Heme/Onc Flashcards
(106 cards)
1
Q
How much iron should be in formula for normal BW infant for the first 9-12 months?
High-risk for iron deficiency infant?
A
6.5-13mg/L
13mg/L
2
Q
List 5 risk factors for iron-deficiency in a child under 2 years
A
- Preterm delivery
- Low socio-economic status
- Maternal anemia or obesity
- Early umbilical cord clamping
- Prolonged bottle use
- Chronic infection
- Lead exposure
- Low intake of iron-rich complementary foods
- Living in an Indigenous community that may be challenged with poverty, food insecurity, high burden of H pylori or high consumption of cow’s milk/evaporated milk
- Low birth weight (<2.5kg)
- Male sex
3
Q
List 2 effective measures to reduce iron deficiency
A
- Feed iron-rich complementary foods from 6mo
- Delayed cord clamping
- If formula feeding, provide iron fortified formula
- Do not use cow’s milk as main milk source until 12mo (limit to 500mL/<20oz)
4
Q
Who should receive additional iron supplementation?
When should it start?
How much should they receive?
What duration of time?
A
Who:
- Predominantly breastfed infants (>50%)
- Low birth weight (<2.5kg)
When: starting at 2-3 weeks postnatal age
How much/how long?:
- <2kg: 2-3mg/kg/day x 1 year
- 2-2.5kg: 1-2mg/kg/day x 1 year
5
Q
What are the stages of iron deficiency anemia in terms of changes seen to laboratory tests?
Why do we treat?
How much supplementation?
For how long?
A
Stages (2 non-anemic, 1 anemic0
- ↓ ferritin→ depleted iron stores
- ↓ transferrin saturation→ decreased iron transport
- ↓ hemoglobin → decreased production of hemoglobin
Why treat: Risk of permanent neurodevelopmental impairment
How much?: 2-6mg/kg/day divided doses
How long?: 3 months (then recheck ferritin/CBC)
6
Q
What are the types of Vitamin K deficiency of the newborn?
A
- early onset (1st 24 hours post-birth)
* Associated with maternal medications that inhibit vit K activity, e.g. anti-epileptics - classic (DOL 2 to 7)
* low intake of vit K - late onset (2 - 12 weeks and up to 6 months of age)
- associated with chronic malabsorption and low vit K intake
- Presents with ICH
7
Q
What are the categories of brain tumours and their presenting symptoms?
A
- General symptoms:
- Headache
- N/V (usually in the morning)
- Due to vasodilation of cerebral vessels overnight so more cerebral blood volume = raised ICP
- Macrocephaly in infants/toddlers
- Irritability
- Obstructive hydrocephalus (papilledema, vomiting, obtundation)
- Neurologic symptoms based on locations:
-
Supratentorial:
- New seizure
- Visual changes
- Visual field defect = involvement of optic nerves or tracts
- Diencephalic syndrome:
- Severe emaciation and FTT in otherwise happy infant
- From tumours in hypothalamus
-
Infratentorial:
- Ataxia, gait abnormalities
- Nystagmus, esp with cerebellar tumours
- Parinaud syndrome:
- Paralysis of up-gaze, pupils mid-dilated and poor reaction to light, convergence or retraction nystagmus and eyelid retraction
- From pineal or dorsal midbrain tumours
- Tumours compress the rostral interstitial nucelus of medial longitudinal fasciculus (riMLF) on mesencephalic tectum
- Head tilt
- Obstructive hydrocephalus
- Resection of infratentorial tumours often leads to cerebellar mutism syndrome
- After surgery, pts have mutism, irritability, ataxia, hypotonia lasting few weeks to 6 months, then period of dysarthria that usually self-resolves
- Occurs in up to 24% of pts with infratentorial resections
- Most common in medulloblastoma
-
Parasellar:
- Endocrine dysfunction (under or overproduction of hormones)
- Visual field defects from growth up into optic nerve
-
Spinal:
- Weakness
- Pain
-
Supratentorial:
8
Q
What are the definitions, symptoms and treatment for pituitary adenomas?
A
- Pituitary adenoma
- Anterior pituitary gland tumor
- Benign
- Microadenoma is <1 cm, and macroadenoma is >1 cm diameter
-
Non-secreting tumors: 30%
- Can be completely asymptomatic
- May cause hypopituitarism from compression
-
Hormone-secreting tumors: 70%
- Prolactinomas cause milk production and inhibit GnRH, resulting in diminished testicular/ovarian function
- Gigantism/acromegaly from GH
- Cushing disease from adrenocorticotropic hormone (ACTH)
- Hyperthyroidism from TSH
- Treatment
- No need to treat microadenoma if not symptomatic
- Medical management of prolactinoma
- Dopamine agonist: decreases size of adenoma and decreases prolactin
- If prolactin levels do not normalize, proceed to surgical debulking
9
Q
What are the most common brain tumours?
A
- Low-grade gliomas
- Most common brain tumor in children
- Neuroepithelial cell of origin
- Generally slow growing, but based on location, can cause significant symptoms
- Pilocytic astrocytoma:
- Infratentorial, usually arising in cerebellum
- Most common glioma in children
- Optic nerve gliomas:
- Associated with NF1
- Diffuse fibrillary astrocytomas
- Pilocytic astrocytoma:
- Medulloblastomas
- Most common malignant (high-grade) brain tumors in children
- Embryonal cell of origin
- Arise in cerebellum
- Can be metastatic to bone marrow (bone marrow biopsy required as part of workup)
10
Q
What is the significance of Factor XII deficiency?
A
Factor XII is a ‘contact factor’
- Does not cause prolonged bleeding
- Used as a factor in PTT testing, so will have MARKED elevation in PTT, with NO SIGNIFICANT BLEEDING
- Patients do not need treatment for major injuries or surgery
11
Q
List the differences between Fanconi anemia, Schwachman-Diamond syndrome, Dyskeratosis congenita and Diamond-Blackfan anemia.
A
Fanconi anemia (“congenital aplastic anemia”)
- Inherited autosomal recessive inheritance
- Typically have bone marrow failure by age 5-10
- Present usually at school age
- Findings: café au lait spots, short stature, abnormal thumbs and radii, microcephaly, renal abN, cardiac defects
- Pancytopenia with macrocytic anemia
- Tx with BM transplant and androgen therapy
- Higher risk of leukemia, MDS, squamous cell carcinoma, head/neck cancers
Schwachman Diamond Syndrome:
- Triad:
- Exocrine pancreatic insufficiency + bone marrow failure + skeletal anomalies
- Present infancy to school age
- Exocrine pancreatic insufficiency + bone marrow failure + skeletal anomalies
- Findings: steatorrhea, FTT, skeletal abN, hepatomegaly
- Neutropenia > anemia > pancytopenia
- Tx with pancreatic enzyme supp and BM transplant
- Higher risk leukemia
Dyskeratosis congenita:
- Telomere maintenance gene mutation
- Dystrophy of hair, nails and bone marrow failure
- Findings: hair and nail dystrophy, reticular skin pigmentation, oral leukoplakia, pulmonary fibrosis, pancytopenia
- Tx with bone marrow transplant and androgen therapy
- Higher risk leukemia, squamous cell carcinoma, head/neck tumours
Diamond-Blackfan Anemia:
- Defect in erythroid progenitor cell, leading to increased apoptosis
- Profound anemia by 2-6 months (macrocytic anemia with no retics, not pancytopenia)
- Usually autosomal dominant
- Presents in infancy
- Findings: growth delay, craniofacial abN (hypertelorism) and thumb abN
- Tx with steroids (80% respond) but if refractory then pRBC transfusions every 1-2 months with chelation for iron
Can do BM transplant only if HLA matched sibling donor (otherwise survival much lower)
12
Q
Polycythemic newborn. Hb 240, Hct 0.75. Wt 2000g. Child requires a partial exchange transfusion. What fluid do you use as the diluent? How much blood to you replace to decrease the Hct to 0.5?
A
- NS 2. volume to be exchanged (ml) = blood volume x (observed-desired hematocrit)/ observed hematocrit - blood volume = 85ml/kg = 170ml in this case - therefore tfn volume = 170 x (0.75-0.5)/0.75 = 56.6ml
13
Q
Transient fever in blood transfusions is usually a result of: a. sensitization to WBC antigens b. acute GVHD c. hepatitis C d. hemolysis
A
a) sensitization to WBC antigens (leukoreduction helps prevent this) - hemolytic reactions can cause fever but are not transient and not the the most common
14
Q
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
A
c. Stop transfusion, run IV TKVO Febrile non-hemolytic transfusion reaction - stop the transfusion, give antipyretics and monitor
15
Q
You are counseling a mother and her young daughter with sickle cell anemia who requires a PRBC transfusion. Which of the following infections is she most at risk of acquiring from a transfusion: a. Hep B b. Hep C c. Parvovirus B19 d. HIV
A
c. Parvovirus B19 - Hep B: 1 in 1.1-1.7 million - Hep C: 1 in 5-7 million - HIV: 1 in 8-12 million - Parvovirus: 1 in 5000 to 1 in 20000 Most common transfusion related infection is yersinia enterocolitica
16
Q
A child with thalassemia major on recurrent transfusions and desfuroxime. What is the most serious complication: a. cardiac hemosiderosis b. chronic anemia c. cognitive impairment d. liver hemosiderosis
A
a. cardiac hemosiderosis - 1 year of tfns = liver hemosiderosis - 2 years of tfns = endo (hypopara, hypogonadotropic hypogonadism) - 10 years = cardiac - this is what kills you if you don’t have iron chelation therapy
17
Q
Which of the following is present in tumour lysis syndrome: a) hyperuricemia b) hyponatremia c) hypokalemia d) hypophosphatemia e) hypercalcemia
A
a) hyperuricemia HyperPO4, hyperK, hypoCa
18
Q
6 year old who has a history of diplopia, headache and ataxia. Where is the lesion? (1) What are the two most likely brain tumours for the lesion (2)
A
- posterior fossa (brainstem and cerebellum) 2. cerebellar astrocytoma and medulloblastoma
19
Q
2 life threatening presentations of anterior mediastinal mass?
A
- SVC syndrome (facial or upper extremity swelling from vascular compression) - cardiac tamponade - respiratory arrest from airway compression
20
Q
A 3 year old girl with fever, arthralgia and lethargy for 10 days has lymphadenopathy,moderate hepatosplenomegaly, no obvious arthritis but screams in pain with minimal examination. WBC 9.5 Hgb 98 Plts 140, smear Normal. Next test: a) bone marrow aspirate b) EBV titers c) follow
A
a) bone marrow aspirate
21
Q
3 week old who is brought to the office because mother thinks he is too yellow. Breastfed. Otherwise well. Total bili is 180. Direct is 8. What do you do? A. septic workup B. investigate for blood group incompatibility C. reassure mother that condition may last for 4-12 weeks D. investigate for metabolic disease
A
C. reassure mother that condition may last for 4-12 weeks Breast milk jaundice
22
Q
You saw a 15 year old M with respiratory distress and bilateral wheeze in the ER. This was the first episode of wheeze. Resolved with IV methylprednisone and ventolin in ER and he was sent home. One week later the radiologist is reviewing the film and notices a widened mediastinum. What is the most likely cause? A. Thymoma B. Hodgkin’s lymphoma C. ALL D. Sarcoidosis
A
B. Hodgkin’s lymphoma
23
Q
Abdominal mass in RUQ (documented on U/S as well). Systolic heart murmur at LSB and RUQ on exam. Conjugated hyperbilirubinemia. Low platelets. What test should you do next? a. DIC work-up b. Bone marrow biopsy
A
a. DIC work-up Platelets, INR (normal-high), PTT (normal-high), fibrinogen (low), D-dimer (high) - Kasabach Merritt
24
Q
12 year old M with recent change in behaviour, irritability, daily headaches and a change in his vision. What is the most important thing to consider? A. Brain Tumour B. Drug use C. Psychiatric Diagnosis
A
A. Brain Tumour
25
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
B. NF 1
26
Name 3 syndromes associated with increased risk of leukaemia:
NF1 T21 Fanconi anemia Schwachman Diamond ataxia telangiectasia
27
Left supraclavicular LN - mgmt? a. Excisional biopsy b. monitor c. EBV serology
a. Excisional biopsy
28
Poor prognosis in ALL is suggested by: a) female b) age \<1 year c) CALLA positive d) mediastinal mass e) splenomegaly
b) age \<1 year CALLA positive indicative of positive response to treatment Poor prognosis: age \<1 or \>10, T cell leukaemia, initial WBC \>50,000, slow response to initial treatment
29
Better prognosis in neuroblastoma is associated with: a) female b) age less than 1 year c) high excretion of VMA d) normal blood pressure e) unilateral
b) age less than 1 year
30
Paraneoplastic syndrome associated with neuroblastoma: a) chorea b) athetosis c) diarrhea d) hypertension e) hypercalcemia
c) diarrhea - due to secretion of vasointestinal peptide (VIP) Also, opsoclonus-myoclonus-ataxia syndrome
31
Opsoclonus is seen in which of the following: a. AML b. ALL c. Neuroblastoma d. Medulloblastoma e. Rhabdomyosarcoma
c. Neuroblastoma
32
Wilm’s tumour is associated with all of the following except: a. 11p abnormalities b. NF c. Wiskott-Aldrich d. Aniridia e. Beckwith Wiedemann
c. Wiskott-Aldrich NF1 rare/contraversial as well - 11p abnormalities, even without BES, can be associated with increased risk of WIlm's tumour (nephroblastoma)
33
14 yo girl with leg pain, radiograph shows lesion in the distal femur with ‘sunburst’ appearance. What is the likely cause? a. Osteoid osteoma b. Ewing’s sarcoma c. Osteogenic sarcoma d. Aneurismal bone cyst
c. Osteogenic sarcoma (AKA osteosarcoma) - most common primary malignant bone tumour in children and teens - note: a. Osteoid osteoma (small benign bone tumour- unremitting, gradual increasing pain at night and relieved by aspirin; Tx- surgical removal - XR shows middle white spot surrounded by dense bone)
34
Poor prognosis for Langerhans cell histiocytosis is associated with: a) mastoiditis b) pancytopenia c) lymphadenopathy d) chronic lung disease e) vertebral body involvement
b) pancytopenia
35
Child (age?) with ataxia, diplopia and headaches. What is the most likely dx? a. brainstem glioma b. cerebellar astrocytoma c. craniopharyngioma d. ependymoma
b. cerebellar astrocytoma cerebella astrocytoma and medulloblastoma are most common posterior fossa tumours
36
Child with Wilm’s tumor. Which is associated? a. Down syndrome b. Prader-Willi syndrome c. Beckwith-Weideman d. Angelman’s syndrome
c. Beckwith-Weideman
37
You are referred the following 16 month old child with a suspicion of non-accidental trauma. On examination you find that the child has nystagmus and a palpable abdominal mass. What is the most likely diagnosis?
Neuroblastoma
38
Scenario of a teen with cancer who family now decides to make him palliative. But his 10 yr old brother, with whom they were quite close as brothers, starts ignoring him, and spending not as much time with him. His parents are concerned. What do you tell them? (1 point)
- it is normal for family members including siblings to experience anxiety, depression, guilt, fear and anger, which might make them pull away - include in discussions about his brother's care - sibs who know about and are involved in the care of their dying sib cope better before and after their death
39
List 3 side effects of L-aparaginase
pancreatitis, hyperglycaemia, platelet dysfunction, coagulopathy
40
List 3 side effects of Vincristine
local cellulitis, peripheral neuropathy, constipation, SAIDH
41
List 3 side effects of Prednisone
hypertension, T2DM, increased infection risk, AVN
42
Teenage boy with stage III Hodgkin’s. Going to have chemo and radiation. 1.) List 2 factors that may affect his fertility 2.) Give 2 options to deal with this possible infertility
1. high cumulative dose of alkylating agents, high radiation doses, health behaviour like tobacco or cigarette smoking 2. sperm banking (for boys who have gone through puberty) - shield testicles during radiation
43
Give four laboratory findings in tumor lysis syndrome.
- hyperkalemia - hyperphosphatemia - hypocalcemia - hyeruricemia
44
List 3 treatments for hyperuricemia
- allopurinol (blocks enzyme in pathway that makes uric acid) - rasburicase (makes uric acid more water soluble and easily excreted) - dialysis
45
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
A. Cyclophosphamide induced hemorrhagic cystitis
46
Tumor lysis syndrome. On alloprirunol urines ph is 7.5. Hyperhydrated. What to do next. a) potassium in IV fluids b) add rasburicase
b) add rasburicase
47
Neutropenic child with central line site red. Which antibiotics?
piperacillin-tazobactam and vancomycin
48
List 6 longterm side effects of Hodkin's Lymphoma treatment - radiation to neck and chest, chemo of cyclophosphamide, VCR plus others.
- peripheral neuropathy - delayed puberty - infertility - hypothyroidism - cardiomyopathy - pulmonary fibrosis
49
Long term complications of curative therapy for cancer. List 4.
- cardiomyopathy - hypothyroidism - infertility - pulmonary fibrosis
50
List 3 long term effects of cyclophosphamide
- bladder cancer - delayed puberty - infertility - AML
51
Older child who is at home for palliative care for her AML. (4) What are four principles of treatment with opiods that would guide your treatment
- dosing needs to be individualized and titrated for the patient - the right dose is one that relieves pain and minimizes side effects - side effects should be anticipated and treated - ensure good basal level of pain control with prn medication available for breakthrough pain - administer medication via the simplest, most effective and least distressing route
52
A girl presents with Hb=80 and MCV=60. Ferritin and iron are normal. Next test: a. Hb electrophoresis b. Barium swallow c. Bone marrow
a. Hb electrophoresis
53
4 year old of Mediterranean descent. Lab findings most consistent with Thal 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 80 MCV 60 RBC 3.3 e. Hb 60 NCV 50 RBC 2.2
ANSWER: a. Hb 100 MCV 60 RBC 4.8 (Mentzer= 12.5) Alpha or beta thal= Should see: microcytic anemia with high RBC count and low or normal retic - Mentzer Index: MCV/ RBC count in millions o \> 13= iron deficiency more common; \<13 beta thal more likely
54
A 3-year-old girl has had persistent fever and sore throat for several days despite antibiotic therapy. There is ulceration of her tonsils and oropharynx, generalized lymphadenopathy, splenomegaly, and purpuric lesions on her lower extremities. WBC 25.6, hemoglobin 80, platelets 30. Most likely: a) infectious mononucleosis b) acute lymphocytic leukemia c) idiopathic thrombocytopenic purpura d) aplastic anemia e) Lymphoma
b) acute lymphocytic leukemia
55
An 8-year-old girl presents with fatigue. Blood work shows: hemoglobin 80, MCV 50, ferritin 150 μ g/L, serum iron 50 μ mol/L. What is your next test: a) barium meal b) osmotic fragility test c) abdominal Tc 99 scintigraphy d) hemoglobin electrophoresis e) bone marrow biopsy
d) hemoglobin electrophoresis - microcytic anemia + normal iron studies = thalassemia \*osmotic fragility test is for hereditary spherocytosis
56
A 2-year-old is in your office for a routine check-up. His mother has no complaints. Growing well. You note pallor and do some blood work. Hemoglobin 80, low retic count. Smear is normocytic normochromic. Next investigation: a) bone marrow aspirate b) hemoglobin electrophoresis c) Coombs' test d) serum ferritin
a) bone marrow aspirate BW does not fit with thalassemia (microcytic), sickle cell (high retics), hemolysis (high retics) or iron deficiency (microcytic) for BMA best choice by elimination. \*IF there was an option to do nothing and repeat in one month, would consider that as could be TEC
57
Child with microcytic hypochromic anemia. You believe it is Fe deficiency. What other features of CBC would be consistent with this? List 3.
- increased RDW - RBC count decreased - MCH decreased - WBC count normal - thrombocytosis
58
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 sclera seem a bit yellow. Her CBC shows a Hgb 70, normal WBC, normal platelets, and a retic count of 24%. What type of anemia is this [1 point]? What test would you do to confirm [1 point]? What treatment could you offer [1 point]?
1. autoimmune hemolytic anemia 2. DAT 3. Hb \<100 and retics \>10% - consider prednisone and IVIG
59
Long term consequence of fanconi anemia?
- pancytopenia - bone marrow failure - AML - squamous cell carcinoma of head, neck and esophagus
60
All are features of iron deficiency anemia EXCEPT: a) pica b) koilonychia c) cheilosis d) mild scleral icterus e) psychomotor retardation
d) mild scleral icterus
61
A blood smear of a patient with iron deficiency anemia would show all EXCEPT: a) Heinz bodies b) poikilocytes c) microcytosis d) hypochromasia e) normal or low reticulocyte count
a) Heinz bodies - see in G6PD b) poikilocytes (abnormal shape cell as general term- yes can see elliptocytic or cigar shaped RBC)
62
9 month old ex 32 weeker takes 40 oz of homo milk per day. Hgb is 60, MCV 50. She is treated with Fe 4mg/kg/day for one month. On repeat testing her Hgb is 62, MCV is 50, and her reticulocytes are 0.01. What to do next: 1. BMA 2. Verify compliance 3. Hb electrophoresis 4. Jejunal biopsy
2. Verify compliance
63
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 bloodwork which shows the following: • MCV 80 normocytic • 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
c. transient erythroblastopenia of childhood \*• HB electrophoresis: AS sickle cell trait
64
7 year old with severe anemia. BM shows arrest of erythroid precursors. Dx? a. Aplastic anemia b. TEC
b. TEC - platelets normal or elevated, retics low - prognosis: spontaneous recovery in 1-2 months \*aplastic anemia: absence of hematopoietic stem cells
65
Fullterm baby delivered to an O+ mom. Looks well but pale. Hb is 70, he is hemodynamically stable. What is the most likely diagnosis? 1. ABO incompatibility 2. Chronic fetal maternal hemorrhage 3. Rh incompatibility
2. Chronic fetal maternal hemorrhage ABO incompatibility: no pallor at birth; presents with jaundice in first 24 hours
66
Baby born at home at 38 wks by midwife. Now presents at 7 days with melena. Hb 70, MCV 112, plts normal. What is the most likely diagnosis?
Vitamin K deficiency leading to hemorrhagic disease of the newborn
67
Neonatal hemophilia – 4 clinical presentation in the neonatal period
- factor VIII and IX do not cross the placenta so symptoms can be present in utero or from birth - intracranial hemorrhage (2% of neonates) - prolonged bleeding with circumcision (30%) - prolonged bleeding from heel poke/venipuncture (newborn screen) - cephalohematoma - subgaleal hemorrhage
68
Labs in keeping with hemophilia? and effect of mixing studies?
Prolonged PTT - 2-3 x ULN - when you mix normal plasma with patient plasma the PTT corrects - platelets and INR are normal (in the lab, INR measures activation of factor X directly by factor VII, BUT in real life factor VII activates factor IX which activates factor X so there is a real life deficiency, but it's not picked up in the lab) - specific assays for factor VIII and Ix show deficiency
69
Newborn with non-hemorrhagic stroke – 4 inheritable etiologies for clot
- congenital heart disease - inherited prothrombotic disorders (factor V Leiden, prothrombin gene mutation) - protein C/S deficiency - antithrombin III deficiency
70
In ITP: a) you will find a big spleen b) no proven link to preceding viral infection c) CNS intracranial bleed is the worse complication
c) CNS intracranial bleed is the worse complication
71
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
A. ANC \<1000 \*C. Hb \<115 - think Evans, TTP, HUS
72
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: A. PLA-1 negative platelets B. IVIG C. Washed maternal platelets
ANSWER: b - IVIG This is autoimmune (likely maternal ITP) - mom and baby have TCP A. PLA-1 negative platelets → NAIT B. IVIG → Can be used in NAIT and AI but more effective in AI than NAIT C. Washed maternal platelets
73
Which of the following predisposes to early hemorrhagic disease of the newborn: a) breastfeeding b) prematurity c) cystic fibrosis d) maternal phenytoin (what we always used to say BUT recent evidence does not support this but says clinical practice up to you (look at mother risk)) e) diarrhea
ANSWER: d) maternal phenytoin (now actually somewhat controversial in evidence, but best answer) a) breastfeeding - at higher risk for late onset, especially if never got vitamin K c) CF - used to think this was true (malabsorption), but evidence does not support this
74
In which of the following is platelet transfusion indicated? a. aplastic anemia b. acute ITP c. chronic ITP d. HUS e. ITP secondary to quinidine toxicity
a. aplastic anemia \*note: in acute ITP if intracranial bleed
75
A patient is being assessed for a bleeding diathesis. Labs show PT 12 s (normal), aPTT 50 s (prolonged), bleeding time 5 minutes (slightly prolonged), platelets 250,000. a) hemophilia b) von Willebrand’s disease c) vitamin K deficiency d) factor V deficiency e) factor X deficiency
b) von Willebrand’s disease - vWD and hemophilia both have normal INR, long PTT, normal platelets, but vWD has normal or long bleeding time and hemophilia has normal bleeding time - vWF tethers platelets to damaged endothelium, and acts as a carrier protein for factor 8
76
A VWD patient presents with bleeding. Which is the best treatment: a. FFP b. DDAVP c. Cryoprecipitate d. Platelets
b. DDAVP Type 1 vWF - quantity issue - treated with desmopressin Type 2 - quality issue - treated with vWF containing concentrate Type 3 - severe quantity issue - treated with vWF containing concentrate \*cryo is best for low fibrinogen but can be used in a pinch for vWD, hemophilia A or B if specific factor concentrate is not available
77
Most consistent with DIC: a) increased fibrinogen b) increased PTT c) decreased PT d) decreased INR e) decreased fibrin split products
b) increased PTT - high d-dimer o Blood replacement (PRBCs) for haemorrhage ▪ +/- plt (thrombocytopenia) ▪ +/- cryoprecipitate (for low fibrinogen) ▪ +/- FFP (replace coagulation factor + natural inhibitors)
78
15 year old girl with periods for a couple of years. Epistaxis and menorrhagia. What to check for? a. von Willebrands b. Hemophilia C c. Factor V Leiden mutation
a. von Willebrands Factor V Leiden mutation predisposes to clots, not bleeding
79
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
b. give Factor VIII before immunizations c apply pressure for 10 minutes
80
2 yo child with new onset petechiae and purpura, nosebleed yesterday. Remainder of physical exam normal. Had viral infection 1 week prior. WBC 5, Hb 100, Plt 5. Smear normal. a.) Most likely diagnosis. b.) List 2 modalities of treatment for this condition.
a. ITP b. reassurance and watchful waiting is generally indicated - if treating could use single dose of IVIG or short course of corticosteroids
81
List 3 common causes of neutropenia .
- viral suppression - chronic idiopathic neutropenia - cyclic neutropenia - drug induced (penicillins) - leukemia/lymphoma
82
Description of newborn with petechiae. WBC normal, Hgb normal, plt 9. Rest of exam is normal. List 3 most likely causes aside from sepsis.
- neonatal alloimmune thrombocytopenia - autoimmune thrombocytopenia (maternal ITP) - TORCH infection
83
4 yo child with abd mass, looks pale, Hb 80, WBC 4. List the Ddx (3). List 3 investigations which will help you distinguish between the diseases listed in your differential before a biopsy can be done.
1. Ddx: - wilms tumour - neuroblastoma - lymphoma - hepatoblastoma 2. Ix: urine VMA and HVA (neuroblastoma) - U/S - CBC (polycythemia and thrombocytosis in Wilms) and peripheral blood smear - U/A (hematuria with Wilms) - increased alpha-fetoprotein for hepatoblastoma
84
Newborn with platelets of 12, transfused and post-transfusion platelets were 16. Mom’s CBC was normal. What do you do? a. transfuse single donor platelets b. transfuse PLA-1 negative platelets c. give IVIG d. give steroids
b. transfuse PLA-1 negative platelets (AKA HPA-1) - Treatment o IVIG prenatally to mom o C/S for delivery o Transfusion 1. washed maternal platelets 2. cross-matched to mom’s serum 3. PLA-1 negative platelets 4. Random platelets (unlikely to improve as 98% of population PLA1 positive)
85
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 bone marrow aspiration in the setting of a probable diagnosis of ITP?
Basically anything that makes you think maybe it's actually leukaemia, not ITP ▪ Abnormal WBC/ differential ▪ Unexplained anemia ▪ Hepatosplenomegaly or lymphadenopathy ▪ Bone or joint pain
86
14 yo girl with sickle cell disease presents with red swollen painful hand. No vitals mentioned. (3) what are three things you do for management. (1) After leaving the hospital, what is one medication that can be taken for prevention of these episodes
1. dactylitis mgmt: - IV hydration - pain control (start with acetaminophen and NSAIDs, often need opioids) - distraction (heat pads, play therapy, massage) - consider ix for osteomyelitis 2. hydroxyurea
87
List 4 indications for blood transfusion in sickle cell disease
Treatment of acute complications: - acute chest syndrome - aplastic crisis - stroke - splenic sequestration Prevention: - prevent stroke in pt with abnormal intracranial dopplers - prior to surgery to prevent ACS after
88
You are seeing a pregnant woman during her first. Her father has haemophilia. Regarding the risk of her transmitting the disorder to her own children you tell her: a. None will have it b. 50% of her sons will have it and all of her daughters will be carriers c. 50% of her sons will have it and 50% of her daughters will be carriers
c. 50% of her sons will have it and 50% of her daughters will be carriers X-linked recessive inheritance
89
What is the number one cause of complications in beta thalassemia major. a. Iron overload b. Megaloblastic anemia c. Cardiomyopathy d. Poor hematopoeisis
a. Iron overload
90
Teenage sickler girl, sexually active has had previous transfusions. She presents looking pale and icteric, with abdominal pain. On exam her right upper quadrant is tender with no guarding. Her hemoglobin is 70, AST 60 and ALT 55. Her bilirubins are elevated. What is her diagnosis? a. liver vaso occlusive crisis b. acute cholecystitis c. hemachromatosis d. Hep C e. Fitz-High Curtis
b. acute cholecystitis
91
3 yo Sickle cell, with fever cough, tachypnea, unwell. What do you need to rule out 1 st ? A. Acute chest crisis B. PE C. Pneumonia D. Asthma
A. Acute chest crisis
92
In which of the following situations should you investigate a child with sickle cell anemia for possible stroke: A. Increasing lethargy B. Recent poor school performance C. Acute worsening of anemia
B. Recent poor school performance - silent cerebral infarct common in kids with sickle cell
93
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: A. Stroke B. Gall stones C. Nephropathy D. Asthma
A. Stroke
94
What is the most common complication of hereditary spherocytosis: a. aplastic anemia b. gallstones c. splenic infarcts d. frontal bossing
ANSWER: b. gallstones (most common) a. aplastic anemia (at risk if get parvoivrus B19 - have high turnover of RBCs so if marrow production is affected they're in trouble) d. frontal bossing (can get but more common in thal major (extra medullary hematopoiesis))
95
The severity of Rh incompatibility at the time of birth is best predicted by: a. cord Hb b. cord bilirubin c. prematurity d. splenomegaly e. maternal anti-D antibody
a. cord Hb
96
Kid with thalessemia trait. Most likely CBC findings.
Microcytic anemia with increased RBCs
97
What treatment is proven to prevent stroke in SCD? a. transfusion b. folate c. hydroxyurea d. Iron
a. transfusion Based on monitoring transcranial dopplers
98
Sickle cell anemia, which is true about strokes? a . usually subclinical strokes b. clinical stroke
a . usually subclinical strokes
99
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
b. chest xray Look for mediastinal mass - lymphoma
100
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 (TB test) c) Bartonella serology d) EBV serology
a) Excisional biopsy
101
3 yo with pallor, leg pain and fatigue for three weeks. Exam reveals tired, pale anicteric child with mild hepatosplenomegaly. No effusions or warmth/erythema of legs. Most likely to confirm dx? a. Abdominal ultrasound b. Bone marrow aspirate
b. Bone marrow aspirate
102
6 month old baby with pallor, hepatosplenomegaly, irritable. Xray shown with very white bones. Diagnosis: A. osteopetrosis B. osteoid osteoma C. rickets
A. osteopetrosis (infantile malignent osteopetrosis) - increased bone density leads to marrow replacement - pancytopenia
103
Which is true of infantile hemangioma? a. start to involute after first decade b. are not present at birth
b. are not present at birth
104
Child with hemihypertrophy. What condition would you suspect that would require serial follow-up?
Beckwith-Wiedemann
105
An 8 year girl comes with the following skin finding [picture of large café-au-lait macule]. List 4 other skin findings in this condition.
NF1 skin findings: - axillary or inguinal freckling - neurofibromas - Lisch nodules
106
Name the three categories of Autoimmune Hemolytic Anemia and labs and treatment.
**Autoimmune Hemolytic Anemia (AIHA) =** Antibody mediated destruction of RBC
* Categories:
* Warm = IgG Ab usually to Rh complex that react with RBC membrane at body temperature
* Cold = IgM Ab to polysaccharide antigens that react at low temps and cause lysis on rewarming (check cold agglutinin titres)
* Paroxysmal Nocturnal Hemoglobinuria = complement mediated intravascular hemolysis after *cold* exposure
* Labs = Positive DAT, increased retics, unconjugated bilirubinemia, low haptoglobin, increased LDH, urine dip with free Hgb
* Treatment: steroids 2mg/kg/day and wean over 4-6 weeks, transfuse (but can be hard to match blood), refer
