Vasculopathy Flashcards
What is vasculopathy?
Vasculopathy is a disorder of the blood vessels characterized by occlusion or dysfunction rather than by inflammation of the vessel wall.
Often due to problems with clotting, typically an inherited or acquired issue with platelets or the coagulation pathway.
What is Factor 5 Leiden?
Factor V that is resistant to degradation by protein C, leading to activated coagulation cascade
This is an inherited coagulopathy.
What are the consequences of Protein C/S deficiency?
Incapable of deactivating factors V & VIII, resulting in activated coagulation cascade
This condition is often referred to as having ‘broken brakes’ in coagulation.
What does an Antithrombin III mutation do?
Blocks factors II & X, leading to a ‘broken brakes’ effect in coagulation
This contributes to increased risk of thrombosis.
What is hyperhomocyteinemia and its associated risk?
A condition that increases the risk of thrombosis by 2-4 times
It is often linked with cardiovascular issues.
How does sickle cell disease contribute to clotting?
Acidosis or low O2 leads to sticky sickle cells, promoting clotting
This is a significant concern in patients with sickle cell disease.
What is anti-phospholipid syndrome?
A condition where patients are more prone to forming clots
Look for history of stroke, MI, DVT, PE, miscarriages.
What are some cutaneous changes related to anti-phospholipid syndrome?
Livedo reticularis, splinter hemorrhages, retiform purpura
These are related to occlusion of blood vessels.
What impact does liver disease have on coagulation?
Affects coagulation production and platelet function
This can lead to increased bleeding risk.
What is purpura fulminans?
Acute condition with disseminated intravascular coagulation (DIC)
Patients typically present as acutely sick.
List risk factors for acquired coagulation disorders.
- Immobilization
- Obesity
- Cancer
- Pregnancy
- Smoking
- Oral contraceptives
These factors can significantly increase the risk of clotting issues.
What is ITP?
Idiopathic Thrombocytopenic Purpura, an autoimmune destruction of platelets
Caused by IgG autoantibodies leading to macrophage destruction in the spleen.
What causes TTP?
Deficiency in ADAMS TS13, leading to platelet aggregation and thrombosis
Remember the acronym ‘FAT RN’: Fevers, Anemia, Thrombocytopenia, Renal, Neurologic.
What does DIC stand for?
Disseminated Intravascular Coagulation
It leads to massive activation of coagulation, ischemia, and diffuse thrombosis.
What is HIT?
Heparin Induced Thrombocytopenia
A condition where heparin causes a drop in platelet count.
Name medications that can cause abnormal platelet function.
- Aspirin
- NSAIDs
These medications can impair platelet aggregation.
What are cholesterol emboli?
Cholesterol fragments that dislodge from a plaque, plugging arterioles
Can occur spontaneously or after cardiac procedures and are associated with peripheral eosinophilia.
What characterizes Schamberg’s disease?
Petechiae with golden-brown hemosiderin staining on lower legs
Typically affects middle-aged adults.
What is the primary diagnosis method for vasculopathy?
Biopsy showing fibrin thrombi with minimal inflammation and no leukocytoclastic vasculitis
This helps differentiate it from other conditions.
What screening labs are used for vasculopathy?
- PT
- PTT
- INR
- Protein C/S
- Factor V Leiden
- Antithrombin III
- Prothombin 22-10 gene mutations
- Antiprothrombin antibodies
- Homocysteine levels
These tests help assess coagulation status.
What is the treatment focus for vasculopathy?
Targets underlying disease, often in conjunction with a hematologist
Treatment may include addressing specific coagulopathies.
What is the treatment for pigmented purpuras?
Topical steroids + vitamin C 500mg BID + rutoside 50mg BID
This combination aims to reduce inflammation and improve skin appearance.
