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Pediatrics > Exam 3 - Hematology > Flashcards

Exam 3 - Hematology Flashcards

1
Q

how long do sickled RBC live

A

20 days or less

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2
Q

what are the 3 complications of SCD

A

vasoocculsive crisis
acute sequestration crisis
aplastic event

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3
Q

what occurs during SCD - vasoocclusive crisis

A

blood flow to tissue is obstructed

obstructed flow = hypoxemia + ischemia = pain in affected area

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4
Q

SCD - vasooclusive crisis poses a risk for ___

A

stroke

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5
Q

what occurs during SCD - acute sequestration crisis

A

obstructed blood flow to liver, lungs or spleen

organs become engorged with blood; leads to acute anemia
respiratory failure occurs if in lungs

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6
Q

what occurs during SCD - aplastic event

A

increase destruction OR decrease production of RBC

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7
Q

SCD - aplastic event: increase destruction is r/t what?

A

fever
infection

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8
Q

SCD - aplastic event: decrease production is r/t what?

A

viral infection (parvovirus B19 aka 5ths disease)

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9
Q

SCD treatment

A

manage pain (morphine preferred; NSAIDS)
promote hydration
minimize crisis
supportive therapy

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10
Q

how long does a crisis occur

A

days to weeks; average of 5-7 days

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11
Q

when do symptoms of SCD become present

A

4-6 months after birth

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12
Q

s/sx of SCD

A

chronic hemolytic anemia
pallor
jaundice
fatigue
cholelithiasis
delayed growth + puberty
avascular necrosis of hips, shoulders
renal dysfunction
retinopathy

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13
Q

what are some things that can trigger a SCD crisis

A

infection
dehydration
hypoxia
trauma
general stress

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14
Q

prenatally, when can SCD testing be performed; what test is used

A

8-10 weeks: CVS
15 weeks: aminocentesis

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15
Q

what will be elevated to confirmed SCD and why

A

elevated reticulocyte d/t short RBC life

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16
Q

when would a person NOT be a candidate for outpt SCD therapy

A

ill appearance
cardiac instability
< 1 y/o
pulmonary infiltrates
splenectomy
hx of pneumon. sepsis
Hbg < 5
lack of ability to adhere to outpt treatment
WBC < 5000 or > 30000
dehydration

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17
Q

what factor is needed for blood clotting

A

factor 8

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18
Q

Hgb A is factor __; Hgb B is factor __

A

A: 8
B: 9

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19
Q

hemophilia is ___ recessive

A

autosomal recessive

females are carriers, will pass to males

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20
Q

how is hemophilia treated

A

factor prophylaxis
RICE

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21
Q

hemarthrosis can occur with hemophilia. what is this

A

bleeding muscle + joint

reoccurrence is common

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22
Q

can you apply heat to affected joints

A

no, ONLY cold packs

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23
Q

most common inherited bleeding disorder

A

Von Willebrand Disease

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24
Q

what is von willebrand disease

A

underproduction of vonwillebrand protein

25
Q

s/sx of VWD

A

epitaxis
bleeding gums
prolonged bleeding
menorrhagia

26
Q

how to tx VWD

A

DDAVP (IV, SQ, intranasally)
birth control for menorrhagia

27
Q

what is immune thrombocytopenic purpura (ITP)

A

acquired thrombocytopenia

plt < 150000; often < 50000 at Dx

28
Q

ITP usually follows a ___ illness and is considered an ___ process

A

viral; autoimmune

29
Q

s/sx of ITP

A

sudden bruising
petechia
bleeding mucous membranes and gums

30
Q

what is the greatest risk with ITP/low plt count

A

intracranial hemorrhage

neuro assessment is important

31
Q

when should plt count return to normal with ITP

A

within 12 months

32
Q

when is a plt transfusion performed with ITP

A

only with active, uncontrolled bleeds

33
Q

peak age for ITP

A

4-6 y/o

34
Q

what seasons does ITP commonly occur

A

late Winter, Spring

35
Q

chronic ITP commonly occurs in which age group and gender

A

adolescent females

36
Q

acute vs. chronic ITP

A

A: recovery within 6 months
C: last > 6 months

37
Q

how long are PO steroids and IVIG administered for ITP

A

steroids: 2-4 weeks
IVIG: 2 days

38
Q

what is disseminated intravascular coagulation (DIC)

A

uncontrolled formation and deposition of thrombi

39
Q

s/sx of DIC

A

insidious onset
excessive bruising
petechia
oozing from puncture site + sites of mild trauma

40
Q

s/sx with progression of DIC

A

purpuric rash
hemoptysis
hypoxemia
oliguria with renal failure
progressive organ failure
intracranial hemorrhage

41
Q

DIC treatment

A

treat underlying condition
normalize PT
vitamin K
PRBC, FFP

42
Q

what occurs with aplastic anemia

A

bone marrow ceases production of normal cells resulting in panycytopenia

43
Q

s/sx of aplastic anemia

A

petechiae
ecchymosis
pallor
epitaxis
fatigue
tachycardia
anorexia
infection

44
Q

aplastic anemia treatment is based on ___

A

symptoms

abx
stem cell transplant: choice if severe

45
Q

with aplastic anemia, the bone marrow has what kind of apperance

A

hypocellular (yellow, fatty with lymphocytes)

46
Q

what should be avoided with low plt count

A

IM injections
ASA, ASA products
NSAIDS
rectal temps
high-risk contact sports

47
Q

what is thalassemia

A

inherited blood disorder with abnormal Hbg synthesis

autosomal recessive

48
Q

s/sx of thalassemia

A

pallor
growth retardation
delayed puberty
severe anemia
hepatosplenomegaly
bronzed/green-yellow skin tone

49
Q

how is thalassemia dx

A

CBC
reticulocyte count
Fe
total Fe binding
Hgb electrophoresis
Hgb A, F

50
Q

thalassemia treatment

A

erythrocyte/PRBC transfusion
chelation therapy
splenectomy

51
Q

long term PRBC transfusion poses a risk for ___

A

hemosiderosis (excessive Fe deposits)

52
Q

why is chelation therapy used with thalassemia

A

prevent organ damage from Fe overload

53
Q

which chelation meds are used for thalassemia

A

deferoxamine: SQ; IV cont. IV over 8-12 hours at HS

deferasirox: PO > 2 y/o

54
Q

a pt with thalassemia can not be ___ dependent in order to have a splenectomy

A

transfusion depended

55
Q

splenectomy poses an increase risk for ___, ___, and ___

A

strep
flu
meningitis

56
Q

what is the only available cure of thalassemia at this time

A

bone marrow transplant

57
Q

bone marrow transplant for thalassemia is reserved for ___ ___ with what kind of donor

A

young children; sibling

58
Q

facial appearance with thalassemia

A

frontal bossing (prominent, protruding forehead)
maxillary prominence
wide-set eyes
flat nose

Pediatrics (12 decks)

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