DISORDERS OF PRIMARY HEMOSTASIS – VASCULAR DISORDERS Flashcards
HEREDITARY VASCULAR DISORDERS
hereditary hemorrhagic telangiectasia
hemangioma thrombocytopenia syndrome
ehlers danlos syndrome
marfan syndrome
pseudoxanthoma elasticum
osteogenesis imperfecta
2 categories of vascular disorders
hereditary and acquired vascular disorders
ACQUIRED VASCULAR DISORDERS
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)
HEREDITARY HEMORRHAGIC TELANGIECTASIA is also called as
osler weber rendu syndrome
Characterized by thin-walled blood vessels with a discontinuous endothelium, inadequate smooth muscle,
inadequate or missing elastin that surrounds the stroma
HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)
mode of inheritance of hereditary hemorrhagic telangiectasia
autosomal dominant 50% chance of transferring to offspring
__are dilated superficial blood vessels that create small, focal red lesions
Telangiectasias
Vascular malformation involves vessels throughout the body, which are dilated, tortuous and disorganized.
o Making them prone to rupture
HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)
It is characterized by the presence of widespread telangiectatic lesions (“spider veins”) of the skin and mucous
membrane
HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)
HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)
lesions develop on
tongue, lips, palate, face, and hands
characteristics of telangiectatic lesion of HHT
reddish or purple
dilated blood vessels
tortuous (matigas)
disorganized
main problem or cause of hereditary hemorrhagic telangiectasia
ENG1, ACVRL 1, SMAD4
function of ENG1 in HHT
encodes protein that is part of TGF - beta transforming growth factor which is a receptor
purpose of ENG1 and its importance
for the blood vessel component and repair
ACVRL1 transpose
activin A receptor type 2 like 1
or activin A receptor-like 1 type 2
activin A receptor type 2 like 1
or activin A receptor-like 1 type 2
what is its purpose
plays a critical role in BV in development and as well as its maintenance
SMAD4 is for
critical in cell growth and development
type of anemia that is usually seen in hereditary hemorrhagic telangiectasia
IDA
Telangiectasias are also present in what conditions
cherry-red hemangiomas,
ataxia-telangiectasia (Louis-Br syndrome),
chronic actinic telangiectasia,
chronic liver disease
pregnancy
patients with HHT will have
frequent nose bleeding
skin lesion red or purple
GIT bleeding leading to anemia
descibe the BT, PFT, tourniquet test and coagulation test in hereditary hemorrhagic telangiectasia
NORMAL
universal finding of hereditary hemorrhagic telangiectasia
epistaxis
a good gauge of the severity of the disease of hereditary hemorrhagic telangiectasia
age at which the epistaxis begins
diagnosis of hereditary hemorrhagic telangiectasia is based on
findings of telangiectasia
history of repeated hemorrhage
family history
describe the platelet count of hereditary hemorrhage telangiectasia
decrease or normal
treatment or supportive care for hereditary hemorrhagic telangiectasis
oral iron for anemia
therapeutic drugs
surgical technique
corticosteroids
sclerotherapies
HEMANGIOMA THROMBOCYTOPENIA SYNDROME is also called as
kasabach-meritt syndrome
treatment for hereditary hemorrhage telangiectasia
oral iron (ferrous sulphate) - anemia
therapeutic drugs to stop or prevent bleeding
surgical technique to stop or prevent bleeding
corticosteroids
sclerotherapies
describe the fibrinogen of hereditary hemorrhage telangiectasia
low fibrinogen level
external hemangiomas may become engorged with blood and resembles what
hematomas
location or area of hemangioma thrombocytopenia syndrome
are visceral or subcutaneous but rarely both
well organized features of hemangioma thrombocytopenia syndrome includes
acute or chronic DIC
MAHA - Microangiopathic hemolytic anemia
contributing factors of hemangioma thrombocytopenia syndrome
platelet trapping or platelet consumption
hemangioma thrombocytopenia syndrome is also known as
kasabach-merritt syndrome
tumors of hemangioma thrombocytopenia syndrome
hemangioendothelioma
tufted angioma
which one is benign
hemangioendothelioma
tufted angioma
tufted angioma is the one benign and hemangioendothelioma is the one malignant
a very rare vascular tumor that arises from the endothelial cells lining of blood vessel
hemangioendothelioma
tufted angioma usually affects what population
infants
thrombocytopenia is usually sever and associated with what conditions
DIC
MAHA
characteristics of MAHA
presence of schistocytes
bleeding diathesis means
tendency or habit to bleed
bleeding diathesis is seen in what conditions
hemangioma thrombocytopenia syndrome (kasabach merritt syndrome)
cause of kasabach merritt
plt trapping and consumption
tx we can provide for kasabach merritt
includes corticosteroid therapy and surgery
administration of plt concentrate or fresh frozen plasma
corticosteroid
surgery to remove the tumor
