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2: Vascular Disorders Flashcards

1
Q

A generalized bleeding that is seen in acquired or congenital defects in secondary hemostasis

A

Anatomic hemorrhage

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2
Q

Hemorrhagic spot, 1 cm or larger in diameter typically forming an irregular blue or purplish patch. Also known as bruise

A

Ecchymoses

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3
Q

Nosebleed that requires intervention

A

Epistaxis

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4
Q

Bleeding from multiple sites, spontaneous and recurrent bleeds or hemorrhage that requires physical intervention of transfusion

A

Generalized hemorrhage

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5
Q

Chronic joint bleed that cause inflammation and immobilization

A

Hemarthroses

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6
Q

Vomiting of bright red blood

A

Hematemesis

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7
Q

Localized collection of extravasated blood, usually clotted, in an organ space or tissue

A

Hematoma

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8
Q

Intact RBCS in the urine

A

Hematuria

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9
Q

Free hemoglobin in the urine

A

Hemoglobinuria

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10
Q

Expectoration of blood secondary to hemorrhage in the larynx, trachea, bronchi, and lungs

A

Hemoptysis

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11
Q

Acute severe blood loss requiring intervention and transfusion

A

Hemorrhage

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12
Q

Containing dark red or black blood

A

Melena stool

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13
Q

Abnormally heavy or prolonged menstrual periods

A

Menorrhagia

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14
Q

A generalized bleeding that may indicate a disorder of primary hemostasis

A

Mucocutaneous hemorrhage or systemic bleeding

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15
Q

Pinpoint purple or red spots on the skin or mucous membranes, approx 1 mm in diameter

A

Petechiae

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16
Q

Purple skin discoloration, typically rounded with a diameter of greater than 3 mm

A

Purpura

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17
Q

Formation, development, or presence of a clot in a blood vessel

A

Thrombosis

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18
Q

Hereditary vascular disorders:

A
  1. Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)
  2. Hemangioma-thrombocytopenia syndrome (Kasabach-Merritt syndrome)
  3. Ehlers-Danlos syndrome
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19
Q

Acquired vascular disorders:

A
  1. Allergic purpura (Henoch-Schonlein purpura)
  2. Parapoteinemia and Amyloidosis
  3. Senile purpura
  4. Drug-induced vascular purpuras
  5. Vitamin C deficiency (Scurvy)
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20
Q

Most common inherited disorder

A

Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)

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21
Q

Characterized by a thin-walled blood vessels with discontinuous endothelium, inadequate smooth muscle, and inadequate/missing elastin in the surrounding stoma

A

Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)

22
Q

Wall of the affected blood vessels are thin and lack of smooth muscle

A

Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)

23
Q

Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) universal finding

A

Epistaxis (nosebleed)

24
Q

Disorders associated with tumors composed of blood vessels that commonly swell and bleed at the surface

A

Hemangioma-thrombocytopenia syndrome (Kasabach-Merritt syndrome)

25
Q

Manifested by hyperextensive skin, hypermobile joints, joint laxity, fragile tissues, bleeding tendency, and primarily subcutaneous hematoma formation

A

Ehlers-Danlos Syndrome

26
Q

Connective tissue elastic fibers in small arteries are calcified and structurally abnormal

A

Pseudoxanthoma Elasticum

27
Q

Subarachnoid and gastrointestinal bleeding are the most common causes of death

A

Pseudoxanthoma Elasticum

28
Q

Characterized by skeletal and ocular defects

A

Marfan syndrome

29
Q

More appropriately applied when the conditions is an acute IgA-mediated disorder

A

Henoch-Schönlein Purpura

30
Q

Universal sign of Allergic purpura (Henoch-Schonlein purpura)

A

Reddish-purple spots

31
Q

Primarily a disease of children (3-7) years old

A

Allergic purpura (Henoch-Schonlein purpura)

32
Q

Platelet function can be inhibiting by myeloma proteins

A

Paraproteinemia

33
Q

Common in elderly men due to lack of collagen support for small blood vessels

A

Senile purpura

34
Q

Most frequent drugs in drug-induced vascular purpura

A

Sulfonamides
Iodides

35
Q

ROW syndrome mode of inheritance

A

Autosomal dominant

36
Q

Lesions blanch when pressure is applied

A

Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)

37
Q

Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome) usually manifests by

38
Q

Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome) type of mutations:

A

Type 1: ENG gene
Type 2: ACRVL1 gene
Type 3: Unknown

39
Q

Juvenile Polyposis/HHT is caused by mutations in the gene

40
Q

Diagnosis of Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)

A
  • Spontaneous recurrent epistaxis
  • cutaneous telangiectasia
  • visceral involvement
  • familial heredity
41
Q

Associations of Kasabach-Merritt Syndrome:

A
  • Giant Cavernous Hemangioma (Vascular tumor)
  • Thrombocytopenia
  • Bleeding Diathesis
42
Q

Complications of Kasabach-Merritt Syndrome:

A
  • microangiopathic hemolytic anemia
  • sequestration of platelets in hemangiomas
  • acute or chronic DIC
43
Q

Can be ascribed to defects in collagen production, structure, or cross-linking, with resulting inadequacy of the connective tissues

A

Ehlers-Danlos Syndrome

44
Q

Mode of inheritance of EDS

A

Autosomal dominant

45
Q

Greater risk for major bleeding of EDS

46
Q

EDS IV is a structural defect in proa1 (III) chain of collagen encoded by

A

COL3A1 gene

47
Q

Pseudoxanthoma Elasticum is also known as

A

Groenberg-Stranberg Syndrome

48
Q

Pseudoxanthoma Elasticum mode of inheritance

A

Autosomal recessive

49
Q

Pseudoxanthoma Elasticum mutations in the gene

A

ABC-C6 gene

50
Q

The skin lesions are urticarial and gradually becomes pinkish, then red, and finally hemorrhagic

A

Henoch-Schenlein Purpura

51
Q

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

A

Dysproteinemia

52
Q

Do not blanch with pressure

A

Senile purpura

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