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Heme/Lymph > Disorders of Hemoglobin > Flashcards

Disorders of Hemoglobin Flashcards

1
Q

2 main categories of hemoglobin disorders?

A

structure variants

thalassemias

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2
Q

Consequences of structural abnormalities?

A 
sickling
instability
altered O2 affinity
increased susceptibility to oxidation to methemoglobin
underproduction
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3
Q

How to diagnose globin problem in lab?

A

Hb electrophoresis

HPLC (high performance liquid chromatography)

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4
Q

What to patients with sickle cell trait gain?

A

protection against malaria

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5
Q

What is the extent of HbS polymerization dependent on?

A

time & concentration

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6
Q

List some clinical settings that predispose to sickling

A 
hypoxia
acidosis
dehydration
cold temp
infections
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7
Q

What is the lifespan of a sickled cell?

A

20 days

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8
Q

Major effects of RBC sickling

A

chronic hemolysis and microvascular occlusion with resultant tissue hypoxia and infarction

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9
Q

When do the effects of sickle cell first show up after birth?

A

10-12 weeks of age

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10
Q

Main clinical manifestations (14)

A 
severe anemia
acute pain crisis
auto-splenectomy
acute chest syndrome
strokes 
aplastic crisis
splenic sequestration crisis
megaloblastic anemia
growth retardation
bony abnormalities
renal dysfunction
leg ulcers
infections
cholelithiasis
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11
Q

What is most common cause of death in sickle cell?

A

acute chest syndrome

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12
Q

What is the main cause for aplastic crisis in sickle cell?

A

parvovirus B19 infection

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13
Q

What happens during a splenic sequestration crisis?

A

acute pooling of blood in spleen
drop in Hb
potential for hypovolemic shock

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14
Q

Why does megaloblastic anemia cocur in Sickle Cell?

A

increased folate consumption because of chronic erythroid hyperproliferation

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15
Q

Lab findings in sickle cell

A 
sickeld cells, target cells, polychromasia
increased reticulocytes
chronic anemia
increased bilirubin
post splenectomy changes in adults
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16
Q

What can happen to the kidneys in pts w/sickle cell trait?

A

sub clinical damage

17
Q

What is Hemoglobin SC disease?

A

sickling disorder
HbC form glu to lys substitution
milder than SS usually

18
Q

What is HbS/Beta thalasemia?

A

hetero HbS with trans beta thalassemia allele
asymptomatic to SS like
HbS>HbA

19
Q

What is a thalassemia?

A

inherited disorder characterized by decreased production of structurally normal globin chains

20
Q

Distribution of beta thal?

A

mediterranean, middle east, some parts of india and pakistan, SE asia

21
Q

distribution of alpha thal?

A

africa, mediterranean, middle east, SE asia

22
Q

Features of thalassemai?

A

microcytic, hypochromic anemias of varying severity

23
Q

Beta-Thal major features

A

absense in beta chain production on both beta alleles
severe anemia
bizarre red cell morphology
transfusion dependence
hybrid of hemolytic anemia and ineffective erythropoiesis

24
Q

Problems in inadequately transfused beta thal major?

A 
stunted growth
mongoloid facies/bony abnormalities/frontal bossing
wasting
hyperuricemia
HSM
infections
folate defic
death in childhood
25
Q

Beta thal minor features

A

heterozygous
asymptomatic
common in mediterranean and asian populations
mild or no anemia
scattered target cells and basophilic stippling
elevated HbA2

26
Q

Beta thal intermedia

A

intermediate severity

27
Q

Alpha thalassemia features/types

A 
gene deletion
silent carrier (1 del) 
trait (2 del)mild
HbH disease (3 del) mild-mod chronic hemolytic anemia HbH is beta tetramers
Hydrops fetalis (4del) stillborn or die first few hours of life
28
Q

Effects of HU (hydroxiuria) dose escalation in sickle cell

A

decreased sickling

29
Q

Does cis or trans mutation in alpha thal matter for treatment?

A

no but it does for genetic counseling

30
Q

Splenic sequestration TX short and long term

A

short term: fluids, O2, RBCs

long term: splenectomy

31
Q

What cells does parvovirus B19 infect?

A

erythroblast

Heme/Lymph (18 decks)

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