Pathoma: Normocytic Anemia with Extravascular Hemolysis

Question 1

Disorders with extravascular hemolysis often have some ________, too.

Answer 1

intravascular hemolysis

Question 2

Hereditary spherocytosis results from ___________.

Answer 2

a hereditary defect in spectrin, ankyrin, or band 3.1

Question 3

Histologically, hereditary spherocytosis leads to __________.

Answer 3

blebs of membrane breaking off, loss of central pallor, and anisocytosis

Question 4

The MCHC (mean corpuscular hemoglobin concentration) will be _______ in hereditary spherocytosis, uniquely.

Answer 4

increased, because the loss of membrane leads to decreased cell volume and resultant increase in hemoglobin concentration

Question 5

Features of the past medical history common to hereditary spherocytosis include ___________.

Answer 5

splenomegaly, bilirubin gallstones, and aplastic anemia second to parvovirus B19

Question 6

Hereditary spherocytosis can be tested by placing RBCs in ___________.

Answer 6

a hypotonic sucrose solution (“osmotic fragility test”)

Question 7

Anemia in hereditary spherocytosis will resolve in response to _________.

Answer 7

splenectomy

Question 8

Following splenectomy (or other splenic dysfunction), _________ will appear on blood smears.

Answer 8

Howell-Jolly bodies (pieces of DNA in RBCs that would normally be removed by the spleen)

Question 9

HgbS precipitates in response to _________.

Answer 9

deoxygenation, acidosis, and dehydration (precipitation is reversible)

Question 10

_____ increases the levels of HgbF.

Answer 10

Hydroxyurea

Question 11

Sickle cell results in _________ hemolysis.

Answer 11

extravascular, due to weakening of the red cell membrane

Question 12

Because sickle cell disease results in extravascular hemolysis, there will also be _________ in those with sickle cell, similar to hereditary spherocytosis.

Answer 12

bilirubin gallstones, jaundice, and splenomegaly

Question 13

__________ hemolysis results in low haptoglobin.

Answer 13

Intravascular

Question 14

__________ leads to target cells.

Answer 14

Dehydration

Question 15

Extramedullary hematopoiesis first occupies the _______, then the ________.

Answer 15

bone marrow; liver

Question 16

Other than splenomegaly, those with sickle cell present with _________ (radiographical sign) and __________ (another type of organomegaly).

Answer 16

crew-cut appearance (extramedullary hematopoiesis of the skull bones); hepatomegaly

Question 17

A common presenting sing of sickle cell disease in infants is ___________.

Answer 17

dactylitis (classically shown on exams with the hand of an African-American child)

Question 18

Those with sickle cell can lose this organ (due to pathology, not medical).

Answer 18

Spleen

Question 19

Without the spleen, patients are more susceptible to _______ organisms.

Answer 19

encapsulated (particularly Salmonella paratyphi osteomyelitis)

Question 20

In sickle cell, the most common cause of death in children is ________, while in adults it is __________.

Answer 20

infection with encapsulated organisms; acute chest syndrome

Question 21

Generall, those with sickle cell trait have less than _______ HbS.

Answer 21

50%

Question 22

Those with sickle trait are usually asymptomatic, except for __________.

Answer 22

in the renal medulla, in which the hypertonicity induces sickling and patients can sometimes develop a decreased ability to concentrate urine

Question 23

__________ can induce cells with any degree of HbS to sickle.

Answer 23

Metabisulfite (positive in those with the disease and the trait)

Question 24

Hemoglobin C has _________ instead of valine.

Answer 24

lysine (lie-CCCCCEEEne)