Hematology/Oncology

Question 1

three reasons for anemia

Answer 1

reduced production
increased destruction
loss of blood

Question 2

When would you expect a low retic count?

Answer 2

Iron def anemia
ALL
Diamond Blackfan anemia

Question 3

when would you expect a high retic count?

Answer 3

Hemolysis

Bloos loss

Question 4

Intrinsic hemolysis

Answer 4

hereditary spherocytosis
G6PD deficiency
hemoglobinopathies

Question 5

Extrinsic hemolysis

Answer 5

AIHA
DIC
IV hemolysis

Question 6

most common childhood hematologic problem

Answer 6

anemia

Question 7

Kid comes in with sxs of anemia and is jaundice and splenomegaly. What kind of anemia do you think?

Answer 7

hemolytic

Question 8

Kid comes in with tachycardia, flow murmur, poor exercise tolerance, headache, fatigue, irritability, poor feeding and syncope

Answer 8

acute anemia

Question 9

This anemia is well tolerated in kids, may show up with tachycardia or a flow murmur

Answer 9

chronic anemia

Question 10

peripheral blood smear shows us

Answer 10

size, shape, inclusions

Question 11

iron studies include

Answer 11

serum Fe, ferritin, TIBC

Question 12

hypochromic, microcytic RBCs

Answer 12

Fe deficiency

Question 13

schistocytes associated with

Answer 13

HUS

Question 14

Blicter cells associated with

Answer 14

G6PD

Question 15

Sickle cells associated with

Answer 15

Hgb SS

Question 16

spherocytes associated with

Answer 16

autoimmune hemolytic

Question 17

most common anemia in childhood

Answer 17

Fe deficient anemia

Question 18

treat Fe deficiency anemia

Answer 18

oral Fe 6mg/kg/day divided TID

Question 19

macrocytic (megaloblastic) anemia

Answer 19

Folate

B12 deficiency

Question 20

B12 absorbed where

Answer 20

terminal ileum (watch with crohns)

Question 21

Folate absorbed where

Answer 21

jejunum (watch with celiac)

Question 22

2-10yr olds at diagnosis. Congenital anomalies in ~ 50% (skeletal, renal, microcephaly, hypogenitalia) pancytopenia (AR)

Answer 22

Fanconi

Question 23

birth – 1 yr, responds to steroids. (prednisone 2mg/kg/day) short stature and congenital anomalies in ~ 30% pt. (AD/AR)

Answer 23

Diamond Black-Fan

Question 24

pancytopenia with hypocellular bone marrow. Sx related to anemia or thrombocytopenia, fevers. No HSM, no jaundice

Answer 24

acquired aplastic anemia

bone marrow not making enough stuff

Question 25

test for isoimmunization

Answer 25

Coombs direct looks for ab on RBC indirect looks for ab in sera

Question 26

AD disorder, splenic sequestration of abnormal cells. Anemia, jaundice, and splenomegaly occur

Answer 26

hereditary spherocytosis

Question 27

treat hereditary spherocytosis

Answer 27

take out spleen

Question 28

most frequent inherited enzyme defect

Answer 28

G6PD deficiency

Question 29

X-linked recessive (males) Anemia, hyperbilirubinemia* jaundice Episodic hemolysis with oxidative stress

Answer 29

G6PD

Question 30

2nd most common inherited enzyme defect

Answer 30

pyruvate kinase deficiency

Question 31

Quantitative deficiency in production of globin chains (alpha or beta)

Answer 31

thalassemia

Question 32

Associated with Hb S, elevated retic count (normo or macrocytic)

Answer 32

sickle cell disease

Question 33

disorders of coagulation

Answer 33

hemophilias vitamin k deficiency DIC

Question 34

most common type of thrombocytopenia

Answer 34

increased consumption | Idiopathic thrombocytopenic purpura

Question 35

types of increased consumption thrombocytopenia

Answer 35

ITP DIC Kasabach-Merritt syndrome

Question 36

most common bleeding d/o of childhood

Answer 36

ITP

Question 37

Petechaie, ecchymoses, decreased platelet count, otherwise healthy child. Often follows a viral infection (rubella, varicella)

Answer 37

ITP

Question 38

treat ITP

Answer 38

IVIG best response | steroids good response

Question 39

hemophilia A

Answer 39

factor VIII

Question 40

hemophilia B

Answer 40

factor IX | Christmas disease

Question 41

treat factor VIII deficiency

Answer 41

desmopressin (or order factor VIII)

Question 42

treat factor IX deficiency

Answer 42

give factor IX

Question 43

Most common inherited bleeding disorder among Caucasians – autosomal dominant

Answer 43

von willebrand

Question 44

treat VWD

Answer 44

desmopressin

Question 45

clotting d/o's are uncommon in kids T or F

Answer 45

TRUE

Question 46

Diseases in which you have a defect in glucuronyl transferase

Answer 46

gilbert's | crigler naijar

Question 47

Abnormal gene promoter leading to decreased transcription of mRNA, leading to less GT enzyme

Answer 47

Gilbert's

Question 48

treat Gilbert's

Answer 48

No treatment needed except in neonate with excessive hyperbilirubinemia

Question 49

type 1 crigler-naijar

Answer 49

gene mutation- no GT activity- letha

Question 50

type 2 crigler naijar

Answer 50

diminished GT activity, treat with PB

Question 51

two main cancers seen in peds

Answer 51

leukemia | brain tumors

Question 52

Most common cause of death by disease

Answer 52

cancer

Question 53

most common leukemia

Answer 53

ALL

Question 54

least common leukemia

Answer 54

CML

Question 55

associated with philadelphia chromosome

Answer 55

chronic myelogenous leukemia

Question 55

associated with philadelphia chromosome

Answer 55

chronic myelogenous leukemia

Question 56

most common lymphoma

Answer 56

Hodgkin

Question 56

most common lymphoma

Answer 56

Hodgkin

Question 57

painless lymphadenopathy, mediastinal mass, fatigue, cough DOB, anorexia, occurs mostly in teenagers

Answer 57

hodgkin

Question 57

biopsy lymph node in hodgkin to look for what

Answer 57

Reed Sternberg cells (malignant B cells)

Question 57

common nodes hodgkin

Answer 57

cervical or supraclavicular

Question 57

classic triad brain tumor

Answer 57

morning HA, vomiting, papilledema

Question 57

most common tumor of childhood

Answer 57

astroytoma

Question 57

neural crest tissue of sympathetic ganglia or adrenal medulla most common solid neoplasm outside CNS

Answer 57

neuroblastoma

Question 57

most common presentation for a neuroblastoma

Answer 57

abdominal pain or mass

Question 57

2nd most common solid tumor, 1st most common abdominal tumor

Answer 57

neuroblastoma

Question 57

second most common abdominal tumor

Answer 57

wilms

Question 57

increasing size of abdomen with PAINLESS abdominal mass. Hematuria or HTN may be present

Answer 57

wilms

Question 57

most common sign for a retinoblastoma

Answer 57

white pupillary reflex

Question 57

most common soft tissue sarcoma in childhood

Answer 57

rhabdomyosarcoma

Question 57

common place for rhabdomyo in younger pt

Answer 57

head, neck, GU

Question 57

common place for rhabdomyo in older pts

Answer 57

extremities, trunk, male GU

Question 57

who gets sarcomas most

Answer 57

adolescents- peaks with growth spurt