Heme/Onc Flashcards
How much iron should be in formula for normal BW infant for the first 9-12 months?
High-risk for iron deficiency infant?
6.5-13mg/L
13mg/L
List 5 risk factors for iron-deficiency in a child under 2 years
- 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
List 2 effective measures to reduce iron deficiency
- 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)
Who should receive additional iron supplementation?
When should it start?
How much should they receive?
What duration of time?
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
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?
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)
What are the types of Vitamin K deficiency of the newborn?
- 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
What are the categories of brain tumours and their presenting symptoms?
- 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:
What are the definitions, symptoms and treatment for pituitary adenomas?
- 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
What are the most common brain tumours?
- 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)
What is the significance of Factor XII deficiency?
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
List the differences between Fanconi anemia, Schwachman-Diamond syndrome, Dyskeratosis congenita and Diamond-Blackfan anemia.
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)
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?
- 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
Transient fever in blood transfusions is usually a result of: a. sensitization to WBC antigens b. acute GVHD c. hepatitis C d. hemolysis
a) sensitization to WBC antigens (leukoreduction helps prevent this) - hemolytic reactions can cause fever but are not transient and not the the most common
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
c. Stop transfusion, run IV TKVO Febrile non-hemolytic transfusion reaction - stop the transfusion, give antipyretics and monitor
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
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
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. 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
Which of the following is present in tumour lysis syndrome: a) hyperuricemia b) hyponatremia c) hypokalemia d) hypophosphatemia e) hypercalcemia
a) hyperuricemia HyperPO4, hyperK, hypoCa
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)
- posterior fossa (brainstem and cerebellum) 2. cerebellar astrocytoma and medulloblastoma
2 life threatening presentations of anterior mediastinal mass?
- SVC syndrome (facial or upper extremity swelling from vascular compression) - cardiac tamponade - respiratory arrest from airway compression
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) bone marrow aspirate
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
C. reassure mother that condition may last for 4-12 weeks Breast milk jaundice
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
B. Hodgkin’s lymphoma
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. DIC work-up Platelets, INR (normal-high), PTT (normal-high), fibrinogen (low), D-dimer (high) - Kasabach Merritt
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. Brain Tumour
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
Name 3 syndromes associated with increased risk of leukaemia:
NF1 T21 Fanconi anemia Schwachman Diamond ataxia telangiectasia
Left supraclavicular LN - mgmt? a. Excisional biopsy b. monitor c. EBV serology
a. Excisional biopsy
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
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
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
Opsoclonus is seen in which of the following: a. AML b. ALL c. Neuroblastoma d. Medulloblastoma e. Rhabdomyosarcoma
c. Neuroblastoma
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)
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)
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
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
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
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
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
List 3 side effects of L-aparaginase
pancreatitis, hyperglycaemia, platelet dysfunction, coagulopathy
List 3 side effects of Vincristine
local cellulitis, peripheral neuropathy, constipation, SAIDH
List 3 side effects of Prednisone
hypertension, T2DM, increased infection risk, AVN
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
- 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