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
