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Hematology 2 PPT > Week 2 - Vascular Disorders > Flashcards

Week 2 - Vascular Disorders Flashcards

1
Q

Abnormal bleeding involving the loss of red blood cells from the microcirculation.

A

Purpura

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2
Q

Characterized by obvious hemorrhages into the skin and mucous membranes that appear as extensive areas of red or dark-purple discoloration.

A

Purpura

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3
Q

If small, purplish hemorrhagic spots on the skin or mucous membranes are evident, it is called.

A

Petechiae

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4
Q

If the patches of bleeding into the tissues are larger, this is referred to as

A

Ecchymosis

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5
Q

Hereditary Hemorrhagic Telangiectasia

A

Rendu-Osler-Weber Syndrome

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6
Q

Autosomal Dominant

A

Hereditary Hemorrhagic Telangiectasia
Rendu-Osler-Weber Syndrome

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7
Q

Characterized by thin-walled blood vessels.

A

Hereditary Hemorrhagic Telangiectasia

Rendu-Osler-Weber Syndrome

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8
Q

○ Discontinuous endothelium
○ Inadequate smooth muscle
○ Inadequate or missing elastin in the stroma

A

Hereditary Hemorrhagic Telangiectasia
Rendu-Osler-Weber Syndrome

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9
Q

Manifests by puberty and progresses throughout life.

A

Rendu-Osler-Weber Syndrome

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10
Q

Universal finding of Rendu-Osler-Weber Syndrome

A

Epistaxis

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11
Q

Mutations in the ENG gene.

A

Type 1 Hereditary Hemorrhagic Telangiectasia

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12
Q

Mutations in the ACVRL1 gene.

A

Hereditary Hemorrhagic Telangiectasia type 2

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13
Q

Unknown mutation of HHT

A

Hereditary Hemorrhagic Telangiectasia type 3

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14
Q

caused by mutations in the gene SMAD4.

A

Juvenile Polyposis/HHT

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15
Q

Dilated superficial blood vessels that create small,
focal red lesions.

A

Telangiectasia

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16
Q

The lesions blanch when pressure is applied.

A

Telangiectasia

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17
Q

● Most obvious on the face, lips, tongue, conjunctiva, nasal mucosa, fingers, toes, trunk, and under the tongue.
● Fragile and prone to rupture.

A

Telangiectasia

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18
Q

Diagnosis of HHT

A

Based on the consensus clinical criteria of Curacao.

Presence of 3 out of 4 criteria:
○ Spontaneous recurrent epistaxis
○ Cutaneous telangiectasia
○ Visceral involvement
○ Familial heredity

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19
Q

Other Disorders with Telangiectasia

A

Cherry-redhemangioma
Ataxia-Telangiectasia (Louis-Bar Syndrome)
Chronic Actinic Telangiectasia

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20
Q

Hemangioma-Thrombocytopenia Syndrome

A

Kasabach-Merritt Syndrome

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21
Q

Hereditary basis has not been established, but condition is present at birth

A

Hemangioma-Thrombocytopenia Syndrome
Kasabach-Merritt Syndrome

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22
Q

Giant hemangioma may occur

A

congenitally soon after birth or affect the skin, liver, and spleen.

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23
Q

Either visceral or subcutaneous, but rarely both.

A

Hemangioma-Thrombocytopenia Syndrome
Kasabach-Merritt Syndrome

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24
Q

External hemangiomas may become engorged with blood and resemble hematomas.

A

Hemangioma-Thrombocytopenia Syndrome
Kasabach-Merritt Syndrome

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25
Q

Giant Cavernous Hemangioma (Vascular Tumor)
Complications

A

Microangiopathic hemolytic anemia

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26
Q

Associated disease of Hemangioma Thrombocytopenia Syndrome

A

Giant Cavernous Hemangioma (Vascular Tumor)
Thrombocytopenia
Bleeding diathesis

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27
Q

Thrombocytopenia complications

A

Sequestration of platelets in hemangiomas

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28
Q

Bleeding Diathesis complications

A

Acute or chronic disseminated intravascular coagulopathy

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29
Q

Ehlers- Danlos Syndrome
Mode of inheritance:

A

Autosomal dominant
Autosomal recessive
X-linked trait

30
Q

Can be ascribed to defects in collagen production.

A

Ehler danlos syndrome

31
Q

Ehler Danlos Syndrome Manifestations

A

● Hyperextensible skin
● Hypermobile joints
● Joints laxity
● Fragile tissues
● Bleeding tendency
● Subcutaneous
hematoma formation

32
Q

EDS IV mode of inheritance

A

Autosomal dominant

33
Q

Mode of inheritance of EDS
Greatest risk for major bleeding.

A

Autosomal dominant

34
Q

Mode of inheritance of EDS
Structural defect in proa1(III) chain of
collagen encoded by COL3A1 gene

A

Autosomal dominant

35
Q

Pseudoxanthoma Elasticum (PXE) other name

A

Groenblag-Strandberg Syndrome

36
Q

Autosomal recessive

A

Pseudoxanthoma Elasticum (PXE)
Groenblag-Strandberg Syndrome

37
Q

Inherited connective tissue disorder that results in
calcification and mineralization of elastic fibers

A

Pseudoxanthoma Elasticum (PXE)
Groenblag-Strandberg Syndrome

38
Q

Pseudoxanthoma Elasticum (PXE)
Groenblag-Strandberg Syndrome mutations

A

Mutations in the ABC-C6 gene.

39
Q

Group of nonthrombocytopenic purpuras characterized by apparently allergic manifestations, including skin rash and edema

A

Allergic Purpura
Anaphylactoid Purpura

40
Q

Associated with certain foods and drugs, cold, insect bites, and vaccinations.

A

Allergic Purpura
Anaphylactoid Purpura

41
Q

Acute IgA-mediated disorder

A

Henoch-Schonlein Purpura

42
Q

Generalized vasculitis involving the skin, joints,
kidneys, GI tract, and, less commonly, the lungs.

A

Henoch-Schonlein Purpura

43
Q

Generalized vasculitis involving the skin, joints,
kidneys, GI tract, and, less commonly, the lungs.

A

Henoch-Schonlein Purpura

44
Q

Frequently confused with ITP.

A

Frequently confused with ITP.

45
Q

Primarily a disease of children. (3-7 years of age)

A

Henoch-Schonlein Purpura

46
Q

Twice as many boys are girls are affected.

A

Henoch-Schonlein Purpura

47
Q

Clinical presentation:
○ Malaise, headache, fever, and rash.

A

Henoch-Schonlein Purpura

48
Q

The skin lesions are urticarial and gradually become pinkish, then red, and finally hemorrhagic.
○ “Palpable purpura”
○ Most commonly found on the feet, elbows, knees, buttocks,
and chest.

A

Henoch-Schonlein Purpura

49
Q

Henoch-Schonlein Purpura
● Laboratory Findings:

A

○ Normal PLT count
○ Elevated WBC count
○ Elevated ESR
○ Normal Coagulation studies
○ Proteinuria and Hematuria
○ Anemia (only if hemorrhagic manifestation is severe)

50
Q

Severe hemorrhagic manifestations as a result of a combination of hyperviscosity and platelet
dysfunction.

A

Dysproteinemia

51
Q

Inhibits platelet aggregation, secretion, and
procoagulant activity.

A

Dysproteinemia

52
Q

Coating of platelet membrane with paraprotein inhibits adhesion and activation receptor.

A

Dysproteinemia

53
Q

Also inhibits assembly of clotting factors on the platelet surface.

A

Dysproteinemia

54
Q

A disorder of women that presents with recurrent
purpura on the lower extremities and resultant
hemosiderin staining of the skin.

A

Waldenstrom Macroglobulinemia

55
Q

● Is caused by production of cryoprecipitable serum proteins or protein complexes.

A

Cryoglobulinemia

56
Q

Exposure to cold may cause purpura on the skin
of the extremities or face

A

Cryoglobulinemia

57
Q

The purpura may cause blister or ulcerate.

A

Cryoglobulinemia

58
Q

Autoimmune mechanisms

A

IgA Myeloma
IgG Myeloma

59
Q

● Interact with platelet receptors.
● Interfere with platelet aggregation.
● Increases plasma viscosity.

A

IgA Myeloma
IgG Myeloma

60
Q

refers to a misfolded, insoluble
protein that accumulates in tissues and organs,
forming deposits known

A

Amyloid protein

amyloid fibrils.

61
Q

Resistant to normal protein degradation process.

A

Amyloidosis

62
Q

Deposited extracellularly and may damage tissue.

A

Amyloidosis

63
Q

Purpura, hemorrhage, and thrombosis maybe part of the clinical presentation.

A

Amyloidosis

64
Q

Due to lack of collagen support for small blood vessels and loss of subcutaneous fats and elastic fibers

A

Solar purpura

65
Q

More common in elderly men than women.

A

Solar purpura

66
Q

Dark blotches are flattened and do not blanch with pressure. (Age Spots)

A

Solar purpura

67
Q

The lesions are limited mostly to the extensor
surfaces of the forearms and backs of the hands.

A

Solar purpura

68
Q

Caused by a deficiency of vitamin C.

A

Scurvy

69
Q

Causes defect in the synthesis of collagen in the walls of small blood vessels

A

Scurvy

70
Q

May present with swelling, pain, or discoloration.

A

Scurvy

71
Q

Gingival bleeding and hemarthrosis may also be
present.

A

Scurvy

72
Q

Perifollicular hemorrhages with hyperkeratosis of the hair follicles and “corkscrew” hairs are characteristic.

A

Scurvy

Hematology 2 PPT (3 decks)

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