096 Hemolytic Anemias

Question 1

Main sign of hemolysis (H20 C96 P709)

Answer 1

Jaundice

Question 2

Telltale sign of intravascular hemolysis (H20 C96 P709)

Answer 2

Hemoglobinuria

Question 3

LDH is increased by up to how many times normal in intravascular hemolysis? (H20 C96 P709)

Answer 3

10x

Question 4

The increased number of reticulocytes is associated with increase in this RBC index

Answer 4

MCV

Question 5

If bone marrow aspiration is done in hemolytic anemia, it will show: (H20 C96 P709)

Answer 5

Erythroid hyperplasia

Question 6

The essential pathophysiologic process common to all hemolytic anemias (H20 C96 P710)

Answer 6

Increased red cell turnover

Question 7

Gold standard for proving that the lifespan of red cells is reduced

Answer 7

Red cell survival study

Question 8

Viral precipitant of aplastic crisis in hemolytic conditions (H20 C96 P710)

Answer 8

Parvovirus B19

Question 9

Most abundant red cell membrane proteins [2] (H20 C96 P710)

Answer 9

glycophorins, band 3

Question 10

Main red cell cytoskeletal protein (H20 C96 P711)

Answer 10

Spectrin

Question 11

Mutations of this gene may account for majority of hereditary spherocytosis (H20 C96 P711)

Answer 11

ANK1

Question 12

This finding on an ordinary blood count should raise the suspicion of hereditary spherocytosis because HS is almost the only condition in which this abnormality occurs (H20 C96 P712)

Answer 12

MCHC >34

Question 13

In severe cases of hereditary spherocytosis, it is recommended that splenectomy is done at what age? (H20 C96 P712)

Answer 13

4-6 years

Question 14

Hemolytic anemia characterized by mutation in SLC4A1 gene. This disorder is also protective against Plasmodium falciparum. (H20 C96 P713)

Answer 14

Southeast Asia ovalocytosis

Question 15

Glycolytic enzymopathy that can cause severe mental retardation (H20 C96 P714)

Answer 15

Triose phosphate isomerase deficiency

Question 16

G6PD Vianchan and G6PD Mahidol are widespread G6PD variants in this region of the world (H20 C96 P714)

Answer 16

Southeast Asia

Question 17

G6PD variant that is distributed worldwide (H20 C96 P714)

Answer 17

G6PD Union

Question 18

The most serious threat of acute hemolytic anemia in adults is the development of: (H20 C96 P716)

Answer 18

Acute renal failure

Question 19

Commonest clinical manifestations of G6PD deficiency [2] (H20 C96 P716)

Answer 19

Neonatal jaundice, favism

Question 20

Aspirin dose that is a possible risk for clinical hemolysis in G6PD deficiency (H20 C96 P716)

Answer 20

> 3 g/day

Question 21

This hemolytic anemia occuring in the first month of life may be associated with deficiency of glutathione peroxidase (GSHPX) due to a transient nutritional deficiency of selenium. (H20 C96 P717)

Answer 21

Infantile poikilocytosis

Question 22

Most frequent infectious cause of hemolytic anemias in areas not endemic with malaria (H20 C96 P718)

Answer 22

Shiga toxin-producing E. coli O157:H7

Question 23

Main mechanism of septicemia causing intravascular hemolysis is exotoxin produced by: (H20 C96 P718)

Answer 23

Clostridium perfringens

Question 24

Syndrome wherein AIHA is accompanied by autoimmune thrombocytopenia (H20 C96 P719)

Answer 24

Evans syndrome

Question 25

Evans syndrome may be a manifestation of this primary immunodeficiency (H20 C96 P719)

Answer 25

Common variable immunodeficiency

Question 26

This drug is increasingly being used together with prednisone as first-line treatment for warm AIHA (H20 C96 P719)

Answer 26

Rituximab

Question 27

Acquired hemolytic anemia characterized by the involvement of Donath-Lansteiner antibody (H20 C96 P719)

Answer 27

Paroxysmal cold hemoglobinuria (PCH)

Question 28

Hemolytic anemias characterized by basophilic stippling [2] (H20 C96)

Answer 28

Pyrimidine 5'-nucleotidae (P5N) deficiency, lead poisoning

Question 29

Best known example of drug-independent drug-induced immune hemolytic anemia (H20 C96 P719)

Answer 29

Methyldopa

Question 30

This group is unique in that hemolysis results from an inherited defect external to red cells (H20 C96 P717)

Answer 30

Familial/Atypical HUS

Question 31

In the absence of liver disease, a full-fledged Budd-Chiari syndrome ought to raise the suspicion of: (H20 C96 P720)

Answer 31

Paroxysmal nocturnal hemoglobinuria

Question 32

Most common cause of death in patients with PNH (H20 C96 P720)

Answer 32

Venous thrombosis (followed by infection and hemorrhage)

Question 33

PNH may terminate into this malignancy (H20 C96 P721)

Answer 33

Acute myeloid leukemia

Question 34

Gold standard diagnostic test for PNH (H20 C96 P721)

Answer 34

Flow cytometry

Question 35

A somatic mutation in this X-linked gene results in PNH (H20 C96 P721)

Answer 35

PIGA

Question 36

Eculizumab binds to this complement component (H20 C96 P721)

Answer 36

C5

Question 37

Eculizumab has to be given intravenously at what frequency because of its short half-life (H20 C96 P721)

Answer 37

14 days

Question 38

Minimum dose of folic acid supplements in PNH (H20 C96 P721)

Answer 38

At least 3 mg/day