DISORDERS OF PRIMARY HEMOSTASIS – VASCULAR DISORDERS

Question 1

HEREDITARY VASCULAR DISORDERS

Answer 1

hereditary hemorrhagic telangiectasia
hemangioma thrombocytopenia syndrome
ehlers danlos syndrome
other genetic disorders

Question 2

acquired vascular disorders

Answer 2

allergic purpura

Question 3

ACQUIRED VASCULAR DISORDERS

Answer 3

allergic purpura (henoch schonlein)
paraproteinemia and amyloidosis
senile purpura
drug induced vascular purpura
vitamin c deficiency (scurvy)

Question 4

HEREDITARY HEMORRHAGIC TELANGIECTASIA is also called as

Answer 4

osler weber rendu syndrome

Question 5

Characterized by thin-walled blood vessels with a discontinuous endothelium, inadequate smooth muscle,
inadequate or missing elastin that surrounds the stroma

Answer 5

HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)

Question 6

__are dilated superficial blood vessels that create small, focal red lesions

Answer 6

Telangiectasias

Question 7

Vascular malformation involves vessels throughout the body, which are dilated, tortuous and disorganized.
o Making them prone to rupture

Answer 7

HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)

Question 8

It is characterized by the presence of widespread telangiectatic lesions (“spider veins”) of the skin and mucous
membrane

Answer 8

HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)

Question 9

HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)

lesions develop on

Answer 9

tongue, lips, palate, face, and hands

Question 10

type of anemia that is usually seen in hereditary hemorrhagic telangiectasia

Answer 10

IDA

Question 11

Telangiectasias are also present in what conditions

Answer 11

cherry-red hemangiomas,
ataxia-telangiectasia (Louis-Br syndrome),
chronic actinic telangiectasia,
chronic liver disease
pregnancy

Question 12

descibe the BT, PFT, tourniquet test and coagulation test in hereditary hemorrhagic telangiectasia

Answer 12

NORMAL

Question 13

universal finding of hereditary hemorrhagic telangiectasia

Answer 13

epistaxis

Question 14

a good gauge of the severity of the disease of hereditary hemorrhagic telangiectasia

Answer 14

age at which the epistaxis begins

Question 15

diagnosis of hereditary hemorrhagic telangiectasia is based on

Answer 15

findings of telangiectasia
history of repeated hemorrhage
family history

Question 16

describe the platelet count of hereditary hemorrhage telangiectasia

Answer 16

decrease or normal

Question 16

HEMANGIOMA THROMBOCYTOPENIA SYNDROME is also called as

Answer 16

kasabach-meritt syndrome

Question 16

treatment for hereditary hemorrhage telangiectasia

Answer 16

oral iron (ferrous sulphate) - anemia
therapeutic drugs to stop or prevent bleeding
surgical technique to stop or prevent bleeding
corticosteroids
sclerotherapies

Question 16

describe the fibrinogen of hereditary hemorrhage telangiectasia

Answer 16

low fibrinogen level

Question 17

external hemangiomas may become engorged with blood and resembles what

Answer 17

hematomas

Question 17

location or area of hemangioma thrombocytopenia syndrome

Answer 17

are visceral or subcutaneous but rarely both

Question 18

well organized features of hemangioma thrombocytopenia syndrome includes

Answer 18

acute or chronic DIC
MAHA - Microangiopathic hemolytic anemia