Peds Heme/Onc

Question 1

Aplastic anemia- Eti

Answer 1

• Idiopathic most common- defects in hematopoietic stem cells & DNA repair

Question 2

Aplastic anemia- Sx

Answer 2

• Weakness & fatigue
• Vulnerability to infections
• Mucosal & skin bleeding
• Pallor, purpura & petechiae
• NO hepatosplenomegaly, lymphadenopathy

Question 3

Aplastic anemia- Dx

Answer 3

• Pancytopenia

- BM biopsy- scant progeitors

Question 4

Aplastic anemia- Tx

Answer 4

• Equine ATG

- Bone marrow transplant

Question 5

Thalassemia- Eti

Answer 5

• Hereditary disorder
• Redcution in globin chains, reducing hemoglobin synthesis
  Alpha & Beta
• Alpha in SE asia/ china, blacks
• Beta- mediterranean

Question 6

Thalassemia- Sx

Answer 6

• Microcytic anemia
  Alpha- Asymptomatic to chronic hemolytic anemia
• Beta- Severe anemia, growth failure, bony deformaties

Question 7

Thalassemia- Dx

Answer 7

• Increased RBC count

Question 8

Thalassemia- Tx

Answer 8

• Folate & blood transfusion

- Splenectomy with hypersplenism

Question 9

Sickle cell- Eti

Answer 9

• Autosomal recessive
• Sickling of RBCs due to aa substitution
• American blacks
• Onset in first year of life

Question 10

Sickle cell- Sx

Answer 10

• Chronic hemolytic anemia
• Pain in bones and chest, produce low grade fever
• Vaso-occlusion

Question 11

Sickle cell- Dx

Answer 11

• Genetic testing & peripheral blood smear

- Hemoglobin electrophoresis

Question 12

Sickle cell- Tx

Answer 12

• Folic acid

- Hodroxyurea

Question 13

Iron deficiency anemia- Eti

Answer 13

• Low serum ferritin
• Most common cause of anemia worldwide
• Due to bleeding, celiac sprue, deficient diet

Question 14

Iron deficiency anemia- Sx

Answer 14

• Microcytic anemia
• Fatigue, dyspnea, palpitations, tachypnea on exertion
• Smooth tounge, brittle nails, spooning
• Pica

Question 15

Iron deficiency anemia- Dx

Answer 15

• Serum ferritin

Question 16

Iron deficiency anemia- Tx

Answer 16

Iron supplementation

Question 17

G6PD deficiency- Eti

Answer 17

Sporatic hemolysis with drugs/ fav beans

• Asian & africans
• Protects from malaria

Question 18

G6PD deficiency- Sx

Answer 18

X-linked

• Episodic hemolysis with oxidant stress
• Pallor, jaundice, hyperbilirubinemia

Question 19

G6PD deficiency- Dx

Answer 19

Heinz bodies, decreased G6PD in erythrocytes

Question 20

G6PD deficiency- Tx

Answer 20

• Avoid drugs, fav

- Phototherapy

Question 21

Idiopathic thrombocytopenic purpura- Eti

Answer 21

• Most common bleedign disorder in children
• 2-5 yo, post viral
• Decreased platelets

Question 22

Idiopathic thrombocytopenic purpura- Sx

Answer 22

• Petechiae
• Eccymosis
• Epsitaxis

Question 23

Idiopathic thrombocytopenic purpura- Dx

Answer 23

Decreased platelets, increased megakaryocytes

Question 24

Idiopathic thrombocytopenic purpura- Tx

Answer 24

• None, avoid ASA & NSAIDs

Question 25

Hemophilia- Eto

Answer 25

Reduction in factor VIII

Question 26

Hemophilia- Sx

Answer 26

- Easy brushing - Unable to stop bleeding - Hemearthrosis - Blood in joints, muscles, viscera - ABn joint fxn

Question 27

Hemophilia- Dx

Answer 27

Prolonged PTT

Question 28

Hemophilia- Tx

Answer 28

Treat with factor VIII Desmoproessin - Avoid high risk activities

Question 29

Von willebrand- Eti

Answer 29

- Inherited bleeding disorder | - Caucasians

Question 30

Von willebrand- Sx

Answer 30

Epistaxis - Easy bruising -

Question 31

Von willebrand- Sx

Answer 31

- Epistaxis - Easy bruising - Tx with desmopression and add clotting factors

Question 32

Acute lymph leukemia- Eti

Answer 32

Most common malig of childhood Lymphocytes / bone marrow - Peak at 4 yrs - Increased risk down syndrome

Question 33

Acute lymph leukemia- Sx

Answer 33

- Fever, infection, pallor, petechiae, bone pain | - Hepatosplenomegaly

Question 34

Acute lymph leukemia- Dx

Answer 34

Cytopenia | CXR: mediastinal widening, long bone demineralization

Question 35

Acute lymph leukemia- Tx

Answer 35

Prenisone

Question 36

Acute myeloid- Eti

Answer 36

- Myeloid cells | - More common in adults

Question 37

Hodgkin- Eti

Answer 37

Common in kids, adolescence

Question 38

Hodgkin- Sx

Answer 38

- Painless cervical lymphadenopathy - Mediastinal mass - Wt loss, nt sweats

Question 39

Non-hodgkin- Eti

Answer 39

- Immunocompromised kids

Question 40

Non-hodkin- Sz

Answer 40

- Cough, dyspnea, swelling of face | - Abd pain, ascites

Question 41

CNS tumors- Eti

Answer 41

Astrocytoma - Rare - Most common solid tumor

Question 42

CNS tumors- Sz

Answer 42

- Morning HA - Vomiting - Papilledema - Increased head circumference - CN palsies

Question 43

CNS tumors- Sz

Answer 43

- Morning HA - Vomiting - Papilledema - Increased head circumference - CN palsies

Question 44

Osteosarcoma- Eti

Answer 44

- Affects long bones - Taller than peers - rapid bone growth, pain, decreased ROM

Question 45

Ewing scarcoma

Answer 45

- 20 yr old white dudes - Small malignant in diaphysis of long bones - Pain at site of tumor