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Flashcards in Hemolytic anemia 2 Deck (44):
1

thalassemia

genetic decreased synthesis of alpha or beta global chains

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alpha genes

chromosome 16
4 genes

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beta genes

chromosome 11
2 genes

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secondary hemochromatosis

iron accumulation

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immunohemolytic anemia

RBC coated with antibodies or complement and removed by spleen

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types of immunohemolytic anemia

warm antibody type
cold agglutinin type
cold hemolysis type

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warm antibody type immunohemolytic anemia

IgG 37C
microspherocytes
antibody specific for Rh antigens
diagnosed via Direct Coombs test

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cold agglutinin type immunohemolytic anemia

IgM

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acute cold agglutinin type

mycoplasma
mononucleosis (EBV)
CMV
influenza
HIV

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chronic cold agglutinin type

idiopathic
lymphoma

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cold hemolysis type immunohemolytic anemia

IgG

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beta thalassemia

decreased synthesis of beta global chain

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mutations associated with beta thalassemia

beta0- no chains due to chain termination mutations
beta+- reduced number of chains due to splicing or promoter region mutations

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Major thalassemia

Cooley's anemia
2 thalassemia alleles

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pathogenesis of major beta thalassemia

reduced beta synthesis with excessive alpha production
insoluble alpha aggregates form causing ineffective erythropoiesis-- most cells die
abnormal cells that get out cause extravascular hemolysis which leads to anemia and tissue hypoxia
marrow expands to compensate which leads to skeletal deformities

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diagnostic findings for beta thallasemia

increased RBC#
decreased MCV, MCH, MCHC
normal RDW
hypo chromic
microcytic
target cells
increased HbA2

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complications of beta thalassemia major

skeletal abnormalities--new bone formation in the outer table --> perpendicular skull radiations

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treatment of beta thalassemia major

marrow transplant

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minor thalassemia

heterozygous thalassemia alleles

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alpha thalassemia

alpha global gene deletions
severity depends on number of genes affected

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1 mutation alpha thalaseemia

silent carrier- normal

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2 mutation alpha thalassemia

trait
microcytosis
hypochromia
mild anemia

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3 mutation alpha thalassemia

HbH disease
excess beta microcytic hypo chromic hemolytic anemia
mild jaundice
moderate hepatosplenomegaly

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4 mutation alpha thalassemia

Hb Bart Hydrops Fettles syndrome
excess gamma with high O2 affinity
severe anemia
ascities/edema
hepatosplenomegaly
skeletal and CV malformations
die in utero

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paroxysmal nocturnal hemoglobinuria

rare acquired stem cell disease with somatic mutations affecting RBC membrane
PIG-A defect for RBC anchor GPI

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consequences of PNH

sensitive to complement mediated lysis
proteins cannot attach to cell
periodic episodes of night time bloody urine

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diagnostic test for PNH

acidified serum test
sucrose hemolysis test
flow cytometry

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treatment for PNH

marrow transplant
growth factors
anti-complement antibody to reduce hemolysis

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acidified serum test

for PNH
acidified serum activates the alternative complement pathway which causes lysis

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sucrose hemolysis test

for PNH
sucrose promotes complement binding which leads to lysis

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flow cytometry

for PNH
looks at cell types that could be missing GPI
granulocytes
monocytes
lymphocytes
RBC
missing CD55 and CD59

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microangiopathic hemolytic anemia (MAHA)

damage to red cells via contact with dense fibrin strands

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causes of MAHA

DIC
thrombotic thrombocytopenia purport
hemolytic uremia syndrome
SLE
malignant HTN

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diagnostic features of MAHA

schistocytes

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treatment for warm antibody hemolytic anemia

corticosteroids
splectomy, rituximab

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drug related hemolytic anemia- happen/drug adsorption

drug binds to RBC and IgG formed against it

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drug related hemolytic anemia- immune complex

IgM forms after exposure to drug
further exposure leads to drug and antibody adsorbing to RBC

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drug related hemolytic anemia- autoantibody formation

IgG forms to drug, possible Rh specificity

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Direct Coombs test

distinguishes immune from non immune hemolytic anemias
antibody on cells agglutinate when exposed to antihuman antibody

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diagnostic markers of cold agglutinin syndrome

red cell agglutinates
rouleaux

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paroxysmal cold hemoglobinuria

intravascular hemolysis when exposed to cold due to IgG agasint P antigen
antibody and early complement bind at low temperatures and then terminal complement binds with warming which causes lysis

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PCH associated with

ssyphillis
viral infection in kids

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diagnostic testing of PCH

Donath-Landsteiner test

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Donati-Landsteiner test

for PCH
RBC and complement mixed at low temps--bind
warm --> intravascular hemolysis