Heme/Onc

Question 1

Hemorrhage and hemolysis will show what on a CBC?

Answer 1

high reticulocyte count

Question 2

When does a normal physiologic nadir occur in Hgb?

Answer 2

8-10wks of life

Question 3

What is the DDx for microcytic anemia?

Answer 3

Fe def. anemia, thalassemia, sickle cell anemia, chronic dz, lead intox, copper def.

Question 4

What it the most common cause of anemia?

Answer 4

Iron deficiency

Question 5

When do we screen for iron def. anemia?

Answer 5

12-15mos

Question 6

What is the Tx for iron def. anemia?

Answer 6

4-6mg/kg/d iron

Question 7

When do we screen for lead poisoning?

Answer 7

12 months in high risk populations

Question 8

How might lead poisoning might present?

Answer 8

Pica, abd pain, CNS change, microcytic/hypochromic anemia, basophilic stippling

Question 9

When is chelation indicated in lead poisoning?

Answer 9

blood lead level >45ug/dL, re-test in 48hrs.

Question 10

Alpha thalassemia causes what fatal condition?

Answer 10

hydrops fetalis–missing 4 genes –/–

Question 11

What are sx’s of hemoglobin H dz(–/-a)?

Answer 11

alpha thalassemia variant-3 of 4 genes missing, chronic hemolytic anemia, neonatal jaundice

Question 12

When does Beta thalassemia present?

Answer 12

btwn 4-12mos of life when nml hgb should be replacing fetal hgb

Question 13

What are sx’s B-thalassemia major?

Answer 13

skeletal changes/extrameduallary hematopoeisis, splenomegaly, liver/gallbladder dz, aplastic crisis, cardiopulmonary d/o

Question 14

What’s the tx of B-thalassemia major?

Answer 14

chronic transfusions, chelation from iron overload

Question 15

What is the life span of sickle cells?

Answer 15

17days vs 120 in normal RBCs

Question 16

What are manifestation of sickle cell?

Answer 16

dactylitis, hemolytic anemia, vaso-occlusive crisis, acute chest syndrome, chronic pain, priapism, hepatobiliary dz, bone pain/infarction, functional asplenia

Question 17

When does splenic infarction occur?

Answer 17

2-4yo

Question 18

Sickle cell tx?

Answer 18

iron, folate, transfusion, chelation, hydroxyurea (promotes Hgb F), stem cell transplant

Question 19

What is the MC RBC enzyme defect that causes hemolytic anemia?

Answer 19

G6PD deficiency

Question 20

What are sx’s of G6PD Def.?

Answer 20

neonatal jaundice, dark urine, no sx’s, , gallstones, splenomegaly

Question 21

What lab findings do you expect w/oxidant stress in G6PD Def?

Answer 21

hemolytic anemia, bite cells, Heinz bodies, elevated ESR, spherocytes

Question 22

Tx of G6PD Def.?

Answer 22

remove stressor, no fava beans, transfusion, avoid sulfas/nitrofurantoin

Question 23

What is autoimmune hemolytic anemia?

Answer 23

acute, self limited hemolysis following an infection or drugs

Question 24

Sx’s of hemolytic anemia?

Answer 24

jaundice, fatigue, pallor, dark urine, splenomegaly

Question 25

Labs in autoimmune hemolytic anemia?

Answer 25

normochromic/normocytic anemia, elevated reticulocytes, LDH, AST, urobilinogen, + Coomb's test

Question 26

Tx for autoimmune hemolytic anemia?

Answer 26

>80% resolve, IVIG, steroids, transfusion

Question 27

Which primary combined immunodeficiency causes eczema, eosinophilia, staph abscess, ostepenia, fractures

Answer 27

hyper IgE syndrome

Question 28

MC bleeding d/o in childhood?

Answer 28

Idiopathic thrombocytopenic purpura

Question 29

MC tumor of infancy?

Answer 29

neuroblastoma

Question 30

How does neuroblastoma present?

Answer 30

abd mass, miosis, anhydrosis, ptosis, constitutional sx's, dancing eyes, dancing feet

Question 31

Second most common abdominal tumor?

Answer 31

Wilm's tumor/nephroblastoma

Question 32

How does Wilm's present?

Answer 32

smooth abd. mass, HTN, microscopic hematuria, usually around age 3

Question 33

MC malignancy in childhood?

Answer 33

acute lymphoblastic leukemia ALL

Question 34

How does ALL present?

Answer 34

fever, bone pain, petechiae, HSM, LAD, respiratory sx's...bone marrow is gold standard Dx