Hematology/Oncology Flashcards

1
Q

three reasons for anemia

A

reduced production
increased destruction
loss of blood

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2
Q

When would you expect a low retic count?

A

Iron def anemia
ALL
Diamond Blackfan anemia

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3
Q

when would you expect a high retic count?

A

Hemolysis

Bloos loss

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4
Q

Intrinsic hemolysis

A

hereditary spherocytosis
G6PD deficiency
hemoglobinopathies

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5
Q

Extrinsic hemolysis

A

AIHA
DIC
IV hemolysis

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6
Q

most common childhood hematologic problem

A

anemia

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7
Q

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

A

hemolytic

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8
Q

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

A

acute anemia

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9
Q

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

A

chronic anemia

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10
Q

peripheral blood smear shows us

A

size, shape, inclusions

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11
Q

iron studies include

A

serum Fe, ferritin, TIBC

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12
Q

hypochromic, microcytic RBCs

A

Fe deficiency

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13
Q

schistocytes associated with

A

HUS

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14
Q

Blicter cells associated with

A

G6PD

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15
Q

Sickle cells associated with

A

Hgb SS

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16
Q

spherocytes associated with

A

autoimmune hemolytic

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17
Q

most common anemia in childhood

A

Fe deficient anemia

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18
Q

treat Fe deficiency anemia

A

oral Fe 6mg/kg/day divided TID

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19
Q

macrocytic (megaloblastic) anemia

A

Folate

B12 deficiency

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20
Q

B12 absorbed where

A

terminal ileum (watch with crohns)

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21
Q

Folate absorbed where

A

jejunum (watch with celiac)

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22
Q

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

A

Fanconi

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23
Q

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

A

Diamond Black-Fan

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24
Q

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

A

acquired aplastic anemia

bone marrow not making enough stuff

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25
Q

test for isoimmunization

A

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

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26
Q

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

A

hereditary spherocytosis

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27
Q

treat hereditary spherocytosis

A

take out spleen

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28
Q

most frequent inherited enzyme defect

A

G6PD deficiency

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29
Q

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

A

G6PD

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30
Q

2nd most common inherited enzyme defect

A

pyruvate kinase deficiency

31
Q

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

A

thalassemia

32
Q

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

A

sickle cell disease

33
Q

disorders of coagulation

A

hemophilias
vitamin k deficiency
DIC

34
Q

most common type of thrombocytopenia

A

increased consumption

Idiopathic thrombocytopenic purpura

35
Q

types of increased consumption thrombocytopenia

A

ITP
DIC
Kasabach-Merritt syndrome

36
Q

most common bleeding d/o of childhood

A

ITP

37
Q

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

A

ITP

38
Q

treat ITP

A

IVIG best response

steroids good response

39
Q

hemophilia A

A

factor VIII

40
Q

hemophilia B

A

factor IX

Christmas disease

41
Q

treat factor VIII deficiency

A

desmopressin (or order factor VIII)

42
Q

treat factor IX deficiency

A

give factor IX

43
Q

Most common inherited bleeding disorder among Caucasians – autosomal dominant

A

von willebrand

44
Q

treat VWD

A

desmopressin

45
Q

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

A

TRUE

46
Q

Diseases in which you have a defect in glucuronyl transferase

A

gilbert’s

crigler naijar

47
Q

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

A

Gilbert’s

48
Q

treat Gilbert’s

A

No treatment needed except in neonate with excessive hyperbilirubinemia

49
Q

type 1 crigler-naijar

A

gene mutation- no GT activity- letha

50
Q

type 2 crigler naijar

A

diminished GT activity, treat with PB

51
Q

two main cancers seen in peds

A

leukemia

brain tumors

52
Q

Most common cause of death by disease

A

cancer

53
Q

most common leukemia

A

ALL

54
Q

least common leukemia

A

CML

55
Q

associated with philadelphia chromosome

A

chronic myelogenous leukemia

55
Q

associated with philadelphia chromosome

A

chronic myelogenous leukemia

56
Q

most common lymphoma

A

Hodgkin

56
Q

most common lymphoma

A

Hodgkin

57
Q

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

A

hodgkin

57
Q

biopsy lymph node in hodgkin to look for what

A

Reed Sternberg cells (malignant B cells)

57
Q

common nodes hodgkin

A

cervical or supraclavicular

57
Q

classic triad brain tumor

A

morning HA, vomiting, papilledema

57
Q

most common tumor of childhood

A

astroytoma

57
Q

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

A

neuroblastoma

57
Q

most common presentation for a neuroblastoma

A

abdominal pain or mass

57
Q

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

A

neuroblastoma

57
Q

second most common abdominal tumor

A

wilms

57
Q

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

A

wilms

57
Q

most common sign for a retinoblastoma

A

white pupillary reflex

57
Q

most common soft tissue sarcoma in childhood

A

rhabdomyosarcoma

57
Q

common place for rhabdomyo in younger pt

A

head, neck, GU

57
Q

common place for rhabdomyo in older pts

A

extremities, trunk, male GU

57
Q

who gets sarcomas most

A

adolescents- peaks with growth spurt