Hemolytic Anemias

Question 1

What results from hemolytic anemia?

Answer 1

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

Question 2

Inherited/intrinsic hemolytic anemias

Answer 2

RBC membrane disorders
RBC enzyme deficiencies
Hemoglobinopathies

Question 3

Acquired/extrainsic hemolytic anemias

Answer 3

autoimmune (ab mediated)
mechanical trauma
infections
chemical
splenic sequestration

Question 4

What is extravascular hemolysis?

Answer 4

spleen, liver, and bone marrow degradation of RBCs

Question 5

What is intravascular hemolysis?

Answer 5

destruction of RBCs w/in the vessels

Question 6

RBC membrane d/o

Answer 6

extra

Question 7

RBC enzyme d/o

Answer 7

extra

intra

Question 8

sickle cell anemia

Answer 8

extra

intra

Question 9

thalassemia

Answer 9

extra

Question 10

autoimmune hemolysis

Answer 10

extra (ab)

intra (complement)

Question 11

PNH

Answer 11

intra

Question 12

Mechanical

Answer 12

intra

Question 13

malaria

Answer 13

intra

Question 14

Lab findings w/hemolysis

Answer 14

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

Question 15

Causes of hemoglobinuria

Answer 15

intravascular

it’s Hb not scavenged by haptoglobin

Question 16

what is hemosiderinuria?

Answer 16

intravascular

iron deposits in renal epithelium then shed in urine

Question 17

Common symptoms of all hemolytic anemias

Answer 17

anemia

hyperbilirubinemia

Question 18

Symptoms of acute decrease in Hb

Answer 18

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

Question 19

Clinical features of hyperbilirubinemia

Answer 19

jaundice
icterus
gallstones (pigmented)

Question 20

Rare complications of hemolytic anemia

Answer 20

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

Question 21

What disease do you get chipmunk facies with?

Answer 21

beta thal major

Question 22

What disease do you see asplenia with?

Answer 22

sickle cell

Question 23

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

Answer 23

thal, PK deficiency, hereditary spherocytosis

Question 24

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

Answer 24

TTP
HUS
DIC
malignant hypertension
CREST syndrome
vasculitis
HELLP syndrome

Question 25

Causes of spherocytic hemolytic anemia (2)

Answer 25

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

Question 26

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

Answer 26

hereditary spherocytosis

Question 27

Mutations in hereditary spherocytosis?

Answer 27

ankyrin (most common) spectrin band3

Question 28

Complications of HS?

Answer 28

gallstones, jaundice aplastic crisis (parvo b19) splenectomy

Question 29

AIHAs qualifying feature

Answer 29

host antibodies reactive with autologous RBCs | shortened in vivo RBC survival

Question 30

Warm AIHA features

Answer 30

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

Question 31

Cold AIHA features

Answer 31

idopathic | secondary-post mono or mycoplasma pneumo, lymphoproliferative disorders

Question 32

anti-I

Answer 32

mycoplasma, cold AIHA

Question 33

anti-i

Answer 33

mono, cold AIHA

Question 34

When are cold aggluinins seen

Answer 34

cold weather | cold AIHA

Question 35

Blood smear findings for warm/cold AIHA

Answer 35

spherocytes | polychromasia

Question 36

What does warm AIHA extravascular hemolysis include?

Answer 36

Ab mediated or complement mediate hemolysis | spherocyte production

Question 37

How do cold agglutinins occur in cold AIHA?

Answer 37

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

Question 38

Symptoms of Warm AIHA?

Answer 38

anemia symptoms | organomegaly

Question 39

Symptoms of cold AIHA?

Answer 39

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

Question 40

What does a positive DAT/Coombs test indicate?

Answer 40

an autoimmune hemolytic anemia or transfusion reaction

Question 41

What are the 3 congenital RBC enzyme disorders?

Answer 41

G6PD deficiency Pyruvate Kinase deficiency other

Question 42

What does the mutation in GDPDH deficiency cause?

Answer 42

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

Question 43

Examples of oxidant stressors

Answer 43

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

Question 44

G6PDH deficiency inheritance?

Answer 44

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

Question 45

G6PD A- ceaveat

Answer 45

only old RBCs hemolyze | reticulocytes and new RBCs do not

Question 46

G6PDH screen findings?

Answer 46

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

Question 47

Examples of mechanical trauma to RBCs

Answer 47

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

Question 48

What is Paroxysmal nocturnal hemoglobinuria?

Answer 48

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

Question 49

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

Answer 49

PNH

Question 50

How to dx PNH?

Answer 50

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