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Heme/Lymph > Hemolytic Anemias > Flashcards

Hemolytic Anemias Flashcards

1
Q

What results from hemolytic anemia?

A

RBC destruction leading to loss of RBC mass and release of cellular contents

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2
Q

Inherited/intrinsic hemolytic anemias

A

RBC membrane disorders
RBC enzyme deficiencies
Hemoglobinopathies

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3
Q

Acquired/extrainsic hemolytic anemias

A 
autoimmune (ab mediated)
mechanical trauma
infections
chemical
splenic sequestration
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4
Q

What is extravascular hemolysis?

A

spleen, liver, and bone marrow degradation of RBCs

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5
Q

What is intravascular hemolysis?

A

destruction of RBCs w/in the vessels

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6
Q

RBC membrane d/o

A

extra

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7
Q

RBC enzyme d/o

A

extra

intra

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8
Q

sickle cell anemia

A

extra

intra

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9
Q

thalassemia

A

extra

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10
Q

autoimmune hemolysis

A

extra (ab)

intra (complement)

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11
Q

PNH

A

intra

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12
Q

Mechanical

A

intra

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13
Q

malaria

A

intra

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14
Q

Lab findings w/hemolysis

A 
normocytic anemia
reticulocytosis
increased LDH, AST, K+
bilirubinemia
hemoglobinemia (free Hb in plasma)--> decreased haptoglobin
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15
Q

Causes of hemoglobinuria

A

intravascular

it’s Hb not scavenged by haptoglobin

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16
Q

what is hemosiderinuria?

A

intravascular

iron deposits in renal epithelium then shed in urine

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17
Q

Common symptoms of all hemolytic anemias

A

anemia

hyperbilirubinemia

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18
Q

Symptoms of acute decrease in Hb

A

restlessness, anxiety, DOE, syncope, confusion (by increasing age)

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19
Q

Clinical features of hyperbilirubinemia

A

jaundice
icterus
gallstones (pigmented)

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20
Q

Rare complications of hemolytic anemia

A

pigment induced kidney injury
folate deficiency due to increased utilization
increased risk of venous and arterial thrombosis (esp atypical sites)

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21
Q

What disease do you get chipmunk facies with?

A

beta thal major

22
Q

What disease do you see asplenia with?

A

sickle cell

23
Q

When do you see splenomegaly, hepatomegaly related to exrtra medullary hematopoiesis?

A

thal, PK deficiency, hereditary spherocytosis

24
Q

When do you see microangiopathic hemolytic anemia? (7 diseases)

A 
TTP
HUS
DIC
malignant hypertension
CREST syndrome
vasculitis
HELLP syndrome
25
Q

Causes of spherocytic hemolytic anemia (2)

A

hereditary pherocytosis
autoimmune hemolytic anemia (AIHA)
-warm
-cold

26
Q

What is the most common inherited hemolytic anemia in the US?

A

hereditary spherocytosis

27
Q

Mutations in hereditary spherocytosis?

A

ankyrin (most common)
spectrin
band3

28
Q

Complications of HS?

A

gallstones, jaundice
aplastic crisis (parvo b19)
splenectomy

29
Q

AIHAs qualifying feature

A

host antibodies reactive with autologous RBCs

shortened in vivo RBC survival

30
Q

Warm AIHA features

A

idiopathic
secondary-lymphoprolypherative disoders, AI disorders, non hematopoietic neoplasms, drugs
IgG

31
Q

Cold AIHA features

A

idopathic

secondary-post mono or mycoplasma pneumo, lymphoproliferative disorders

32
Q

anti-I

A

mycoplasma, cold AIHA

33
Q

anti-i

A

mono, cold AIHA

34
Q

When are cold aggluinins seen

A

cold weather

cold AIHA

35
Q

Blood smear findings for warm/cold AIHA

A

spherocytes

polychromasia

36
Q

What does warm AIHA extravascular hemolysis include?

A

Ab mediated or complement mediate hemolysis

spherocyte production

37
Q

How do cold agglutinins occur in cold AIHA?

A

IgM binds in cold, then mediates agglutination, blocks superfiscial distal vessels
IgM can also mediate complement mediated RBC injury, leads to extravascular hemolysis

38
Q

Symptoms of Warm AIHA?

A

anemia symptoms

organomegaly

39
Q

Symptoms of cold AIHA?

A 
follows cold exposure
symptoms of anemia
acrocyanosis
raynaud
post infection 2-3 weeks
40
Q

What does a positive DAT/Coombs test indicate?

A

an autoimmune hemolytic anemia or transfusion reaction

41
Q

What are the 3 congenital RBC enzyme disorders?

A

G6PD deficiency
Pyruvate Kinase deficiency
other

42
Q

What does the mutation in GDPDH deficiency cause?

A

defect in the pathway resonsible for reducing oxidants
decreased glutathione production
buildup of G6P
enzyme stability decreases with age of the RBC

43
Q

Examples of oxidant stressors

A

infection
fava beans
drugs-dapsone, sulfonamides, primaquine, nitrofurantoin, quinolones

44
Q

G6PDH deficiency inheritance?

A

X linked
common in malarial endemic areas
G6PD A- predominant abnormal varient, 11% all AAs

45
Q

G6PD A- ceaveat

A

only old RBCs hemolyze

reticulocytes and new RBCs do not

46
Q

G6PDH screen findings?

A

Heinz bodies
NADPH fluorescence (low to intermediate)
Quantitative assay to confirm

47
Q

Examples of mechanical trauma to RBCs

A 
heat
defective cardiac valves
TTP
Malignant HTN
Antiphospholipid antibody syndrome
DIC
disseminated cancer
48
Q

What is Paroxysmal nocturnal hemoglobinuria?

A

rare
acquried mutation in PIGA gene (X) resulting in decreased GPI linked proteins
lack of GPI linked anti-complement proteins makes cells susceptible to complement lysis
stem cell disorder, RBCs most sensitive to lysis

49
Q 
What is it?
chronic low level hemolysis 
acute episodic hemolysis events
hemoblobinuria
venous thromboses
A

PNH

50
Q

How to dx PNH?

A

flow cytometry for GPI linked proteins-examined on RBCs and WBCs
CD55 (DAF)
CD59

Heme/Lymph (18 decks)

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