Hemolytic Anemias Flashcards Preview

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Flashcards in Hemolytic Anemias Deck (50):
1

What results from hemolytic anemia?

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

2

Inherited/intrinsic hemolytic anemias

RBC membrane disorders
RBC enzyme deficiencies
Hemoglobinopathies

3

Acquired/extrainsic hemolytic anemias

autoimmune (ab mediated)
mechanical trauma
infections
chemical
splenic sequestration

4

What is extravascular hemolysis?

spleen, liver, and bone marrow degradation of RBCs

5

What is intravascular hemolysis?

destruction of RBCs w/in the vessels

6

RBC membrane d/o

extra

7

RBC enzyme d/o

extra
intra

8

sickle cell anemia

extra
intra

9

thalassemia

extra

10

autoimmune hemolysis

extra (ab)
intra (complement)

11

PNH

intra

12

Mechanical

intra

13

malaria

intra

14

Lab findings w/hemolysis

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

15

Causes of hemoglobinuria

intravascular
it's Hb not scavenged by haptoglobin

16

what is hemosiderinuria?

intravascular
iron deposits in renal epithelium then shed in urine

17

Common symptoms of all hemolytic anemias

anemia
hyperbilirubinemia

18

Symptoms of acute decrease in Hb

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

19

Clinical features of hyperbilirubinemia

jaundice
icterus
gallstones (pigmented)

20

Rare complications of hemolytic anemia

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

21

What disease do you get chipmunk facies with?

beta thal major

22

What disease do you see asplenia with?

sickle cell

23

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

thal, PK deficiency, hereditary spherocytosis

24

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

TTP
HUS
DIC
malignant hypertension
CREST syndrome
vasculitis
HELLP syndrome

25

Causes of spherocytic hemolytic anemia (2)

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

26

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

hereditary spherocytosis

27

Mutations in hereditary spherocytosis?

ankyrin (most common)
spectrin
band3

28

Complications of HS?

gallstones, jaundice
aplastic crisis (parvo b19)
splenectomy

29

AIHAs qualifying feature

host antibodies reactive with autologous RBCs
shortened in vivo RBC survival

30

Warm AIHA features

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

31

Cold AIHA features

idopathic
secondary-post mono or mycoplasma pneumo, lymphoproliferative disorders

32

anti-I

mycoplasma, cold AIHA

33

anti-i

mono, cold AIHA

34

When are cold aggluinins seen

cold weather
cold AIHA

35

Blood smear findings for warm/cold AIHA

spherocytes
polychromasia

36

What does warm AIHA extravascular hemolysis include?

Ab mediated or complement mediate hemolysis
spherocyte production

37

How do cold agglutinins occur in cold AIHA?

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

38

Symptoms of Warm AIHA?

anemia symptoms
organomegaly

39

Symptoms of cold AIHA?

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

40

What does a positive DAT/Coombs test indicate?

an autoimmune hemolytic anemia or transfusion reaction

41

What are the 3 congenital RBC enzyme disorders?

G6PD deficiency
Pyruvate Kinase deficiency
other

42

What does the mutation in GDPDH deficiency cause?

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

43

Examples of oxidant stressors

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

44

G6PDH deficiency inheritance?

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

45

G6PD A- ceaveat

only old RBCs hemolyze
reticulocytes and new RBCs do not

46

G6PDH screen findings?

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

47

Examples of mechanical trauma to RBCs

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

48

What is Paroxysmal nocturnal hemoglobinuria?

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

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

PNH

50

How to dx PNH?

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