DISORDERS OF PRIMARY HEMOSTASIS – VASCULAR DISORDERS

Question 1

HEREDITARY VASCULAR DISORDERS

Answer 1

hereditary hemorrhagic telangiectasia
hemangioma thrombocytopenia syndrome
ehlers danlos syndrome
marfan syndrome
pseudoxanthoma elasticum
osteogenesis imperfecta

Question 2

2 categories of vascular disorders

Answer 2

hereditary and acquired vascular disorders

Question 3

ACQUIRED VASCULAR DISORDERS

Answer 3

allergic purpura (henoch schonlein)
purpura associated with infections (septic emboli and purpura fulminans)
purpura secondary to dysproteinemia (waldernstroms’s purpura, cryoglibulinemia, hyperviscosity syndrome, amyloidoisis)
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

mode of inheritance of hereditary hemorrhagic telangiectasia

Answer 6

autosomal dominant 50% chance of transferring to offspring

Question 7

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

Answer 7

Telangiectasias

Question 8

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

Answer 8

HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)

Question 9

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

Answer 9

HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)

Question 10

HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)

lesions develop on

Answer 10

tongue, lips, palate, face, and hands

Question 11

characteristics of telangiectatic lesion of HHT

Answer 11

reddish or purple
dilated blood vessels
tortuous (matigas)
disorganized

Question 12

main problem or cause of hereditary hemorrhagic telangiectasia

Answer 12

ENG1, ACVRL 1, SMAD4

Question 13

function of ENG1 in HHT

Answer 13

encodes protein that is part of TGF - beta transforming growth factor which is a receptor

Question 14

purpose of ENG1 and its importance

Answer 14

for the blood vessel component and repair

Question 15

ACVRL1 transpose

Answer 15

activin A receptor type 2 like 1

or activin A receptor-like 1 type 2

Question 16

activin A receptor type 2 like 1

or activin A receptor-like 1 type 2

what is its purpose

Answer 16

plays a critical role in BV in development and as well as its maintenance

Question 17

SMAD4 is for

Answer 17

critical in cell growth and development

Question 18

type of anemia that is usually seen in hereditary hemorrhagic telangiectasia

Answer 18

IDA

Question 19

Telangiectasias are also present in what conditions

Answer 19

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

Question 20

patients with HHT will have

Answer 20

frequent nose bleeding
skin lesion red or purple
GIT bleeding leading to anemia

Question 21

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

Answer 21

NORMAL

Question 22

universal finding of hereditary hemorrhagic telangiectasia

Answer 22

epistaxis

Question 23

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

Answer 23

age at which the epistaxis begins

Question 24

diagnosis of hereditary hemorrhagic telangiectasia is based on

Answer 24

findings of telangiectasia
history of repeated hemorrhage
family history

Question 25

describe the platelet count of hereditary hemorrhage telangiectasia

Answer 25

decrease or normal

Question 26

treatment or supportive care for hereditary hemorrhagic telangiectasis

Answer 26

oral iron for anemia therapeutic drugs surgical technique corticosteroids sclerotherapies

Question 27

HEMANGIOMA THROMBOCYTOPENIA SYNDROME is also called as

Answer 27

kasabach-meritt syndrome

Question 28

treatment for hereditary hemorrhage telangiectasia

Answer 28

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

Question 29

describe the fibrinogen of hereditary hemorrhage telangiectasia

Answer 29

low fibrinogen level

Question 30

external hemangiomas may become engorged with blood and resembles what

Answer 30

hematomas

Question 31

location or area of hemangioma thrombocytopenia syndrome

Answer 31

are visceral or subcutaneous but rarely both

Question 32

well organized features of hemangioma thrombocytopenia syndrome includes

Answer 32

acute or chronic DIC MAHA - Microangiopathic hemolytic anemia

Question 33

contributing factors of hemangioma thrombocytopenia syndrome

Answer 33

platelet trapping or platelet consumption

Question 34

hemangioma thrombocytopenia syndrome is also known as

Answer 34

kasabach-merritt syndrome

Question 35

tumors of hemangioma thrombocytopenia syndrome

Answer 35

hemangioendothelioma tufted angioma

Question 36

which one is benign hemangioendothelioma tufted angioma

Answer 36

tufted angioma is the one benign and hemangioendothelioma is the one malignant

Question 37

a very rare vascular tumor that arises from the endothelial cells lining of blood vessel

Answer 37

hemangioendothelioma

Question 38

tufted angioma usually affects what population

Answer 38

infants

Question 39

thrombocytopenia is usually sever and associated with what conditions

Answer 39

DIC MAHA

Question 40

characteristics of MAHA

Answer 40

presence of schistocytes

Question 41

bleeding diathesis means

Answer 41

tendency or habit to bleed

Question 42

bleeding diathesis is seen in what conditions

Answer 42

hemangioma thrombocytopenia syndrome (kasabach merritt syndrome)

Question 43

cause of kasabach merritt

Answer 43

plt trapping and consumption

Question 44

tx we can provide for kasabach merritt

Answer 44

includes corticosteroid therapy and surgery administration of plt concentrate or fresh frozen plasma corticosteroid surgery to remove the tumor