VASCULAR DISORDERS Flashcards
A.k.a OSLER WEBER RENDU DISEASE
Hereditary hemorrhagic telangiectasia
Gene mutation in Hereditary hemorrhagic telangiectasia
Autosomal dominant
Juvenile type in Hereditary hemorrhagic telangiectasia
SMAD4
Type III IN Hereditary hemorrhagic telangiectasia
Unknown
Type II in Hereditary hemorrhagic telangiectasia
ACVRL
Type I Hereditary hemorrhagic telangiectasia
ENG
Other name for congenital hemangiomata
Kasabach-Merrit Syndrome
Hemangioma thrombocytopenia syndrome
Transcribe DIC
Disseminated intravascular coagulation
Indicative of DIC
Congenital hemangiomata
Vascular disorder common to newborn
Congenital hemangiomata
Immunoglobulin deposit in the cutaneous BV resulting to fragility, purpuraa d trauma
Multiple myeloma
Due to abnormal production of L chain by plasma cells in the BM
Systemic amyloidosis
Characterized by insoluble amyloid filament attached to a polysaccharide
Systemic amyloidosis
Causes cerebral infarcts in children and intracranial hemorrhage in adult
Moyamoya disease
Blockage of arteries
Moyamoya disease
Results to fragile arterioles causing recurrent ischemic strokes and cognitive impairement
Cerebral small vessel disease
Causes narrowing and weakening of BV
Vasculitis
Causes narrowing and weakening of BV
Vasculitis
Seen in SLE and wegener’s granulomatosis
Antineutrophilic cytoplasmic antibody-positive
Transcribe ANCA
Antineutrophilic cytoplasmic antibody-positive
Targets the primary granules of neutrophils
Antineutrophilic cytoplasmic antibody-positive
What cANCA targets
Proteinase 3
What does pANCA targets
Myeloperoxidase
Associated with cryoglobulin deposits
Cryoglobulinemia
Characteristic of blood in cryoglobulinemia
Hyper viscous
Cryoglobulin precipitates at what temperature
Cold temperature
What factor is low in cryoglobulinemia
FXIII
Seen in waldenstrom’s primary macroglobulinemia
Cryoglobulinemia
What are the infected associated prupura
- Rickettsial disease
- Brazillian purpura fever
- Rat Bite fever
- Vibrio vulniricans infection
- Strongyloides
- Acquired purpura fulminans
Occurs to women during menstrual cycle
Purpura simplex
Miscellaneous causes of purpura can be a result of
- Contact dermatitis
- Steroid use
- Drug reaction
- Allergies
Observed in miscellaneous causes of purpura
- Kaposi sarcoma
- Vascular tumor
Observed in miscellaneous causes of purpura
- Kaposi sarcoma
- Vascular tumor