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HEMATOLOGY 2 > DISORDERS OF PRIMARY HEMOSTASIS – VASCULAR DISORDERS > Flashcards

DISORDERS OF PRIMARY HEMOSTASIS – VASCULAR DISORDERS Flashcards

1
Q

HEREDITARY VASCULAR DISORDERS

A

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

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2
Q

2 categories of vascular disorders

A

hereditary and acquired vascular disorders

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3
Q

ACQUIRED VASCULAR DISORDERS

A

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)

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4
Q

HEREDITARY HEMORRHAGIC TELANGIECTASIA is also called as

A

osler weber rendu syndrome

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5
Q

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

A

HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)

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6
Q

mode of inheritance of hereditary hemorrhagic telangiectasia

A

autosomal dominant 50% chance of transferring to offspring

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7
Q

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

A

Telangiectasias

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8
Q

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

A

HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)

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9
Q

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

A

HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)

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10
Q

HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)

lesions develop on

A

tongue, lips, palate, face, and hands

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11
Q

characteristics of telangiectatic lesion of HHT

A

reddish or purple
dilated blood vessels
tortuous (matigas)
disorganized

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12
Q

main problem or cause of hereditary hemorrhagic telangiectasia

A

ENG1, ACVRL 1, SMAD4

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13
Q

function of ENG1 in HHT

A

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

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14
Q

purpose of ENG1 and its importance

A

for the blood vessel component and repair

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15
Q

ACVRL1 transpose

A

activin A receptor type 2 like 1

or activin A receptor-like 1 type 2

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16
Q

activin A receptor type 2 like 1

or activin A receptor-like 1 type 2

what is its purpose

A

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

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17
Q

SMAD4 is for

A

critical in cell growth and development

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18
Q

type of anemia that is usually seen in hereditary hemorrhagic telangiectasia

A

IDA

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19
Q

Telangiectasias are also present in what conditions

A

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

20
Q

patients with HHT will have

A

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

21
Q

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

A

NORMAL

22
Q

universal finding of hereditary hemorrhagic telangiectasia

A

epistaxis

23
Q

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

A

age at which the epistaxis begins

24
Q

diagnosis of hereditary hemorrhagic telangiectasia is based on

A

findings of telangiectasia
history of repeated hemorrhage
family history

25
Q

describe the platelet count of hereditary hemorrhage telangiectasia

A

decrease or normal

26
Q

treatment or supportive care for hereditary hemorrhagic telangiectasis

A

oral iron for anemia
therapeutic drugs
surgical technique
corticosteroids
sclerotherapies

27
Q

HEMANGIOMA THROMBOCYTOPENIA SYNDROME is also called as

A

kasabach-meritt syndrome

28
Q

treatment for hereditary hemorrhage telangiectasia

A

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

29
Q

describe the fibrinogen of hereditary hemorrhage telangiectasia

A

low fibrinogen level

30
Q

external hemangiomas may become engorged with blood and resembles what

A

hematomas

31
Q

location or area of hemangioma thrombocytopenia syndrome

A

are visceral or subcutaneous but rarely both

32
Q

well organized features of hemangioma thrombocytopenia syndrome includes

A

acute or chronic DIC
MAHA - Microangiopathic hemolytic anemia

33
Q

contributing factors of hemangioma thrombocytopenia syndrome

A

platelet trapping or platelet consumption

34
Q

hemangioma thrombocytopenia syndrome is also known as

A

kasabach-merritt syndrome

35
Q

tumors of hemangioma thrombocytopenia syndrome

A

hemangioendothelioma
tufted angioma

36
Q

which one is benign

hemangioendothelioma
tufted angioma

A

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

37
Q

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

A

hemangioendothelioma

38
Q

tufted angioma usually affects what population

A

infants

39
Q

thrombocytopenia is usually sever and associated with what conditions

A

DIC
MAHA

40
Q

characteristics of MAHA

A

presence of schistocytes

41
Q

bleeding diathesis means

A

tendency or habit to bleed

42
Q

bleeding diathesis is seen in what conditions

A

hemangioma thrombocytopenia syndrome (kasabach merritt syndrome)

43
Q

cause of kasabach merritt

A

plt trapping and consumption

44
Q

tx we can provide for kasabach merritt

A

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

45
Q
A

HEMATOLOGY 2 (18 decks)

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