Heme/Onc

Question 1

Risk factors for polycythemia in a neonate - (4+)

Answer 1

During gestation, erythropoietin production is stimulated by hypoxemia. Risk factors for polycythemia among neonates include delivery in high-elevation areas; gestational age of more than 40 weeks; small size for gestational age; maternal diabetes; maternal hypertension; maternal history of cigarette smoking; trisomy 13, 18, or 21; neonatal Graves disease; congenital adrenal hyperplasia; cyanotic congenital heart disease; and neonatal hypothyroidism.

Question 2

Irritable, tachypneic neonate with hypoglycemia, thrombocytopenia, and poor feeding with hct of 62.

Answer 2

Consider viscosity associated consequences of polycythemia. Can monitor and treat with IV hydration vs. consider PET.

Question 3

Define polycythemia. Neonates with polycythemia should be treated if and with?

Answer 3

1. Hct >65%.
2. Partial exchange transfusion if they are symptomatic (cyanosis, tachypnea, poor feeding) with a Hct greater than 60% or have a Hct greater than 70%, whether symptomatic or not. The long-term complications of polycythemia may be related to the effects of hypoxia during gestation.

Neonatal blood and viscosity peak between 2-4 hrs of life!

Question 4

SVC syndrome + mediastinal mass in a child with respiratory distress (no heart failure). Greatest concern with regard to maintaining airway?

Answer 4

SVC and R atrial collapse. Factors preventing collapse of the right atrium include gravity (do not lie supine) and negative intrathoracic pressure (no sedation).

Question 5

Symptomatic anemia and marked reticulocytosis following Bactrim.

Peripheral smear?

Answer 5

G6PD: blister cells and polychromatic macrocytes on peripheral smear.

Often see Heinz bodies.

Question 6

Heinz body/ Bite cell differential

Answer 6

G6PD, oxidant drugs, unstable hemoglobin

Heinz body is basically hemoglobin

Question 7

Howell Jolly body differential

Answer 7

DNA bodies found in hyposplenism, severe hemolytic anemia

Question 8

Burr cell

Answer 8

Uremia, liver disease

Question 9

Target cell RBC

Answer 9

thalassemia, hemoglobinopathy, post-splenectomy, liver disease

Question 10

Tear drop cell

Answer 10

myelofibrosis or underlying marrow process/infiltrate

Question 11

For mismatch repair deficiency associated tumors, can use ___ (chemo) to treat.

Answer 11

Immunotherapy, such as pembrolizumab.

ALWAYS check for MSI in colorectal cancers, especially if Stage II (full thickness). Adjuvant therapy is not indicated if positive since they are low risk for recurrence.

Question 12

Epidermal growth factor receptor inhibitors such as panitumumab or cetuximab are inappropriate for KRAS or RAS gene mutations because?

Answer 12

They are only active in tumors with nonmutated KRAS, NRAS and BRAF genes.

Question 13

Post-colon cancer management (primary care):

1. Imaging
2. Labs
3. Colonoscopy q? for rectal cancer and CRC

Answer 13

1. Imaging - repeat CT scans, q6-12 mo within 5 years.
2. Labs: repeat CEA q6 months
3. Repeat colonoscopy q3-6mo for first 2-3 years after surgery if at increased risk of recurrence for rectal cancer, 1 year for CRC then q3-5

Question 14

Amsterdam II criteria for Lynch syndrome

Answer 14

• at least 3 family members
• one of which is a first degree relative of the other two
• diagnosed under age 50 - and no FAP

Question 15

Secondary polycythemia to testosterone: hold if __ > __%

Answer 15

hematocrit > 54%

Question 16

__ can be used post-renal transplant to treat secondary polycythemia

Answer 16

Lisinopril - Posttransplant erythrocytosis occurs in up to 15% of patients after kidney transplantation. ACE inhibitors reduce erythrocytosis in 90% of patients in a dose-response manner.

Question 17

Lynch syndrome: if diagnosed in a family member - screening guidelines for the patient? (3)

Hint:
Colonoscopy at age__ and repeat __
If female, consider __
EGD at age __ and repeat ___; also test for __

Answer 17

1. Colonscopy screening beginning age 20-25 or 2-5 years before earliest known diagnosis and repeat q1-2 years
2. Total hysterectomy and BSO after age 40
3. Screen with EGD at age 30-35 and repeat q3-5 years, also test for H. pylori

Question 18

angulated or net-like config that reflects the vascular structure in the skin, often brick red or purple is __, a manifestation of disrupted __. Consider __ causes first; one drug-related cause is __, which can be associated with ___ (underlying disorder that results in thrombus formation).

Answer 18

Retiform purpura = disrupted arterial flow; consider thrombus or emboli.

Warfarin-induced skin necrosis is associated with Protein C deficiency (due to transient hyper-coagulable state).

IF so, d/c the warfarin, give vitamin K/ FFP, and use alternative AC.

Question 19

Congenital deficiency -> delayed bleeding after surgery or trauma, heavy menstrual bleeding and often preterm labor/miscarriage, delayed wound healing, cardiac fibrosis

Answer 19

plasminogen activator inhibitor-1 (PAI-1) deficiency

Question 20

Genetic counseling and BRCA testing should be recommended in patients with a strong family history of these cancers - breast, ovarian, __, and __.

Answer 20

Breast, ovarian, pancreatic and prostate with Gleason >7

Question 21

When to use adjuvant chemotherapy for breast cancer?

Answer 21

Hormone negative or HER2+ cancers that are greater than 5 mm in size, lymph node positive, or both

Question 22

Multiple family members with lobular breast cancer or gastric cancer. Next step in management?

Answer 22

Endoscopy for inherited gastric cancer + check for CDH1 mutation. Consider prophylactic gastrectomy in those with the mutation

Question 23

ITP evaluation in adults

Answer 23

Immune thrombocytopenic purpura can be idiopathic, triggered by medications, or associated with other disorders, such as systemic lupus erythematosus, chronic lymphocytic leukemia, lymphoma, HIV, hepatitis C, or Helicobacter pylori infection.

Question 24

CML associated with this gene switch and tested with a tyrosine kinase inhibitor called?

Answer 24

BCR-ABL and imatinib

Question 25

In patients with myelodysplastic syndrome requiring frequent transfusions, supplemental treatments to help decrease transfusion requirements, such as—-, should be used to improve quality of life and decrease transfusion-associated iron overload and alloimmunization.

Answer 25

Lenolamide (a hypomethylating agent). Only true care is a hsct transplant.

Question 26

Treatment for polycythemia Vera?

Answer 26

Phlebotomy for hct <45, hydroxyurea, or ruxolitinib