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Josh's RC Questions > Heme-Onc SAQ > Flashcards

Heme-Onc SAQ Flashcards

1
Q

A. 4 year old boy presents with several small bruises. He has a platelet count of 35 and a Hb of 120.

A. What is the diagnosis?

B. List two management options?

C. He presents a week later with recurrent nosebleeds. His platelets are now 12 and his Hb is 104. List two treatments?

A

A. ITP = Idiopathic thrombocytopenia purpura

B.

  1. Observe with follow up + family education
  2. IVIG or oral corticosteroids

C.

  1. IVIG 0.8-1mg/kg IV x 1 dose
  2. Prednisone 4mg/kg/d x4d w/o taper or 2mg/kg/d x1-2wk with taper
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2
Q

A 4 year old male comes in looking pale, tachycardic, and with abdominal pain and a large left-sided abdominal mass. Hgb 40. Sickledex screen is positive.

a) How do you confirm the underlying diagnosis?
b) What is the etiology of the acute presentation?
c) What is the treatment for the acute presentation?

A

a) Hb electrophoresis (for sickle cell disease)
b) Splenic sequestration
c) 1. Fluids, 2. Simple transfusion, 3. Pain control

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3
Q

A 2 year old boy has just immigrated to Canada. He was diagnosed with Sickle Cell disease at 1 year. In addition to referring to a Hematologist (nearest one is 500 km away) and confirming the diagnosis

What are FIVE essential things to do in the first visit.

A
  1. Review history of previous vaso-occlusive crisi, chest crisis, neurologic sequelae, transfusion history
  2. Review penicillin prophylaxis, hydroxyurea and folic acid. Start if not already on it
  3. Review immunizations and update if needed
  4. Do Hgb for baseline.
  5. Counsel family about pain, fever, difficulty breathing, stroke needs to come to ED
  6. Baseline renal + liver function tests, U/A, CXR, puse oximetry, ECHO. Arrange TCD, ophtho.
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4
Q

FIVE signs/symptoms of a child with an infratentorial tumor

A
  1. H/A
  2. N/V
  3. Ataxia - gait disturbance, poor coordination, nystagmus
  4. Papilledema, diplopia, blurred vision
  5. Torticollis

DDx

  1. Astrocytoma
  2. Brainstem glioma
  3. Medulloblastoma
  4. Ependymoma
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5
Q

Blood work consistent with HUS in a baby

A. Name 3 bugs

B. What’s the pathophysiology of anemia?

A

A.

  1. O1577H E coli (STEC)
  2. Neuraminidase producing strep pneumo
  3. Salmonella
  4. Shigella

B. Microangiopathic hemolytic anemia. Toxins cause microvascular injury with endothelial damage. RBCs get mechanically damaged as they pass through damanged + thrombotic microvasculature.

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6
Q

A 4 year old boy has bloody diarrhea then presents with petechiae and decreased urine output.

A. What is the likely diagnosis?

B. What is the pathogenesis of his anemia?

A

A. Hemolytic uremic syndrome

  1. MAHA
  2. Thrombocytopenia
  3. Renal failure

B. Microangiopathic hemolytic anemia. Toxins cause microvascular injury leading to endothelial damage. RBCs get mechnically damaged as they pass through damaged + thrombotic vasculature

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7
Q

Kid with sickle cell disease presents in respiratory distress, chest xray has two focal consolidation

A. What does he have

B. Name 4 management

C. What one medication would you give for prophylaxis?

A

A. Acute chest crisis

  • New infiltrate on CXR
  • AND 2 of 5
    • Fever >=38.5
    • Resp distress
    • Hypoxia
    • Cough
    • Chest pain

B.

  1. Give O2 as needed
  2. Simple transfusion
  3. Antibiotics
  4. Pain control
  5. Cautious IV fluids
  6. Incentive spirometry + chest physio

C. Hydroxyurea

  • Increases HbF levels
  • Decreases VOC, ACS, admission + transfusions by 50%
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8
Q

Exact picture above was shown

What are 5 physical features?

A

Neuroblastoma

  • Periorbital ecchymosis, orbital proptosis
  1. Localized: mass effects - Spinal cord compression, bowel obstruction, SVC syndrome
  2. Horner syndrome
  3. Opsoclonus myoclonus
  4. VIP induced secretory diarrhea (vasoactive intestinal peptide)
  5. Catecholamine release: flushing, HTN, sweating
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9
Q

Splenomegaly is very common in the pediatric population. A patient is diagnosed with splenomegaly in the context of an infectious mononucleosis.

At what point would it be safe to recommend a return to contact sports? (1 line)

A

Avoid contact sports + strenuous atheletic activities during first 2-3 wks of illness or while splenomegaly is present

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10
Q

Name TWO long-term complications of hemophilia.

A
  1. Chronic arthropathy
  2. Inhibitors against F8 or F9
  3. Transfusion-transmitted infectious diseases
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11
Q

Picture of a child with battle sign (dark bruises under eyes). Known to have abnormal eye movements described as dancing eyes. Give diagnosis.

A

Neuroblastoma

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12
Q

3 common childhood etiologies for neutropenia (aside from sepsis).

A
  1. Familial benign neutropenia
  2. Drug induced neutropenia
  3. Leukemia
  4. Shwachman-Diamond
  5. Bone marrow disorders
  6. Nutritional neutropenia (B12, folate, copper deficiency)
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13
Q

5 hr old baby with petechial rash. She is well otherwise. Platelets 9, WBC 10, Hgb 170. Mother 40 yo pregnancy normal and healthy. What is the differential diagnosis – List 3?

A
  1. Neonatal Alloimmune Thrombocytopenia
  2. Neonatal Autoimmune Thrombocytopenia (maternal ITP)
  3. Sepsis
  4. TAR
  5. WAS
  6. CAMT
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14
Q

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%.

a) What type of anemia is this [1 point]?
b) What test would you do to confirm [1 point]?
c) What treatment could you offer [1 point]?

A

a) Autoimmune hemolyic anemia

  1. Warm (IgG, extravascular hemolysis)
  2. Cold (IgM, “cold agglutinin disease”)
  3. Biphasic (IgG, “paroxysmal cold hemoglobinuria”)
  • Idiopathic
  • Secondary to drugs, infection (EBV, mycoplasma, CMV, HIV), autoimmune disease, immunodeficiency, lymphoproliferative diseases (lymphom)

b) DAT + peripheral smear
c) Transfusion (if oxygenation delivery issues) vs glucocorticoids (if severe hemolysis + significant anemia)

  1. Warm AIHA: supportive care, transfusion (“least incompatible”), steroids, ?IVIG, rituximab, splenectomy
  2. Cold or biphasic AIHA: supprotive care, transfusions. If severe, plasmapheresis.
  • Tends to remit spontaneously after a few weeks or months
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15
Q

12 yo girl presents with pallor, scleral icterus and shortness of breath. Exam – spleen 3 cm palpable. Labs – Hb 65, WBC normal, Platelets normal, total bilirubin 55g/L, direct bilirubin 3g/L.

a) What is the most likely diagnosis?
b) What investigation would you use to make this diagnosis?
c) What therapy would she benefit from?

A

a) Autimmune hemolytic anemia

  • 2-12yo
  • Onset can be acute with fever, jaundice, pallor, hemoglobinuria or more gradual with fatigue + pallor
  • Splenomegaly (primary site of IgG coated RBCs)
  • Profound anemia, increased ritcs
  • Normal plts + WBC

b) DAT + peripheral blood smear (spherocytes, polychromasia, nucleatic RBCs)
c) pRBC transfusion (if oxygenation delivery issues) vs Prednisone (if severe hemolysis + significant anemia)

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16
Q

Three treatments for hyperuricemia from tumour lysis syndrome.

A
  1. Hyperhydration
  2. Allopurinol
  3. Rasburicase
17
Q

A 3 year old child presents with a 4-5 day history of fever, URTI symptoms. CBC shows a Hgb of 120, WBC 2.0, Neutrophils 2%, Lymphocytes 80%, Monocytes 9%, Eosinophils 8%, Plt 300. Name 3 management steps.

A

Normal Hgb, low WBC, low neutro, high lymp, high plts

Febrile neutropenia - likely viral induced

  1. ABCs
  2. Draw BCx, NP swab
  3. Start empiric IV antibiotics
  4. Monitor CBC in 24-48H
18
Q

What are 4 biochemical manifestations of tumor lysis syndrome?

A
  1. High uric acid
  2. High phosphate
  3. High potassium
  4. Low calcium
19
Q

What are two serious/life threatening manifestations of a mediastinal mass, and how do they present? Question was in table form: (presentation, explanation)

A
  1. SVC syndrome
    • Presentation: increased IJV distention, plethora, shock
    • Explanation: compression of mass on SVC causing decreased preload and thus decreased cardiac output causing shock and increased venous backflow
  2. Extrinsic airway obstruction
    • Stridor, increased WOB, leaning forward b/c worse when supine, tachycardia, anxiety
    • Explanation: external compression of mass onto trachea
20
Q

Boy with edema, petechiae, etc. post gastro with bloody diarrhea. What’s diagnosis?

A

Hemolytic uremic syndrome

  1. MAHA
  2. Thrombocytopenia
  3. Renal failure
21
Q

7 yo boy from Kenya presents with tachypnea and chest pain. His lab work shows a hgb of 40.

a) What is the likely diagnosis?
b) What are four important management steps?

A

a) Acute chest crisis in context of sickle cell anemia

b)

  1. Admit, ABCs, continuous SpO2 monitoring, + O2
  2. CXR if not already done
  3. pRBC transfusion
  4. Antibiotics (3rd gen cephalosporin + macrolide = CFTX + azithro)
  5. Analgesia
  6. Cautious IV maintenance fluids
22
Q

A 4 year old boy presents with petechiae. He has a platelet count of 55 and Hb of 120.

a) How would you manage him?
b) He presents a week later with recurrent nosebleeds. His platelets are now 5 and his Hb is 80. What is your next step in management?

A

a)

  1. Observation with close F/U and parental education
  2. Give IVIG or steroid

b) Prolonged epistaxis and low pts at risk for ICH

  1. Admit: IVIG 0.8-1g/kg x1 + IV steroids
  2. Consider transexamic acid
  3. Consult hematology to consider BMA b/c now has 2 cell lines down
23
Q

Chart with CBC findings of hereditary spherocytosis. Has been asymptomatic. Now Hb 55, Retic 3%, WBC 3.8, Plt 196, Bili 30. Was 3 months ago Hb 86, Retic 11%, WBC 7.6, Plt 184, Bili 20.

A. What is the explanation? (1)

A
  • Aplastic crisis (parvo B19) or hypoplasic crisis (other infections) - at risk b/c high RBC turnover + heightened erythmoid marrow activity
  • Megaloblastic anemia
24
Q

A 16 year old female was treated for high risk ALL. She received cyclophosphamide and radiation. She is premenstrual and at Tanner Stg 1. She is at less than the 3rd%ile for height.

a) What are the two likely causes of her delayed puberty?
b) What three investigations would you order?

A

a)

  1. Premature ovarian failure due to chemo (cyclophosphamide causes infertility) or radiation
  2. Hypogonadotrophic hypogonadism due to LH/FSH deficiency (if received radiation to pituitary) or from chronic disease

b)

  1. LH, FSH, estradiol, prolactin, TSH + fT4, HCG
    • Consider GnRH stim
      • If LH/FSH 0, then worry that they’re not producing
      • If LH/FSH goes up a little bit, then already in puberty
      • If LH/FSH are super high and estrogen & testosterone are low, then gonads are not working
  2. AUS to ensure there is uterus + ovary present
  3. Bone age
25
Q

Teenage boy with tage 3 Hodgkin lymphoma, going to have chemo and radiation:

A. List two factors that may affect his fertility:

B. Two options for having children in the future:

A

A.

  1. Type of chemo + dose
  2. Radiation if near gonads
  3. Azoospermia as a paraneoplastic phenomenon

B.

  1. Sperm banking
  2. Adoption
  3. Donor sperm/IVF
26
Q

What are 6 long term side effects from hodgkin lymphoma treatment?

A
  • Radiation
    1. Secondary malignancy (breast, thyroid, skin)
    2. Hypothyroidism
    3. Cardiomyopathy (+ anthracycline)
    4. infertility (+alkylator)
  • Akylator
    • MDS/leukemia
  • Bleomycin
    • Pulmonary fibrosis
  • Steroid
    • AVN
27
Q

A 18 month old boy has been seen mouthing objects. He has a microcytic anemia which is unresponsive to iron therapy. His smear shows basophilic stippling. What test would you order next?

A

Serum lead level

Basophilic stippling

  • blue granules throughout cytoplasm of red cell
  • Ribosomal precipitates
  • DDx: lead + heavy metal, thalassemia, alcohol abuse
28
Q

A baby was born with severe hydrops. She has a large renal mass. She is on maximal ventilator and inotropic support. She has a very distended abdomen with poor perfusion to her lower limbs. Her electrolytes are within the normal range. She is about to go to surgery.

a) What would you tell her parents about her prognosis?
b) What are two complications you should expect in the intra-operative period?

A

A. If renal mass = Congenital mesoblastic nephroma

  • Most common renal solid tumour in the neonatal period
  • Most frequent benign renal tumour in childhood
  • Prenatal U/S: polyhydramnios, hydrops, prem
  • If assoc’d with hydrops fetalis, poor prognosis + usually fatal
  • Tx of choice: radical nephrectomy. Usually sufficient for cure

B.

  1. Injury to any of the GI organs
  2. Bleeding to major vessles
  3. Pleural injury -> pneumothorax
  4. HTN
  5. ARF/CRF
29
Q

Ways that hemophilia can present in a 1 month-old

A
  1. Bleeding with circumcision
  2. Bleeding from umbilicus or delayed cord falling off
  3. Bleeding with venipunctures
  4. ICH
30
Q

What are the 3 investigations for hereditary thrombosis?

A
  1. CBC + diff, INR, PTT
  2. Plt aggregation study
  3. Factor V leiden
  4. Antithrombin III
  5. Protein C+S
  6. Homocysteine
31
Q

List one associated malignancy with each of the following (not listed):

  1. Down syndrome
  2. Neurofibromatosis type 1
  3. Fanconi anemia
  4. Diamond blackfan anemia
  5. Shwachman diamond syndrome
A
  1. Down syndrome: TMD, ALL, AML
  2. Neurofibromatosis type 1: optic glioma, ALL
  3. Fanconi anemia: ALL, AML
  4. Diamond blackfan anemia: MDS+ AML
  5. Shwachman diamond syndrome: MDS + AML
32
Q

Child with microcytic hypochromic anemia. You believe it is Fe deficiency. What other features of CBC would be consistent with this? List 3.

A
  1. High RDW
  2. Low RBC
  3. Low retics
  4. Normal plt + WBC
33
Q

Child with Down syndrome. List three associated hematological disorders.

A
  1. Transient myeloproliferative disorder
  2. AML (Acute megalokaryoblastic leukemia0
  3. ALL
34
Q

List 4 indications for blood transfusion in sickle cell disease

A
  1. Acute chest crisis
  2. Splenic sequestration
  3. Aplastic crisis
  4. Prevent stroke (keep HbS <30%)
  5. Pre-op

Josh's RC Questions (39 decks)

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