Exam 3 - Hematology Flashcards

(58 cards)

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
s/sx of VWD
epitaxis bleeding gums prolonged bleeding menorrhagia
26
how to tx VWD
DDAVP (IV, SQ, intranasally) birth control for menorrhagia
27
what is immune thrombocytopenic purpura (ITP)
acquired thrombocytopenia plt < 150000; often < 50000 at Dx
28
ITP usually follows a ___ illness and is considered an ___ process
viral; autoimmune
29
s/sx of ITP
sudden bruising petechia bleeding mucous membranes and gums
30
what is the greatest risk with ITP/low plt count
intracranial hemorrhage neuro assessment is important
31
when should plt count return to normal with ITP
within 12 months
32
when is a plt transfusion performed with ITP
only with active, uncontrolled bleeds
33
peak age for ITP
4-6 y/o
34
what seasons does ITP commonly occur
late Winter, Spring
35
chronic ITP commonly occurs in which age group and gender
adolescent females
36
acute vs. chronic ITP
A: recovery within 6 months C: last > 6 months
37
how long are PO steroids and IVIG administered for ITP
steroids: 2-4 weeks IVIG: 2 days
38
what is disseminated intravascular coagulation (DIC)
uncontrolled formation and deposition of thrombi
39
s/sx of DIC
insidious onset excessive bruising petechia oozing from puncture site + sites of mild trauma
40
s/sx with progression of DIC
purpuric rash hemoptysis hypoxemia oliguria with renal failure progressive organ failure intracranial hemorrhage
41
DIC treatment
treat underlying condition normalize PT vitamin K PRBC, FFP
42
what occurs with aplastic anemia
bone marrow ceases production of normal cells resulting in panycytopenia
43
s/sx of aplastic anemia
petechiae ecchymosis pallor epitaxis fatigue tachycardia anorexia infection
44
aplastic anemia treatment is based on ___
symptoms abx stem cell transplant: choice if severe
45
with aplastic anemia, the bone marrow has what kind of apperance
hypocellular (yellow, fatty with lymphocytes)
46
what should be avoided with low plt count
IM injections ASA, ASA products NSAIDS rectal temps high-risk contact sports
47
what is thalassemia
inherited blood disorder with abnormal Hbg synthesis autosomal recessive
48
s/sx of thalassemia
pallor growth retardation delayed puberty severe anemia hepatosplenomegaly bronzed/green-yellow skin tone
49
how is thalassemia dx
CBC reticulocyte count Fe total Fe binding Hgb electrophoresis Hgb A, F
50
thalassemia treatment
erythrocyte/PRBC transfusion chelation therapy splenectomy
51
long term PRBC transfusion poses a risk for ___
hemosiderosis (excessive Fe deposits)
52
why is chelation therapy used with thalassemia
prevent organ damage from Fe overload
53
which chelation meds are used for thalassemia
deferoxamine: SQ; IV cont. IV over 8-12 hours at HS deferasirox: PO > 2 y/o
54
a pt with thalassemia can not be ___ dependent in order to have a splenectomy
transfusion depended
55
splenectomy poses an increase risk for ___, ___, and ___
strep flu meningitis
56
what is the only available cure of thalassemia at this time
bone marrow transplant
57
bone marrow transplant for thalassemia is reserved for ___ ___ with what kind of donor
young children; sibling
58
facial appearance with thalassemia
frontal bossing (prominent, protruding forehead) maxillary prominence wide-set eyes flat nose