Path- RBC Path Hertz

Question 1

Etiology of chronic blood loss

Answer 1

iron loss 
GI causes (neoplasm) 
gynecologic causes (menstruation)

Question 2

hereditary spherocytosis: etiology

Answer 2

autosomal mutation of spectrin, band 3 or 4.2, and ankyrin

Question 3

hereditary spherocytosis: morphology

Answer 3

spherocytes
Howell-Jolly body (small dark nuclear remnants)
anisocytosis (unequal-sized RBC’s)

Question 4

hereditary spherocytosis: presentation

Answer 4

anemia 
splenomegaly 
cholelithiasis 
jaundice 
elevated MCHC

Question 5

hereditary spherocytosis: complications

Answer 5

aplastic crises (parvovirus B19)
hemolytic crises (infectious mono)
gallstones (pigment stones

Question 6

hereditary spherocytosis: diagnosis

Answer 6

osmotic fragility test

Question 7

hereditary spherocytosis: treatment

Answer 7

splenectomy

Question 8

Conditions protective against malaria

Answer 8

G6PD deficiency
Thalassemia
Sickle cell disease

Question 9

G6PD deficiency: etiology

Answer 9

X-linked gene mutation in G6PD. causes GSH problems leading to oxidative injury and hemolytic anemia

Question 10

G6PD deficiency: pathogenesis

Answer 10

asymptomatic unless exposed to one of the following:
drugs (antimalarials, sulfonamide, large dose ASA, Vit K derivates)
infections
fava beans

Question 11

G6PD deficiency: intravascular or extravascular hemolysis?

Answer 11

both!

Question 12

G6PD deficiency: morphology

Answer 12

heinz bodies (RBC intracellular inclusions of denatured oxidized, precipitated HB)
bite cells

Question 13

G6PD deficiency: presentation

Answer 13

anemia
hemoglobinemia
hemoglobinuria 2-3 days post exposure

Question 14

G6PD deficiency: diagnosis

Answer 14

colorimetric testing
molecular analysis
repeat studies ~1 month later to allow time for regeneration

Question 15

G6PD deficiency: complications

Answer 15

G6PD Mediterranean variant = more severe hemolysis

Question 16

Sickle cell disease: epidemiology

Answer 16

most common familial hemolytic anemia in the world

most common hemoglobinopathy

Question 17

Sickle cell disease: etiology

Answer 17

autosomal recessive beta-globin gene point mutation creating HbS. deoxygenation results in formation of long polymers that aggregate. it can be reversible with oxygen, but chronic damage can lead to irreversibly sickled cells

Question 18

Sickle cell disease: major pathologic manifestations

Answer 18

chronic hemolysis
microvascular occlusions
tissue damage

Question 19

Sickle cell disease: morphology

Answer 19

sickle cells=]
target cells
spherocytes
reticulocytes

Question 20

Sickle cell disease: presentation

Answer 20

splenomegaly in children 
autosplenectomy in adults 
vascular congestion 
thrombosis
infarction of bones, liver, kidneys, retina, brain, lung, skin 
hemosiderosis 
gallstones 
prominent cheek bones 
hyperbilirubinemia 
impaired growth in children 
hypostheuria (can't concentrate urine)

Question 21

Sickle cell disease: diagnosis

Answer 21

Sickledex
Hb electrophoresis
X ray: crewcut
extramedullary hemaopoiesis

Question 22

Sickle cell disease: complications

Answer 22

chronic hemolytic anemia 
microvascular obstruction --> vaso-occlusive pain crises
acute chest syndrome 
aplastic crises (parvo B19)
infection-prone 
hand-foot syndrome (dactylics) 
splenic sequestration crises

Question 23

Sickle cell disease: treatment

Answer 23

PCN prophylaxis against pneumococcus and haemophilu

hydroxyurea (increases HbF and has an anti-inflammatory effect d/t WBC inhibition)

Question 24

Sickle cell disease: factors affecting rate/degree of sickling

Answer 24

1. [HbS]
2. MCHC
3. Intracellular pH
4. RBC transit time