Vascular Disorders (F) Flashcards
What are vascular disorders?
These are disorders affecting the blood vessels or vasculature resulting to easy bruising and spontaneous bleeding
How is dx made for vascular disorders?
Dx is made by ruling out other sources of hemostatic disorders
What are the true vascular disorders?
1) Rendu-Osler-Weber Syndrome
2) Kasabach-Merritt Syndrome
3) Ehlers-Danlos Syndrome
4) Henoch-Schonlein Purpura
5) Senile Purpura
Answer the ff questions:
Given disease: Rendu-Osler-Weber Syndrome
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) Hereditary hemorrhagic telangiectasia
2) Autosomal dominant
3) a. Thin-walled blood vessels
b. Discontinuous endothelium
c. Inadequate smooth muscle
d. Inadequate or missing elastin
4) a. Telangiectasia - dilated superficial blood vessels that create small, focal red lesions
b. Associated w/ cherry-red hemangiomas, ataxia-telangiectasia, chronic actinic telangiectasia, chronic liver disease, pregnancy
5) a. Epistaxis - good gauge of severity
b. Oral cavity - common sites of bleeding
c. GIT - common sites of bleeding
d. Urogenital tract - common sites of bleeding
6) a. Bleeding time: prolonged
7) Not mentioned
Answer the ff questions:
Given disease: Kasabach-Merritt Syndrome
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) Hemangioma-thrombocytopenia syndrome
2) Unknown; present at birth
3) a. Sequestration of PLTs in the hemangiomas
4) a. Associated w/ DIC, microangiopathic hemolytic anemia
5) a. Visceral, subcutaneous or cutaneous hemangioma
b. Giant cavernous hemangioma or vascular tumor
c. Bleeding diathesis
6) a. PLT ct: decreased
b. Bleeding time: prolonged
7) a. Corticosteroids
b. Surgery
Answer the ff questions:
Given disease: Ehlers-Danlos Syndrome
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) None
2) Autosomal dominant, autosomal recessive, X-linked
3) a. Defects in collagen production
b. Defects in collagen structure
c. Inadequacy of the connective tissues
4) a. 11 distinct varieties of this disorder were identified
5) a. Hyperextensible skin
b. Hypermobile joints
c. Fragile tissues
d. Subcutaneous hematoma
e. Bleeding of easy bruising to arterial rupture
6) a. Bleeding time: prolonged
b. PLT abnormalities: present
7) Not mentioned
Answer the ff questions:
Given disease: Henoch-Schonlein Purpura
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) Allergic purpura; anaphylactoid purpura
2) None
3) a. Immune complexes w/ IgA Abs
b. IgA autoimmunity against blood vessel wall components
c. Presence of infectious organism that could damage the endothelial lining of blood vessels
4) a. Associated w/ certain foods, certain drugs, cold, insect bites, and vaccinations
b. Complications include polyarthralgia and renal disease
5) a. Malaise
b. Headache
c. Fever
d. Edema
e. Abdominal pain
f. Petechiae
g. Renal lesions
h. Urticarial skin rash - pinkish -> red -> hemorrhagic
i. Palpable purpura - appears very rapid and itchy
j. Brownish-red eruption
6) a. WBC ct: increased
b. ESR: increased
c. PLT ct: normal
d. Proteinuria: present
e. Hematuria: present
7) a. Corticosteroids
Answer the ff questions:
Given disease: Senile Purpura
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) Actinic purpura
2) None
3) a. Lack of collagen support for small blood vessels
b. Loss of subcutaneous fat and elastic fibers
c. Increased capillary fragility
4) a. Associated w/ advancing age
b. More common in elderly men than in women
5) a. Dark blotch - lesions that do not blanch w/ pressure and resolve slowly that leave a brown stain in the skin
b. Lesions in extensor surfaces of the forearm, back of the hands, face, and neck
6) a. Coagulation tests: normal
b. PLT ct: normal
7) Not mentioned
What are the other conditions that affects blood vessels?
1) Paraproteinemia
2) Amyloidosis
3) Other hereditary disorders
a. Autosomal dominant disorders
- > Marfan syndrome
- > Osteogenesis imperfecta
b. Autosomal recessive disorders
- > Pseudoxanthoma elasticum
- > Homocystinuria
4) Vitamin C deficiency
Answer the ff questions:
Given disease: Paraproteinemia
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) None
2) None
3) a. Inhibition of adhesion and activation receptors
b. Inhibition of clotting factor assembly on PLT surface
c. Inhibition of fibrin polymerization
4) a. Associated w/ IgA myeloma, IgG myeloma, and Waldenstrom macroglobulinemia
b. Can become severe if hyperviscosity is present
5) a. Bleeding
6) a. Bleeding time: prolonged
b. TT: prolonged
c. PT: prolonged
d. Activated PTT: prolonged
7) a. Therapeutic plasma exchange
b. Chemotherapy if plasma cell malignancy is present
Answer the ff questions:
Given disease: Amyloidosis
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) None
2) None
3) a. Amyloid deposition in vascular walls
4) a. Amyloid - fibrous protein composed of aggregated fibrils; various proteins can serve as subunits of fibril
5) a. Purpura
6) a. PLT ct: decreased
7) a. Chemotherapy w/ allogenic stem cell transplantation
Answer the ff questions:
Given disease: Vitamin C deficiency
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) Scurvy
2) None
3) a. Insufficient consumption of vitamin C-rich foods
b. Lack of vitamin C needed for collagen production
4) None
5) a. Spontaneous bleeding
b. Ulceration of gums
c. Loss of teeth
6) None
7) a. Oral vitamin C supplements
b. Intravenous vitamin C supplements
What are the miscellaneous therapeutic drugs that affect the blood vessels?
1) Drug-induced Vasculitis
Answer the ff questions:
Given miscellaneous therapeutic drug: Drug-induced Vasculitis
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) None
2) None
3) a. Development of Abs against vessel wall components
b. Development of immune complexes
c. Change in vessel wall permeability
4) a. Associated w/ drugs include aspirin, warfarin, barbiturates, diuretics, digoxin, methyldopa, and several antibiotics; most frequent cause are sulfonamides and iodides
5) a. Few petechiae to generalized petechial eruptions
6) None
7) a. Discontinue offending drug
