Hem/Onc

Question 1

Normal pediatrics CBC levels vary based on ____ and _____

Answer 1

Age
Gender

Question 2

Hgb starts ___ at birth, and _____ ending at about _____ by 18 years.

Answer 2

High (16.5)
Downtrends
14-15

Question 3

Reticulocytosis can elevate and MCV. If you have an elevated MCV without a high retic - could indicate a primary bone marrow disorder.

Question 4

thalassemias have a ____ RDW which means the RBCs are standard size.

Answer 4

Normal

Question 5

IDA will have a _____ RDW. Why?

Answer 5

High.
Much variation in the sizes of the cells, because there’s not a dependable source of iron for their creation.

Question 6

What are reticulocytes?

Answer 6

Youngest RBCs in circulation.

Question 7

If you have anemia with HIGH reticulocyte count, then the bone marrow response is ______
Anemia with low retic - ____ bone marrow response/

Answer 7

Good
Poor

Question 8

What could cause a poor bone marrow response (low reticulocyte count)?

Answer 8

Marrow aphasia
Infiltration with malignant cells
Depression from infection
Nutritional deficiency

Question 9

What is the most reliable morphology indicator of the classification of anemia?
Most reliable physiologic indicator?

Answer 9

MCV (big, small or normal)
Reticulocyte count - what is the source of the anemia?

Question 10

Decreased RBC production would have a ____ retic
Increased blood loss (hemorrhage) or increased destruction (hemolytic anemia) would have a ____ retic

Answer 10

Low
High

Question 11

4 labs to draw in a basic anemia workup (and 1 secondary test)

Answer 11

CBC w/ Diff with smear
Reticulocyte count
Total/direct bili (asses for hemolysis)
DAT (assess for autoimmune hemolysis)

Secondary: iron panel.

Question 12

If you have a negative DAT, it could indicate a physiologic process (SCD, G6PD). If you have a positive DAT, it could indicate ______

Answer 12

Hemolysis related to autoimmune/immune mediated.

Question 13

Differentials for macrocytic anemia

Answer 13

Folic acid or B12 deficiency
Malabsorption
Surgical resection (of bowel)
Primary bone marrow disease

Question 14

Differential for normocytic anemia

Answer 14

Aplastic anemia (anemia secondary to bone marrow infiltration)
Anemia secondary to Hypothyroidism, renal disease or chronic illness
Transient erythroblastopenia of childhood (TEC)

Question 15

Differential microcytic anemia

Answer 15

IDA, thalassemias, sideroblastic anemia (mitochondrial disorder or lead/isoniazid Tox)
Anemia of chronic disease
Hgb E

Question 16

how will the CBC differ between IDA and thalassemia?

Answer 16

IDA has an increased RDW and a low reticulocyte count
Thalassemia has a low RDW and normal reticulocyte

Question 17

Why might you not want to measure ferritin during an acute illness?

Answer 17

It’s an acute phase reactor, so it will be falsely high.

Question 18

What is the overall best screen for total body iron on the iron panel?

Answer 18

Ferritin - fluctuates the least over time.

Question 19

MVC in kids should be _____ + age.

Answer 19

70

Question 20

How do you prescribe iron?
Dose?

Answer 20

With vit C - either OJ or gummies.
NOT with milk - this will prevent absorption.
4-6mg/kg
You absorb about 3mg/kg so if you’re on the upper end of 6, split BID doseing.

Question 21

Thalassemia is a ______ hbg disorder and SCD is a ______ Hgb disorder.

Answer 21

Quantitative
Qualitative.

Question 22

Beta thalassemia is most prevalent in ______ and alpha is most in _____

Answer 22

Mediterranean
Southeast Asian

Question 23

When do children with major thalassemias start to show s/s?

Answer 23

6-12mo when cross over in Hgb happens.

Question 24

High reticulocyte count in G6PD

Question 25

Class III is the most common class of G6PD

Question 26

Meds than can trigger hemolysis in G6PD

Answer 26

Aspirin in high doses, Macrobid, Antimalarial, primaquine, nitrate, sulfonamides, quinine.

Question 27

ITP most often happens ______

Answer 27

After a viral infection

Question 28

If patient with ITP has platelet count <10, need treatment. Treatment?

Answer 28

IVIG, Oral prednisone, IV anti-D (WinRho)

Question 29

Abnormal aPTT

Answer 29

Factor VIII (vWD) IX, XI, heparin

Question 30

Abnormal PT

Answer 30

Factor VII Coumadin Occ liver disease Occ DIC rVIIa effect

Question 31

Abnormal PT and aPTT

Answer 31

Common pathway factors (II, V, X) Fibrinogen Liver disease vit K deficient Coumadin DIC

Question 32

Doing a mixing study. if an inhibitor is present (such as lupus anticoagulant), then the result after the mix will be the same (long) as it was before the mix. If a factor deficiency is present, then the result will be normal after the mix.

Question 33

How are Ehler-Danilo’s and clotting related?

Answer 33

ED is a lack of collagen. Exposed collagen is what starts the clotting cascade. If it’s not there, then there might not be a clot.

Question 34

Hemophilia A is factor ____ deficiency Hemophilia B is _____ deficiency

Answer 34

VIII eight (Ay -> Ayte) IX nine

Question 35

VWD has a ______ bleeding pattern whereas hemophilias tend to have a _____ bleeding pattern.

Answer 35

Mucosal (nose, oral, gums, easy bruising) Soft tissue (welts large hematomas))

Question 36

Most common type of vWD - type 1 - a quantitative deficiency of vWF.

Question 37

SCD - clinically presents with anemia (normochromic, normocytic), high retic, high neutrophilia, high throbocytosis.

Question 38

Children with SCD get prophylactic _____ until age ____ and only after they’ve had all vaccines and have not had any significant infections.

Answer 38

Penicillin 5

Question 39

Other meds for SCD?

Answer 39

Folic acid supplementation Hydroxyurea (start at 9mo)

Question 40

What should the PCM do with a SCD (newborn screen)?

Answer 40

Start penicillin therapy (VK 125mg bid) and refer to heme.

Question 41

SCDpateints get a CXR every other year, a EKG/Echo every 1-2 years, PFTs every 1-2 years, eye exams every 1-2 years and an MRI/MRA and RUQUS prn

Question 42

Patients with sickle cell trait are more at risk for: 30 fold increase in risk of sudden death in black army recruits with SCT.

Answer 42

Heat stroke, heat exhaustion and rhabdo. VTE Renal medullary carcinoma ESRD Hematuria Impaired urinary concentration.

Question 43

Parents of a child with SCD or sickle cell trait are concerned that their child is going to the bathroom more than their peers. This is?

Answer 43

Normal. They don’t have as much ability to concentrate urine so they go more frequently.

Question 44

Most common cancer in children <15 is leukemia (ALL)

Question 45

Compared with adults, child cancer treatment often lacks _____

Answer 45

Radiation. Chemo alone is more common.

Question 46

Neutropenic precautions are initiated for ANC <1000. <500 is an automatic ______

Answer 46

Admission with broad spectrum IV abx (Ceftriaxone).

Question 47

What are the most common infectious pathogens in neutropenic cancer patients?

Answer 47

Those that come from elsewhere on the child’s body: Staph epideritis and aureus (MRSA), E. coli, enterococcus, Psuedomonas , fungus (aspergillosis, candida)

Question 48

Varicella, PJP, HSV can be serious in immunocompromised kids.

Question 49

Administration of neurogenic or neulasta for neutropenia _______ duration but does not _____

Answer 49

Shortens duration Eliminate it.

Question 50

At what point with weight loss would you get a nutritionist involved? How about appetite stimulant? TPN is last resort.

Answer 50

>5% = nutritionist, increase calories, PO supplements, antiemetics >10% = appetite stimulants, supplemental feeds (NG or Gtube).

Question 51

Enlarged ______ and ______ are always abnormal.

Answer 51

Epitrochlear and supraclavicular

Question 52

Warning signs with enlarged lymph nodes:

Answer 52

Firm,/hard,rubbery, non mobile ,non tender.

Question 53

Mediastinal masses could present with the following s/s:

Answer 53

Cough, dyspnea, orthopnea, strior, SVC syndrome (SOB on lying down)

Question 54

Concerning symptoms in pediatric headaches:

Answer 54

AM headache wakes them from sleep Incapacitating, vomiting, changing pattern of HA Abnormal Neuro exam Vision loss/change Head tilt Ataxia (gait/coordination abnormality) <3yo. Think brain tumor.

Question 55

At what point is the WBC high enough that it’s almost always leukemia?

Answer 55

>100,000

Question 56

What is the pathophys of tumor lysis syndrome and what 3 electrolyte abnormalities will result?

Answer 56

When the malignant cells of the tumor are lysed with chemo/radiation, their intracellular contents spill out: Hyperuricemia -> renal failure Hyperkalemia -> arrhythmias Hyperphosphatemia -> hypocalcemia

Question 57

Tx for ALL leukemia AML leukemia

Answer 57

Chemo Chemo and stem cell transplant

Question 58

If ALL is diagnosed between age 1 and 10 with WBC < 50,000, then prognosis is better.

Question 59

Back pain in a child - never normal! Do not miss. Could be a sign of cancer. Get MRI.

Question 60

How does a Wilms tumor usually present?

Answer 60

Asymptomatic abdominal mass in flank in well appearing child.

Question 61

Biggest difference in presentation between wilms tumor and Neuroblastoma tumor?

Answer 61

Wilms - asymptomatic, healthy happy kid Nueroblastoma? Sick kid.

Question 62

Most common presentation of rhabdomyosarcoma?

Answer 62

(Soft tissue malignancy that can involve ANY skeletal muscle including eye muscles) Painless, enlarging mass, symptoms related to mass, head, neck, orbit, extremities, GU, trunk.

Question 63

Osteosarcoma is resistant to radiation. Distal has better prognosis as well as stage.