Ped Hem/Onc Kate

Question 0

peripheral pancytopenia w/ hypocellular bone marrow

Answer 0

acquired aplastic anemia

Question 1

most common inherited anemia?

Answer 1

Congenital Aplastic Anemia- Fanconi anemia

Question 2

defective DNA repair that is caused by genetic mutation- pancytopenia

Answer 2

congenital aplastic anemia

Question 3

weakness, fatigue, pallor, frequent infections, purpura, petechiae, bleeding, neutropenia, low platelet count

Answer 3

acquired aplastic anemia

Question 4

hx of pica w/ decreased serum ferritin, serum iron, increased TIBC

elevated RDW

Answer 4

iron deficiency anemia

Question 5

megaloblastic anemia finding on peripheral smear

Answer 5

hypersegmented neutrophil

Question 6

spherocytes on peripheral smear indicate

Answer 6

hereditary spherocytosis

Question 7

a-thalassemia severity is related to?

Answer 7

of gene deletions

Question 8

B-minor thalassemia presents?

Answer 8

asymptomatic, mild, microcytic hypochromic anemia

Question 9

B-major thalassemia presents?

Answer 9

homozygous for B-globin mutation, early death

Question 10

chronic hemolysis: pallor, fatigue, jaundice, & development of gallstones during childhood & adolescence
Pain due to vaso-occlusion

Answer 10

sickle cell

Question 11

labs showing thrombocytopenia and/or neutropenia, followed by progression to pancytopenia

Answer 11

congenital aplastic anemia

Question 12

short stature, cafe-au-lait spots, cephalic issues, developmental delay

Answer 12

congenital aplastic anemia

Question 13

peripheral pancytopenia w/ hypocellular bone marrow

low reticulocyte count

Answer 13

acquired aplastic anemia

Question 14

what is the most common anemia

Answer 14

iron deficiency anemia

Question 15

heinz bodies on peripheral smear?

Answer 15

G6PD

Question 16

osmotic fragility test?

Answer 16

detects elevated RBC hemolysis

for hereditary spherocytosis

Question 17

what are the two diagostics for hereditary spherocytosis

Answer 17

spherocytes on smear

osmotic fragility test

Question 18

lead poisoning shows what on peripheral smear?

Answer 18

basophilic stippling

Question 19

primary polycythemia is

Answer 19

congenital erythrocytosis- elevated RBCs

Question 20

secondary polycythemia is

Answer 20

in response to hypoxemia

Question 21

most common cause of secondary polycythemia

Answer 21

cyanotic congenital heart disease

Question 22

what is the most common bleeding disorder of childhood

Answer 22

idiopathic thrombocytopenic purpura

Question 23

ITP etiology?

Answer 23

often follows viral infection

Question 24

most common inherited bleeding disorder?

Answer 24

Von Willibrand disease

Question 25

Petechiae, ecchymosis, epistaxis

Answer 25

ITP

Question 26

hemophilia A is

Answer 26

factor VIII deficiency

Question 27

hemophilia B is

Answer 27

factor IX deficiency

Question 28

which hemophilia is most common

Answer 28

A

Question 29

most common inherited thrombotic condition (in caucasians, anyways)

Answer 29

Leiden mutation

Question 30

palpable purpura on lower extremities, arthralgias, abd. pain, renal disease

Answer 30

Henonch-Schonlein Purpura (HSP)

Question 31

most common small vessel vasculitis

Answer 31

HSP

Question 32

inherited thrombotic disorders (4)

Answer 32

protein C deficiency protein S deficiency antithrombin deficiency Leiden mutation

Question 33

complications of thrombotic disorders

Answer 33

DVT, PE

Question 34

uncontrolled proliferation of immature lymphocytes

Answer 34

ALL

Question 35

complications of tumor lysis syndrome

Answer 35

hyperkalemia hyperuricemia hyperphosphatemia ---> acute renal failure

Question 36

CBC with WBC > 50,000/uL in 20% of pts

Answer 36

ALL

Question 37

25% of pts have WBC > 100,000/uL

Answer 37

AML

Question 38

Auer rods on peripheral smear indicate

Answer 38

AML

Question 39

where will you see blasts on the peripheral smear

Answer 39

ALL & AML

Question 40

Anemia, thrombocytopenia, leukocytosis, relatively few blasts

Answer 40

CML

Question 41

most common solid tumor of childhood

Answer 41

brain tumor

Question 42

most common brain tumor

Answer 42

glial tumor | astrocytomas & ependymomas

Question 43

deadlier, more aggressive brain tumor

Answer 43

medulloblastoma

Question 44

malignant marker cells in Hodgkin Disease?

Answer 44

Reed-Sternberg Cells

Question 45

painless cervical or supraclavicular adenopathy, mediastinal mass, weight loss, fever, night sweats, fatigue, splenomegaly, hepatomegaly

Answer 45

Hodgkin Disease

Question 46

LAD, abd pain, fevers, cough, dyspnea, weight loss, night sweats All developing over 1-3 weeks

Answer 46

Non-Hodgkin Lymphoma

Question 47

most common solid neoplasm outside the CNS

Answer 47

neuroblastoma

Question 48

labs w/ anemia & increased urinary catecholamines

Answer 48

neuroblastoma

Question 49

fever, weight loss, abdominal mass, irritability, bone pain, hematuria, HTN

Answer 49

nephroblastoma

Question 50

nighttime bone pain

Answer 50

Ewing's Sarcoma

Question 51

most common soft tissue sarcoma in kids

Answer 51

rhabdomyosarcoma

Question 52

enlarging abdomen, elevated aFP

Answer 52

hepatic tumor

Question 53

bone tumor that occurs most often in the pelvis, long bones | minority in soft tissue

Answer 53

Ewing's Sarcoma

Question 54

normal platelet count elevated PT, aPTT decreased fibrinogen

Answer 54

liver disease

Question 55

weakness, fatigue, purpura, petechiae, frequent infections, bleeding

Answer 55

acquired aplastic anemia