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Block 6 > Hemolytic anemia 2 > Flashcards

Hemolytic anemia 2 Flashcards

1
Q

thalassemia

A

genetic decreased synthesis of alpha or beta global chains

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2
Q

alpha genes

A

chromosome 16

4 genes

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3
Q

beta genes

A

chromosome 11

2 genes

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4
Q

secondary hemochromatosis

A

iron accumulation

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5
Q

immunohemolytic anemia

A

RBC coated with antibodies or complement and removed by spleen

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6
Q

types of immunohemolytic anemia

A

warm antibody type
cold agglutinin type
cold hemolysis type

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7
Q

warm antibody type immunohemolytic anemia

A

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

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8
Q

cold agglutinin type immunohemolytic anemia

A

IgM

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9
Q

acute cold agglutinin type

A 
mycoplasma
mononucleosis (EBV)
CMV
influenza
HIV
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10
Q

chronic cold agglutinin type

A

idiopathic

lymphoma

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11
Q

cold hemolysis type immunohemolytic anemia

A

IgG

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12
Q

beta thalassemia

A

decreased synthesis of beta global chain

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13
Q

mutations associated with beta thalassemia

A

beta0- no chains due to chain termination mutations

beta+- reduced number of chains due to splicing or promoter region mutations

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14
Q

Major thalassemia

A

Cooley’s anemia

2 thalassemia alleles

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15
Q

pathogenesis of major beta thalassemia

A

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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16
Q

diagnostic findings for beta thallasemia

A 
increased RBC#
decreased MCV, MCH, MCHC
normal RDW
hypo chromic
microcytic
target cells
increased HbA2
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17
Q

complications of beta thalassemia major

A

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

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18
Q

treatment of beta thalassemia major

A

marrow transplant

19
Q

minor thalassemia

A

heterozygous thalassemia alleles

20
Q

alpha thalassemia

A

alpha global gene deletions

severity depends on number of genes affected

21
Q

1 mutation alpha thalaseemia

A

silent carrier- normal

22
Q

2 mutation alpha thalassemia

A

trait
microcytosis
hypochromia
mild anemia

23
Q

3 mutation alpha thalassemia

A

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

24
Q

4 mutation alpha thalassemia

A 
Hb Bart Hydrops Fettles syndrome
excess gamma with high O2 affinity
severe anemia
ascities/edema
hepatosplenomegaly
skeletal and CV malformations
die in utero
25
Q

paroxysmal nocturnal hemoglobinuria

A

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

26
Q

consequences of PNH

A

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

27
Q

diagnostic test for PNH

A

acidified serum test
sucrose hemolysis test
flow cytometry

28
Q

treatment for PNH

A

marrow transplant
growth factors
anti-complement antibody to reduce hemolysis

29
Q

acidified serum test

A

for PNH

acidified serum activates the alternative complement pathway which causes lysis

30
Q

sucrose hemolysis test

A

for PNH

sucrose promotes complement binding which leads to lysis

31
Q

flow cytometry

A 
for PNH
looks at cell types that could be missing GPI
granulocytes
monocytes
lymphocytes
RBC
missing CD55 and CD59
32
Q

microangiopathic hemolytic anemia (MAHA)

A

damage to red cells via contact with dense fibrin strands

33
Q

causes of MAHA

A 
DIC
thrombotic thrombocytopenia purport
hemolytic uremia syndrome
SLE
malignant HTN
34
Q

diagnostic features of MAHA

A

schistocytes

35
Q

treatment for warm antibody hemolytic anemia

A

corticosteroids

splectomy, rituximab

36
Q

drug related hemolytic anemia- happen/drug adsorption

A

drug binds to RBC and IgG formed against it

37
Q

drug related hemolytic anemia- immune complex

A

IgM forms after exposure to drug

further exposure leads to drug and antibody adsorbing to RBC

38
Q

drug related hemolytic anemia- autoantibody formation

A

IgG forms to drug, possible Rh specificity

39
Q

Direct Coombs test

A

distinguishes immune from non immune hemolytic anemias

antibody on cells agglutinate when exposed to antihuman antibody

40
Q

diagnostic markers of cold agglutinin syndrome

A

red cell agglutinates

rouleaux

41
Q

paroxysmal cold hemoglobinuria

A

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

42
Q

PCH associated with

A

ssyphillis

viral infection in kids

43
Q

diagnostic testing of PCH

A

Donath-Landsteiner test

44
Q

Donati-Landsteiner test

A

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

Block 6 (24 decks)

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