IM vaccine
———————
Immunizations in kid with hemophilia A:
Defer 2 mos shot
a) Give Factor 8
b) Pressure over site x10min post-immunization
Apply pressure for 10min
Ideally vaccines should be given SC if possible with the smallest gauge needle
15 year old girl with periods for a couple of years. Epistaxis and menorrhagia. What to check for?
a) Von Willebrand’s
b) Hemophilia C
c) Factor V Leiden mutation
VWD
A 3 y.o. Chinese boy sees you in the emergency room after falling off his bicycle 24 hours ago. On exam, you see a swollen right knee and a yellow bruise on the anterior chest. What would explain this? a) Chinese herbal remedies b) Hemophilia c) Child abuse d) Von Willebrand disease -------------------- 6-year Chinese boy presents after falling off his bike with a single bruise on his chest and a swollen, painful left knee. What is the most likely diagnosis? a) ITP b) Child abuse c) Hemophilia --------------------- 6 you Chinese boy presents with multiple bruises of various ages after falling off his bike. What is the most likely diagnosis? a) ITP b) Child abuse c) Hemophilia
Hemophilia
Hemarthrosis suggests secondary hemostasis
= F8/F9 deficiency, vWD
A young boy presents with a normochromic, normocytic anemia. He is stable. What is your next Course of action?
a) Start iron supplementation
b) Refer to heme for BMA
c) Follow up in 2 weeks
Follow up in 2 weeks
How severe is the anemia?
Would want reticulocyte count
WBC, Plts
Most likely TEC
7 year old Child with anemia hgb 40, BMA shows arrested erythroid precursors. Cause?
a) TEC
——————-
2 yo, Hb 50, reticulocytes 1%. Bone marrow, no active precursors. Dx?
a. aplastic anemia
b. ALL
c. Transient erythroblastopenia of childhood
——————-
7 year old with severe anemia. BM shows arrest of erythroid precursors. Dx?
a) Aplastic anemia
b) TEC
TEC = transient erythroblastoPENIA of childhood
- Most common ACQUIRED red cell aplasia
- 6mo - 3yo
- often after viral illness
- severe, transient normocytic anemia + reticulocytopenia
- 20% with neutropenia
- plts N/high
Tx
- recover in 1-2mo
- pRBC for severe anemia but if >1 transfusion, consider alt Dx
- no use for steroids
18mo child with normocytic anemia (normal platelets, WBC). Most common diagnosis: a) Transient erythroblastopenia -------------- 4y boy who presents with pallor after a viral illness. Hgb 88, retics 0. WBC and platelets normal. What is the most likely diagnosis? a) TEC b) ALL c) Diamond-Blackfan anemia d) Iron deficiency anemia
Transient erythroblastopenia of childhood
- most common ACQUIRED red cell dysplasia
- 6mo-3yo
- peak conincides with IDA
- usually after viral illness
- transient, severe normocytic anemia + low retics
- 20% have neutropenia
- plts N/high
- Normal RBC adenosine deaminase levels (vs high in Diamond blackfan)
Tx
- recover in 1-2mo
- pRBC for severe anemia, but if >1 transfusion, consider alt Dx
- no use for steroids
Note: normocytic anemia with low retics could be diamond blackfan, but would expect dysmorphisms
Describes kid with HUS; what do you see on smear
a) Schistocytes
b) Megakaryocytes
c) Reticulocytes
HUS
- microvascular injury with endothelial damage
1. MAHA
2. Renal insufficiency
3. Thrombocytopenia
Kid with what sounds like diamond blackfan anemia. What is most commonly associated?
a) Short thumb
b) Dysmorphic facies
Dysmorphic features
- Macrocytic anemia, low retics
- Plt + WBC normal
- Normal B12/folate
- short stature, wide-set eyes, snub nose, thick upper lip, triphalangeal thumbs
- AML + MDS
Abnormal thumbs, anemia, no thrombocytopenia. Other findings?
a) Absent radius
b) Craniofacial abnormalities
Craniofacial abnormalities
Diamond blackfan
- dysmorphic features: hypertelorism, triphalageal thumb
- macrocytic/normocytic anemia, low retics
- normal WBC + plts
- Normal chromosomal breakage analysis (unlike Fanconi anemia)
TAR = thrombocytopenia + absent radium
- thumb present
- normal WBC + Hb
Fanconi anemia - AR - absent radius + abN thumb - CAL - hypogonadism - pancytopenia - HL, Cardia, GI, Renal Dx: chromosomal breakage analysis of blood lymphocytes, skin fibroblasts, amniotic fluid cells, or chorionic villus Bx
Kid with high intake of milk. CBC reveals normocytic anemia with Hgb AS. Most likely? a. TEC b. FE def c. Sickle Cell Dx --------------------- 2 yo child who comes in with Hb \_\_ g/L (low). drinks 1L cows milk a day. Labs – MCV 80, no hemolysis. Smear normal. Hb electrophoresis – AS. Diagnosis: a. nutritional iron deficiency anemia b. sickle cell disease --------------------- 18mos with increasing pallor and previously well. Drinks 1L of milk daily. Lab studies show normal WBC and platelets, Hgb 50, MCV 80, normal smear, Hb electrophoresis: AS. a) Iron deficiency anemia b) Sickle cell disease c) TEC d) ALL
TEC
Normocytic anemia
MCV normal 80-96
HgbAS is a benign carrier trait, not a disease. Cannot be diagnosed with CBC b/c usually normal
IDA is microcytic anemia. Would expect hypochromic microcytic smear with elliptocytes
ALL would expect pancytopenia and blasts on smear
2 year old presents with progressive pallor over the last few weeks. You discover his Hb is 48, with a reticulocyte count of 1%. He drinks 1 L of homo milk a day. Because he has no risk factors on history or physical, you do some blood work which shows the following: MCV 80 HB electrophoresis: AS Blood smear: normal. What is the most likely diagnosis?
a) Sickle cell disease
b) Congenital red cell aplasia
c) Transient erythroblastopenia of childhood
TEC
A 9 month old ex-32 weeker takes 40 oz of homo milk per day. Hgb is 60, MCV 50. She is treated with Fe 4 mg/kg/day for one month. On repeat testing, her Hgb is 62, MCV is 50, and her retics are 0.01. What to do next? a) BMA b) Verify compliance c) Hb electrophoresis d) Jejunal biopsy --------------------- Child with iron deficiency anemia, started on iron. Hb now 64, retic count given. What next: a) Verify compliance b) BMA c) Hb electrophoresis
DDx for microcytic anemia that doesn’t respond to oral iron (6)
Verify compliance
DDx for microcytic anemia that doesn’t respond to oral iron
- Poor compliance
- Incorrect dose or medication
- Malabsorption of administered iron
- Ongoing blood loss
- Concurrent vit B12 or folate deficiency
- Wrong Dx: TAILS
Which of the following would most likely be seen with iron deficiency?
a) pica
Pica (eating non-nutritive substances)
18 month old male (13kg) with history of excessive milk intake presents on routine physical exam with pallor Subsequent blood work reveals Hb 45, MCV 56. What would be the best management? a) Limit milk to 500 cc/day b) Start elemental iron 60mg PO tid c) Give PRBC transfusion 130cc d) Change to protein hydrosylate formula ------------------------- Hb 48 in an 11 m.o. M with tachycardia, pallor, excess milk intake. a) Restrict milk intake to < 500 cc/day b) Fe TID at 15 mg/kg c) Transfuse 10 cc/kg blood d) Change to protein hydrolysate formula
Limit milk vs iron (but the volumes + doses are wrong)
Limit milk to 720mL/d
Iron 3-6mg/kg/d div TID
Transfuse only in severe cases (hemodynamically unstable, CHF, ongoing blood loss)
An otherwise healthy girl presents with pallor. Her iron, and ferritin levels are normal. Her bloodwork shows a Hb of 70 with an MCV of 50. What study is most likely to give you her diagnosis?
a) Bone marrow aspirate
b) Hemoglobin electrophoresis
Hemoglobin electrophoresis
Microcytic anemia T - thalassemia A - anemia of chronic disease I - IDA L - lead S - sideroblastic anemia
Schwachman diamond baby. Which vitamin level would be normal?
a) Vit A
b) Vit E
c) Vit B12
d) Vit D
Vit B12
Schwachman diamond Pancreatic insufficiency - fat soluble vit ADEK Pancytopenia - neutropenia > anemia > thrombocytopenia - Hirschprung + imperforate anus
2 year with normal history but found to be pale on exam. Labs show normocytic normochromic anemia. Smear normal. What next test? a) Bone marrow b) Osmotic fragility c) Ferritin d) Hg electrophoresis -------------------------------------- 18 mo found to be pale on normal routine exam. Blood work shows Hb 80 (normocytic and normochromic), retics low, normal WBC and platelets. What additional test would you do? a) Ferritin b) Coombs c) Bone marrow d) Hb electrophoresis
What is the DDx for normocytic anemia?
Bone marrow to r/o bone marrow failure problems (aplastic anemia, malignancy)
An unexplained normocytic anemia with inadequate retic response should prompt a BM exam
Normochromic anemia A. Increased destruction or loss of RBC -> high retics 1. Hemolysis 2. Hypersplenism 3. Chronic occult bleeding
B. Failure of RBC production in context of primary or secondary BM failure -> low retics C - anemia of chronic disease R - renal failure (EPO deficiency) A - aplastic anemia (usu bi or pancytopenia) M - malignancy T - TEC I - infection M - meds E - endocrinopathies
A 10 year old with sickle cell anemia presents with fever and respiratory distress. On CXR there is a new infiltrate. Your next most important step: a) Order type and screen and transfuse b) IV antibiotics c) IV hydration at 2x maintenance d) Start hydroxyurea ------------------------ Child with sickle cell disease presenting with fever and respiratory symptoms and a change on X-ray. Management? a) Start 2x maintenance IV fluids b) Antibiotics c) Transfuse pRBCs ------------------------- A child with sickle cell disease presents with fever and shortness of breath. He also has abdominal pain. He is admitted and IV access is obtained. What would be the MOST appropriate next step? a) IV antibiotics b) Cross and type and blood transfusion c) Hydration at 2 times maintenance d) Ultrasound
Cross and type and blood transfusion
- only method to abort a rapidly progressing episode of ACS
- ABx would not treat the resp distress
3 yo Sickle cell, with fever cough, tachypnea, unwell. What do you need to rule out 1st?
a) Acute chest crisis
b) PE
c) Pneumonia
d) Asthma
Acute chest crisis
New radiodensity on CXR with any 2 of the following:
- Fever
- Resp distress
- Cough
- Hypoxia
- Chest pain
Causes
- infection (most common): S pneuo, mycoplasma, chlamydia
- fat emboli
2 yo girl from Mediterranean background. What values are most representative of thalassemia minor? a) Hgb 100 MCV 75 RBC 2.61 b) Hgb 100 MCV 60 RBC 4.81 c) Hgb 80 MCV 75 RBC 2.81 d) Hgb 80 MCV 60 RBC 3.21 -------------------- What picture is most consistent with thalassemia minor? a) Hb 100 MCV80 RBC 2.81 b) Hb 100 MCV 60 RBC 4.81 c) Hb 60 MCV 80 RBC 4.81 d) Hb 60 MCV 60 RBC 3.71 --------------------- 4-year old of Mediterranean descent. Lab findings most consistent with thalassemia minor? a) Hb 100 MCV 60 RBC 4.8 b) Hb 100 MCV 75 RBC 4.3 c) Hb 80 MCV 75 RBC 3.3 d) Hb 60 MCV 50 RBC 2.2
Hgb 100 MCV 60 RBC 4.81
Microcytic anemia
MCV/RBC <13
Given this haemoglobin electrophoresis, what is the most likely diagnosis?
HbA - none
HbA2 - 2%
HbF- 75%
HbS - 25%
a) Sickle cell trait
b) Sickle cell disease
c) Beta thalassemia
d) Alpha thalassemia
Sickle cell disease
Broad umbrella term. Includes sickle cell anemia and compound heterozygotes HbSC, SB-thal
Electrophoresis suggests HbS-B0
Sickler with dactylitis, Hgb 95, which will be associated with worse prognosis
a) Anemia
b) Dactylitis
Dactylitis
Child with history of multiple acute chest syndrome episodes is at most risk for which of the following:
a) Stroke
b) Gallstones
c) Asthma
d) Priapism
Stroke
SCD patient: which of the following would be most suggestive of stroke? a) Fatigue b) Sudden decrease in Hb c) Decrease in school performance ------------------ In a child with sickle cell disease, what would be a sign of stroke a) Sudden onset anemia b) Painful limp c) Fatigue d) Poor scholastic performance
Decrease in school performance
What med can't a G6PD kid take? a) Chloroquine b) Trimethoprim ----------------------------------- What can a G6PD patient not take? a) ASA b) Trimethoprim c) Chloroquine d) Acetaminophen e) Quinine
Chloroquine
Cannot take sulfa drugs, but Trimethoprim on its own without sulfamethoxazole should be okay
Antibacterials - sulfonamides - TMP-SMX - nitrofurantoin Antimalarials - chloroquine, other quines Antihelminths Others: vit K analogs, ASA, methylene blue, rasburicase Illness = sepsis, hepatitis, DKA
What treatment is proven to prevent stroke in SCD? a) Transfusion b) Folate c) Hydroxyurea d) Iron ----------------------------- Which treatment modality has been proven to reduce the risk of second episode of stroke in sickle cell patient? a) Hydroxyurea b) Transfusion c) Folic acid d) Penicillin
Transfusion
Sickle cell most common neurologic sequelae a) Silent stroke b) Clinical Stroke c) Seizures ---------------------- Sickle cell anemia, which is true about strokes? a) Usually subclinical strokes b) Clinical stroke
Subclinical strokes
Teenage girl with SSD. Acute fever, jaundice, abdo pain. Suspect cholecystitis, confirmed with U/S. After treating for one week, what do you suggest? a) Cholecystectomy b) Ursodiol c) ERCP ---------------------- 13yo, obese with HbSS and RUQ pain, tenderness, fever, guarding and jaundice. U/S shows multiple gallstones, dilated CBD, inflamed walls. You diagnose acute cholangitis and admit for hydration and triple antibiotics. After the acute process is treated, what do you suggest: a) Transfusion therapy b) Ursodiol c) Cholecystectomy d) Nothing -------------------------- Sickle cell girl who is obese, RUQ pain, increased liver enzymes, had been treated and recovers from the event. What do you recommend? a) Cholescystectomy b) Low-fat diet c) Ursodiol
Cholecystectomy
Asymptomatic stones can be observed without intervention
Symptomatic stones or stones obstructing the common bile duct commonly require cholecystectomy
You are seeing a 1 wk old Chinese boy with 1 day of jaundice. His bili is 270 (mostly indirect), Hg 95 retics 9%, Mom is AB+ and he is B+. He otherwise looks well. What is the diagnosis?
a) Sepsis
b) Thalassemia
c) G6PD def
d) ABO incompatibility
G6PD
No ABO setup
Retic % normal range is 0.2-2%
>3% is normal response to anemia
Sickle cell with recurrent chest crisis. What is he at increased risk of?
a) Recurrent cerebral infarction
b) Asthma
c) Gallstones
Recurrent cerebral infarction
What is the following is most associated with mortality in child with thalassemia major?
a) Fe overload
b) Cardiomyopathy
c) Hyperglycemia
Cardiomyopathy (d/t iron overload from transfusions)
What is the number one cause of complications in beta thalassemia major? a) Iron overload b) Megaloblastic anemia c) Cardiomyopathy d) Poor hematopoeisis ----------------------- What is the number one cause of complications in beta thalassemia major. a) Iron overload b) Megaloblastic anemia c) Cardiomyopathy d) Poor hematopoiesis
Iron overload
Child getting large volumes of PRBC transfusion. What ECG complication do you expect to see?
a) Peaked T-waves
b) U waves
c) Short PR interval
Peaked T waves
Baby with HB of 220, same Hb at 4 wks. What is this associated with?
a) Prematurity
b) T21
c) The small twin in twin-to-twin transfusion syndrome
d) Cystic fibrosis
T21
Small twin in TTTS has anemia b/c donating
The large recipient twin has polycythemia
Term neonate requires blood transfusion. Getting consent from dad. What do you tell him the most common infection in blood transfusion?
a) Hep A
b) Hep B
c) Hep C
d) CMV
Hep B
Parvo B19: 1 in 5000 HTLV 1+2: 1 in 1 million Hep B: 1 in 1.5 million Hep C: 1 in 5 million HIV: 1 in 10 million vCJD: < 1 in 10 million Syphillis: <1 in 100 million
CMV “risks vary with donor/recipient but rare (risk decreased with leukoradiation)”
You are counseling a mother of a SCD patient who will be requiring blood transfusions. Which of the following would she be most likely to receive in a blood transfusion?
a) Hep B
b) Hep C
c) Parvovirus B19
d) Variant of Creutzfeldt Jacob disease
Parvovirus B19 1 in 5000
vCJD <1 in 10 million
What is the advantage of using leukoreduced white cells?
a) Decreases hemolytic reactions
b) Decreases infectious complications
c) Decreases febrile transfusion reactions
Decreases febrile non-hemolytic transfusion reactions
Ineffective for transfusion related GVHD
A 7 year old boy who has had recent personality changes, decline in school performance and visual changes. Which is the first diagnosis to rule out?
a) Brain tumour
b) DM
c) ADHD
d) Depression
Brain tumour
Brain GAME (% of all pediatric brain tumours) Glioma (brainstem 10-20%) Astrocytoma (40-60%; supratentoria) Medulloblastoma (10-20%) Ependymoma (9%)
Posterior fossa:
- medulloblastoma > ependymoma > brainstem glioma > atypical teratoid/rhabdoid
- H/A, vomiting, ataxia
Child with aniridia, what investigation will this child need regularly?
a) CBC
b) Abdominal U/S
c) Echocardiogram
Abdo U/S
WAGR W - Wilms tumour A - aniridia -> 75% have glaucoma G - genitourinary malformations R - metal retardation
When you order blood for preterm babies, why is it irradiated?
a) To decrease the risk of CMV transmission
b) To prevent graft vs. host disease
Prevent GvHD
CPS Statement - Most centres now γ-irradiate blood to deactivate lymphocytes and prevent graft-versus-host disease, which is rare in newborn infants
A child is receiving a blood transfusion. She becomes febrile and develops chills. What is your management?
a) Continue with transfusion, give methylprednisolone
b) Stop transfusion, give steroids
c) Stop transfusion, run IV TKVO
d) Continue transfusion and slower rate
Stop transfusion, run IV TKVO
If fever (+/- chills/rigors)
>1C increase in temp
AND T>38 during or up to 4H post infusion
A. Immediate Mgmt
1. Stop transfusion, maintain IV access
2. Check pt ID, VS + assess for serious Sx (T>=39, rigors/chills, tachycardia, hypotension, anxiety, N/V, dyspnea, chest/back pain, hemoglobuinuria/oliguria, bleeding from IV site)
If right pt, right product, no serious Sx:
- most likely febrile non-hemolytic transfusion reaction
- give acetaminophen
- continue transfusion cautiously
- stop if any serious Sx develop
If wrong pt, wrong product or serious Sx
- suspect 1) hemolytic transfusion reaction or 2) bacterial contamination
- Do not restart trnasfusion
- Send BCx + first post-transfusion urine (check for Hgb)
Description of a teen with wt-loss, fatigue, slight anemia, normal WBC. Next step?
a) CXR
b) BMA
CXR
R/o medastinal mass
Then can do work up with BMA
“Any pt with persistent, unexplained LND should have CXR to r/o large mediastinal mass before underlying LN Bx”
Kid with Burkitt’s and TLS. Urine pH 7.0. What’s next.
a) Hemodialysis.
b) Potassium
c) ACE inhibitor
d) Rasburicase
Rasburicase
Child with ALL finished chemo 1 month ago and is exposed to Varicella. How do you treat?
a) VZV vaccine
b) VZIG
c) VZV vaccine + admit for IV acyclovir
d) Admit for IV acyclovir
VZIG
A 12 year old boy presents with an X-ray (described, no picture given) of mottled bone on right rib with new bone forming on top. What is the most likely diagnosis?
A. Ewings
B. Osteosarcoma
C. Osteochondroma
Ewing
Knew G Ewing when young. He was weird (ribs, pelvis). Kids like ELMO b/c it’s RAD
Lytic
Mottling
Onion peeling
Soft tissue mass. Small round cell. Systemic Sx
Tx: chemo, RADIATION, surgery
Chemotherapy that causes low sodium and vomiting
a) Vincristine
b) Cyclophosphamide
c) Anthracycline
Cyclophosphamide
Vincristine side effect?
a) Peripheral neuropathy
Peripheral neuropathy
What to add to patient burkitt lymphoma with tumour lysis syndrome risk already hyperhydrated and alkalinized?
a) Rasburicase
Rasburicase
Child with ataxia, diplopia, torticollis. Diagnosis? a) Cerebellar astrocytoma b) Brainstem glioma ------------------------------------ Child (age?) with ataxia, diplopia and headaches. What is the most likely dx? a) Brainstem glioma b) Cerebellar astrocytoma c) Craniopharyngioma d) Ependymoma -------------------------------------- Child with head tilt, ataxia, nystagmus. Most likely diagnosis? a. posterior fossa hemorrhage b. cerebellar astrocytoma c. brainstem glioma d. craniopharyngoma
Cerebellar astrocytoma
Cerebellar herniation can present with torticollis!
Torticollis + nystagmus = cerebellar
Head tilt + diploplia = brainstem
Wilm’s tumour is most associated with: a) Tuberous sclerosis b) Fragile X c) Angelman’s d) Beckwith Wiedemann ------------------------------------------ Child with Wilm’s tumor. Which is associated? a) Down syndrome b) Prader-Willi syndrome c) Beckwith-Wiedemann d) Angelman’s syndrome
Beckwith-Wiedeman
Wilm's - 2-3yo 1. Beckwith-Wiedeman 2. WAGR 3. Denys-Drash - most common mets: lungs, LN, liver Tx 1. Surgery! Resection - do not Bx b/c upstages the disease 2. +/- chemo 3. +/- radiation
Which cancer will most likely have bone marrow infiltration?
a) Wilms
b) Neuroblastoma
c) Hepatoblastoma
d) ?bone CA of some sort ( I think osteosarcoma)
—————————
Which of the following would most likely be found in the bone marrow?
a) Neuroblastoma
b) Wilms tumour
d) Hepatoblastoma
Neuroblastoma
What is a risk factor for child leukemia?
a) Parent treated for leukemia
b) In utero radiation
c) Maternal alcohol
d) NF type 1
————————
Which condition is most associated with childhood leukemia?
a) Electromagnetic field exposure during pregnancy
b) NF 1
c) Mom or dad with hx of leukemia
NF1
Childhood leukemia A. Genetic conditions 1. Li-Fraumeni 2. Down syndrome 3. NF1 4. Fanconi 5. Diamond Blackfan 6. Shwachmann-Diamond 7. Bloom 8. SCID
B. Environmental
- Ionizing radiation
- Drugs
- Alkylating agents
16 y.o. boy undergoing treatment for non-Hodgkin’s lymphoma. Forty-eight hours after his last chemotherapy, he develops mild dysuria and hematuria. Platelet count was 90 pre-treatment. He is happy and well-looking. He is sexually active. What is the most likely cause of his hematuria:
a) Cyclophosphamide-induced hemorrhagic cystitis
b) Thrombocytopenia due to myelosuppression
c) Chlamydial urethritis
d) Urine infection
——————-
ALL kid just had chemo. He is sexually active. Now 48 h after has hematuria and mild dysuria. Otherwise well. Plts were 90 before chemo. Dx?
a) Cyclophosphamide induced hemorrhagic cystitis
b) Myelosuppression with thrombocytopenia
c) Chlamydia urethritis
d) UTI
Cyclophosphamide-induced hemorrhagic cystitis
Thrombocytopenia would have to be rapid + profound to induce hematuria within 48H
Chlamydia + UTI are possible, but less likely based on timing
Beckwith-Wiedemann syndrome U/S. A question about how frequently ultrasound should be done.
AUS Q3mo until 8yo
Serum AFP Q1.5-3mo until 4yo
Which of the following is present in tumour lysis syndrome?
a) Hyponatremia
b) Hypokalemia
c) Hyperuricemia
d) Hypophosphatemia
Hyperuricemia
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 blood work shows WBC 18, Plt 110 and anemia. What is the next best step?
a) Bone Marrow Aspirate
b) EBV Serology
c) Work up for systemic JIA
BMA
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?
a) Neuroblastoma
b) Langerhans cell histiocytosis
c) ALL
LCH
A Child has a distended abdomen. An ultrasound shows an adrenal mass and hepatomegaly. Which of the following tests will make the diagnosis?
a) MIBG Scan
b) Serum AFP
c) Urine HVA/VMA
d) Abdo/Chest CT
Urine HVA/VMA
Neuroblastoma
MIGB is for staging, not diagnosis
Kid with 2.5cm supraclavicular node. 8kg wt loss in past 6mos. What do you do first?
a) IV Piperacillin
b) IV fluids and allopurinol
c) IV methypred
d) Isoniazid
IV fluids + allopurinol to prevent TLS
IF CXR was an option, do this first
Supraclavicular node -> Hodgkin lymphoma, which isn’t usually assoc’d with TLS, but there are other malignant DDx for wt loss + LND
15 year old female with resp distress with first episode of wheeze in ER. Resoled with ventolin and IV methylpred. One week later, CXR done de to ongoing symptoms and mediastinal mass seen. What is the most likely diagnosis:
a) ALL
b) HL
c) sarcoidosis
d) TB
HL
8 year old healthy child with 1.5 x 2 cm supraclavicular lymph node, firm, non-tender, mobile with no erythema. What to do? a) Excisional biopsy b) PPD c) Bartonella serology d) EBV serology ------------------------ 8 yo boy with left supraclavicular node 1.5cm x 2cm noted over several days. Painless, non-tender, mobile. No overlying erythema. What is the next diagnostic test? a) Bartonella serology b) Excisional biopsy c) TB skin test
Excisional Bx
Supraclavicular LN is not good
Do CXR first
Teenager with right supraclavicular node, weight loss, fever, anorexia in last several weeks. Spleen tip palpable. Hb 120, WBC 10, Platelets 150. Diagnostic test?
a. CXR
b. Bone marrow aspirate
c. CT abdo
If next step, then CXR
Dx for lymphoma more from LN Bx/excision
but BMA are part of the IVx to do for lymphoma
Teen with weight loss, generalized lymphadenopathy including supraclavicular node, and palpable spleen tip. More fatigue recently. Afebrile. WBC 10, Hgb 120, Plt 150. Normal Monospot. What is the next step in evaluation: a. BM aspirate b. Chest X-ray c. Excise node d. ANA ------------------------------------ Teenager with generalized lymphadenopathy and right supraclavicular node. What to do next? a) CXR b) BMA c) CT abdo ------------------------------------ Teenager has lymphadenopathy (including supraclavicular) and fevers, weight loss. What do you do? a. Abdominal ultrasound b. Chest xray c. CT chest/abdo d. Bone marrow ------------------------------------ 14 yo male with constitutional symptoms, weight loss, generalized lymphadenopathy including supraclavicular. Hb 120, WBC 10, Plt 150. Splenomegaly present. What is your initial investigation? a) CXR b) Abdominal CT c) Bone marrow d) Tb skin test ------------------------------------ Teen boy with adenopathy and supraclavicluar node. Has been feeling crappy for a few weeks. Spleen tip palpable. HIV test and Monospot are negative. Next test to do: a) Bone marrow biopsy b) CXR c) Other irrelevant stuff (no smear or CBC)
CXR to r/o mediastinal mass
Then LN Bx for Dx
4 yo girl with fever, splenomegaly, diffuse lymphadenopathy, purpuric rash on legs. Ulcerated pharynx. WBC 24, HB 80, Plts 20. Likely diagnosis?
a) Leukemia
b) Lymphoma
c) Mononucleosis
d) idiopathic thrombocytopenic purpura
e) aplastic anemia
Leukemia
High WBC
Low Hgb
Low plt
An 8 month old baby presents with an abdominal mass, thrombocytopenia, systolic murmur and a murmur over the liver. Ultrasound shows a 3 cm mass in the liver. What would you do?
a) DIC work-up
b) Bone marrow
c) Pulmonary imaging
d) Liver function tests
DIC workup
Kasabach Merritt
Baby with petechiae. Plt 12. After transfusion, Plt are 16. Mom’s CBC normal. What is best management?
a) PLA1 negative platelets
b) IVIG
PLA1 negative plts
NAIT
- fetal plts have Ag inherited from father that mother lacks. Mother develops Ab that cross placenta + destroys fetal plts
- baby low plts, mother normal plts
- Prenatal: give mother IVIG
- Postnatal
1) maternal washed plts
2) irradiated (CMV neg) HPA comapitble (HPA-1a) neg plts
3) consider HUS for ICH - thrombocytopenia resolves in 4-6wks
Maternal ITP
- mother has autoimmune condition production IgG Ab to all plts Ag. Destroys her own plts + also cross placenta + destroys fetal plts
- both have low plts
- lower risk of neonatal ICH
- Tx
1) IVIG +/- steroids
2) If emergency, give random donor plts - thrombocytopenia resovles in 4-6wks
Newborn with trisomy 21. High WBC, thrombocytopenia, anemia, HSM, petechiae. What does he have?
a) CMV
b) Sepsis
c) Transient myeloproliferative
Transient myeloproliferative
See notes
A baby’s platelets are found to be low on day of life 2. His mother’s platelets are normal. What is the most likely diagnosis?
a) Maternal ITP
b) NAIT
NAIT
When would you do bone marrow in ITP? a) Hb <115 b) Previous steroid use c) ANC <1000 d) Febrile --------------------------------- Child with thrombocytopenia (platelets 15 x 109/L). which finding would suggest biopsy? a) Fever > 39.5 b) Previous steroid use c) Hb < 115 d) ANC < 1000 -------------------------------- In ITP, when to do bone marrow? a) Fever > 39.5 b) Absolute neutophil < 1000 c) Haemoglobin 115 -------------------------------- Which of the following is an indication for bone marrow biopsy in a child with ITP who has platelets of 12 000? a) ANC <1000 b) Previous use of steroids c) Hb <115 d) Fever > 39 ---------------------------------- When would you do bone marrow in ITP? a. Hb <115 b. Previous steroid use c. ANC <1000 d. Febrile
Neutropenia ANC <1000
Picture given of large hemangioma over V1 to scalp and upper eyelid distribution with bluish tint. Baby presents with this facial lesion and high output CHF. Apart from thrombocytopenia what else would you expect to see on CBC or smear:
a) Neutropenia and mild anemia
b) Normal INR/PTT
c) Schistocytes
d) High Fibrinogen
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Picture of a baby sucking on a pacifier and looking relatively content. Severe thrombocytopenia. Has a large lesion (looks kinda like the picture here) overlying his left forehead and eyelid (not a port-wine stain). What is the most likely finding on labs?
a) Normal INR and PTT
b) Elevated fibrinogen
c) Schistocytes and RBC fragments on smear
Schistocytes + RBC fragments
Kasabach Merritt syndrome
- Hemangioma
- MAHA
- Consumptive coagulopathy
- Thrombocytopenia
Child with ITP and no other concerning risk factors on exam or history. What will his platelet count be in one year from now?
a) Probably will be normal
b) Depends on results of the current bone marrow
c) Depends on the treatment given
Probably will be normal
70-80% have spontaneous resolution within 6mo
20% go on to have chronic ITP
10 yo boy with SOB. CXR reveals a large mediastinal mass and a moderate right-sided pleural effusion. The pleural fluid contains numerous lymphoblasts that are TdT-positive and express T-cell antigens. On bone marrow aspiration, lymphoblasts with identical characteristics represent 12% of total cells. What is the diagnosis? a) Hodgkin lymphoma b) T cell ALL c) Burkitt lymphoma d) lymphoblastic lymphoma e) Neuroblastoma
lymphoblastic lymphoma
BM lymphocytes are >25% in leukemia, so since this is only 12%, can’t be T-cell ALL
