096 Hemolytic Anemias Flashcards

1
Q

Main sign of hemolysis (H20 C96 P709)

A

Jaundice

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2
Q

Telltale sign of intravascular hemolysis (H20 C96 P709)

A

Hemoglobinuria

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3
Q

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

A

10x

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4
Q

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

A

MCV

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5
Q

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

A

Erythroid hyperplasia

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6
Q

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

A

Increased red cell turnover

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7
Q

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

A

Red cell survival study

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8
Q

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

A

Parvovirus B19

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9
Q

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

A

glycophorins, band 3

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10
Q

Main red cell cytoskeletal protein (H20 C96 P711)

A

Spectrin

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11
Q

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

A

ANK1

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12
Q

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)

A

MCHC >34

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13
Q

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

A

4-6 years

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14
Q

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

A

Southeast Asia ovalocytosis

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15
Q

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

A

Triose phosphate isomerase deficiency

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16
Q

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

A

Southeast Asia

17
Q

G6PD variant that is distributed worldwide (H20 C96 P714)

A

G6PD Union

18
Q

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

A

Acute renal failure

19
Q

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

A

Neonatal jaundice, favism

20
Q

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

A

> 3 g/day

21
Q

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)

A

Infantile poikilocytosis

22
Q

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

A

Shiga toxin-producing E. coli O157:H7

23
Q

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

A

Clostridium perfringens

24
Q

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

A

Evans syndrome

25
Q

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

A

Common variable immunodeficiency

26
Q

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

A

Rituximab

27
Q

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

A

Paroxysmal cold hemoglobinuria (PCH)

28
Q

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

A

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

29
Q

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

A

Methyldopa

30
Q

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

A

Familial/Atypical HUS

31
Q

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

A

Paroxysmal nocturnal hemoglobinuria

32
Q

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

A

Venous thrombosis (followed by infection and hemorrhage)

33
Q

PNH may terminate into this malignancy (H20 C96 P721)

A

Acute myeloid leukemia

34
Q

Gold standard diagnostic test for PNH (H20 C96 P721)

A

Flow cytometry

35
Q

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

A

PIGA

36
Q

Eculizumab binds to this complement component (H20 C96 P721)

A

C5

37
Q

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

A

14 days

38
Q

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

A

At least 3 mg/day