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Hematology > Intrinsic Defects Leading to Increased Erythrocyte Destruction > Flashcards

Intrinsic Defects Leading to Increased Erythrocyte Destruction Flashcards

1
Q

Results when the rate of RBC destruction exceeds the increased rate of RBC production

A

Hemolytic anemia

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2
Q

Has a rapid onset and is isolated (sudden), episodic, or paroxysmal

A

Acute hemolysis

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3
Q

May not be evident if the bone marrow is able to compensate, but it may be punctuated over time with hemolytic crises that cause the anemia

A

Chronic hemolysis

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4
Q

Are passed to offspring by mutant genes from the parents

A

Inherited

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5
Q

Develop in individuals who were previously hematologically normal but acquire an agent of condition that lyses RBCs

A

Acquired

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6
Q

Destruction of the RBCs due to a defect within the RBCs themselves

A

Intrinsic hemolytic conditions

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7
Q

Arise from outside the RBC, typically substances in the plasma or conditions affecting the anatomy of the circulatory system

A

Extrinsic hemolytic conditions

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8
Q

Intrinsic vs. Extrinsic:

Most are inherited

A

Intrinsic hemolytic conditions

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9
Q

Intrinsic vs. Extrinsic:

Most are acquired

A

Extrinsic hemolytic conditions

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10
Q

Takes places most often within the bloodstream; occurs by fragmentation

A

Intravascular hemolysis

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11
Q

Can refer either to lysis within the macrophage and not in the bloodstream or to the fact that most of the macrophages are in tissues

A

Extravascular hemolysis

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12
Q

Is the result to the RBC membrane that causes a breach sufficient for the cell contents to spill directly into plasma

A

Fragmentation

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13
Q

Occurs when RBCs are engulfed by macrophage and lysed by their digestive enzymes

A

Macrophase-mediated

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14
Q

Mechanisms to salvage hemoglobin iron and prevent oxidation reactions during fragmentation hemolysis:

A

Haptoglobin
Hemopexin
Albumin

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15
Q

Haptoglobin is a protein in plasma that contains _____ receptor that bind to the heme for it to be recycled

A

CD163

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16
Q

Receptor of Hemopexin

A

CD91

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17
Q

Provide vertical structural integrity to the cell by anchoring the lipid bilayer to the underlying spectrin cytoskeleton

A

Ankyrin complex and protein 4.1 complex

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18
Q

“vertical” because it is perpendicular to the plane of the cytoskeleton

A

Ankyrin complex and protein 4.1 complex

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19
Q

Prevents loss of membrane and the resultant decrease in the surface are-to-volume ratio of the RBC

A

Ankyrin complex and protein 4.1 complex

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20
Q

Provide the horizontal mechanical stability

A

Spectrin-actin-protein 4.1 junctional complex

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21
Q

Prevents the membrane from fragmenting in response to mechanical stress

A

Spectrin-actin-protein 4.1 junctional complex

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22
Q

Caused by the defects in proteins that disrupt the vertical interactions between transmembrane proteins and the underlying protein cytoskeleton

A

Hereditary Spherocytosis

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23
Q

Results from gene mutation

A

Hereditary Spherocytosis

24
Q

The defects in vertical membrane protein interactions cause RBCs to lose unsupported lipid membrane over time because of local disconnections of the lipid bilayer and underlying cytoskeleton

A

Hereditary Spherocytosis

25
Q

Three key clinical manifestations of Hereditary Spherocytosis

A

Anemia, Jaundice, Splenomegaly

26
Q

Hallmark: Spherocytes on the peripheral blood film

A

Hereditary Spherocytosis

27
Q

Laboratory findings of Hereditary Spherocytosis

A

Increased MCHC
Decreased serum haptoglobin
Increased serum indirect bilirubin and lactate dehydrogenase

28
Q

Tests for Hereditary Spherocytosis

A

Osmotic fragility
Eosin-5’-maleimide (EMA) binding test

29
Q

demonstrated increased RBC fragility in blood specimens in which the RBCs have decreased surface area-to-volume ratios

A

Osmotic fragility

30
Q

Cells with decreased surface are-volume ratio have limited capacity to expand in hypotonic solutions, hence undergo lysis

A

Osmotic fragility

31
Q

Anticoagulant of Osmotic fragility

A

Heparin

32
Q

Not sensitive, nonspecific test for Hereditary Spherocytosis

A

Osmotic fragility

33
Q

Is a fluorescent dye that binds to transmembrane proteins band 3, Rh, RhAg, and CD47 in the RBC membrane

A

EMA

34
Q

Caused by defects in proteins that disrupts the horizontal or lateral interactions in the protein cytoskeleton

A

Hereditary Elliptocytosis

35
Q

Ankyrin is _____

A

Vertical

36
Q

Spectrin is ______

A

Horizontal or lateral

37
Q

Characteristic findings: elliptical or cigar shaped RBCs on the peripheral blood film

A

Hereditary Elliptocytosis

38
Q

Considered a sever form of Hereditary Elliptocytosis

A

Hereditary pyropoikilocytosis

39
Q

RBCs show thermal sensitivity

A

Hereditary pyropoikilocytosis

40
Q

Southeast asian ovalocytosis

A

Hereditary Ovalocytosis

41
Q

A condition caused by mutation in the gene for band 3 that results in increased rigidity of the membrane

A

Hereditary ovalocytosis

42
Q

Due to a defect in membrane cation permeability that causes RBCs to be overhydrated

A

Overhydrated Hereditary Stomatocytosis

43
Q

The RBC membrane is excessively permeable to sodium and potassium at 37C

A

Overhydrated Hereditary Stomatocytosis

44
Q

Also known as hereditary xerocytosis

A

Dehydrated Hereditary Stomatocytosis

45
Q

Due to a defect in membrane cation permeability that causes the RBCs to be dehydrated

A

Dehydrated Hereditary Stomatocytosis

46
Q

RBC membrane is excessively permeable to potassium

A

Dehydrated Hereditary Stomatocytosis

47
Q

Potassium leaks out of the cell

A

Dehydrated Hereditary Stomatocytosis

48
Q

Most common form of stomatocytosis

A

Dehydrated Hereditary Stomatocytosis

49
Q

Excessive potassium leaks out of the RBCs at room temp in vitro but not at body in vivo

A

Familial Pseudohyperkalemia

50
Q

RBCs have marked swelling and hemolysis when stored at 4C for 24-48 hrs

A

Cryohydrocytosis

51
Q

Rh null or decreased expression of Rh membrane protein

A

Rh Deficiency Syndrome

52
Q

Is a term used to describe a group of rare inherited disorders characterized by neurologic impairment and acanthocytes on the PBS

A

Neuroacanthocytosis

53
Q

Characterized by fat malabsorption, progressive ataxia, neuropathy, retinitis pigmentosa, and acanthocytosis

A

Abetalipoproteinmeia

54
Q

An X-linked disorder caused by mutations in the KX gene

A

McLeod Syndrome

55
Q

Characterized by chorea, hyperkinesia, cognitive impairments, and neuropsychiatric symptoms

A

Chorea acanthocytosis

56
Q
A

Hematology (28 decks)

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