PATH- 9-22 - ABNORMAL Hemostasis Flashcards
Thrombocythemia
When INC platelet count is a clonal proliferation and neoplastic.
A: 7 Causes of Thrombocytopenia
B: List AT LEAST 2 examples of each
C: Quantitative or Qualitative Platelet Disorder?
Thrombo
Thrombocytopenia (low platelet count) = HHID BAD
- Bone Marrow Alteration (marrow hypOplasia/aplasia/neoplastic replacement/leukemia)
- Hereditary Thrombocytopenia (Fanconi’s anemia / [May Hegglin anomaly]
- [Acquired Abnormal Hematopoiesis] (B12 Deficiency / PRE-Leukemia)
- Drug induced ([Heparin - HIT] / Quinidine / Sulfonamides)
- Dilutional (Hemodialysis / Heart-lung machine)
- ITP and TTP (IgG Immune Thrombocytopenic purpura) and (Thrombotic Thrombocytopenic purpura)
- HUS 2º to ESOH
A: causes of Thrombocytosis: (3)
B: Normally Platelets are between _______ in the blood. During [Autonomous Thrombocythemia] platelets reach over a _______ as _______ and can occlude small _______
C: Quantitative or Qualitative Platelet Disorder?
causes of Thrombocytosis:
1) Splenectomy - platelets are normal in function
2) Reactive Thrombocytosis to CA/ Drugs/Infection
3) Autonomous Thrombocythemia = clonal disorder
B: Normally Platelets are between [200-500 K] in the blood. During [Autonomous Thrombocythemia] platelets reach over a million as [platelet clusters] and can occlude small vessels
C: Quantitative
A: 3 Causes of QuaLitative Platelet Disorders
B: List 2 examples of each
- Dz:(Multiple Myeloma / Liver Dz / paraproteinemia)
- Drug: (Aspirin / NSAID)
- Diet: ([Omega 3 Fatty Acids])
Glanzmann’s thrombasthenia
B: Is this Autosomal Dominant or Recessive?
[GP-2b-3A] Defect that causes Aggregation defects and INC bleeding time
B: Autosomal recessive
A: What Platelet dz is characterized by [GP1B/2A] defect, INC bleeding time and adhesion problems?
B: Autosomal Dom or Recessive?
A: Bernard-Soulier disease
B: Autosomal Recessive
A: Storage Pool Dz
B: Gray Platelet Syndrome
A: Storage Pool Dz= DEC in [Dense Beta Granules] —> NO AGGREGATION
B: Gray Platelet Syndrome = Purpura development with no known origin
Polycythemia vera
[Myeloproliferative disorder of Platelets] in which HCT is 60 (normal = 45)
Vascular disorders are usually called ______ purpuras because these disorders do not result in ______
Platelet function and coagulation are ______.
C: 4 “shared” sx between Vascular Disorders and Platelet Disorders
Vascular disorders are usually called non-thrombocytopenic purpuras because these disorders do not result in severe bleeding diathesis.
Platelet function and coagulation are normal.
C:
Easy bruising,
- bleeding from mucosa,
- purpura,
- vasculitis
5 Causes of Congenital SubEndothelial Disorders
- Ehler Danlos Syndrome - Hypermobile joints and Hyper flexible skin,
- osteogenesis imperfecta,
- drugs,
- infections,
- amyloidosis
5 Causes of Acquired SubEndothelial Disorders
- Purpura simplex,
- amyloids,
- steroid purpura from prednisone
- Cushing’s syndrome (steroid excess),
- Henoch-Schonlin purpura (usually drug induced)
A: _____ causes of Endothelial disorders is the most common. These include: (3)
vs.
B: _____\_ Endothelial Disorders come from: (2)
A: CONGENITAL cause of Endothelial disorders is the most common. These include:
1) [Hereditary Hemorrhagic Telangiectasia - HHT]
2) Arteriovenous malformation
3) [Giant Hemangioma from (Kasaback-Merritt syndrome)]
B: ACQUIRED Endothelial Disorders come from:
- Inflammation
- Vasculitits from Rickettsia / Drugs / Viruses
Nutritional Disorder that can cause Abnormal Hemostasis
Scurvy (Vitamin C deficiency)
A: Defects in [Clotting Factor 8] =_______
B: Defects in [Clotting Factor 9] = _______ or _______
C: BOTH result in _______ and are transmitted as [sex linked _______]
D: Labs for these will show INC _______ with no change in _______
A: Defects in [Clotting Factor 8] =Hemophilia A
B: Defects in [Clotting Factor 9] = Hemophilia B or [Christmas Disease]
C: BOTH result in Bleeding and are transmitted as [sex linked recessive]
D: Labs for these will show INC aPTT with no change in platelets
A:
- Which coagulation pathway does aPTT measure?
- Which 4 factors are measured specifically?
B:
- Which coagulation pathway does PT measure?
- Which 5 factors are measured?
A: aPTT (intrinsic) –> 8, 9, 11 and 12 (actually measures all factors but 7, 13, protein C and S)
B: PT (extrinsic) [Thrombin Factor 2A], 5, 7, 10 and fibrinogen
A: Mechanism of Von Willebrand’s Disease (2)
B: [Von Willebrand factor] binds to what 3 glycoprotein receptors on platelets?
C: Describe Type 1 and Type 3 Von Willebrand’s Disease
D: Describe Type 2 Von Willebrand’s Disease
E: How does this affect Bleeding Time? aPTT?
A: Hemostatic defect due to [von Willebrand (ristocetin co-factor) and (factor 8 antigen)].
B: [vWillebrand factor] binds to platelet receptors (glycoprotein 1b, 2b, 3a) AND to collagen and subendothelium.
C: Type-1 and Type-3 von Willebrand’s diseases are characterized by a decrease in the circulating level of the vWF.
D: Type-2 von Willebrand’s disease is characterized by a qualitative defect in the vWF
E: Bleeding Time WILL be Elevated with [mild aPTT elevation due to Factor 8 reduction]
Hemophilia
A: Effect on Bleeding Time
B: Effect on aPTT
A: NO EFFECT ON BLEEDING TIME
B: aPTT is ELEVATED
A: Describe Primary Fibrinolysis
A2: This condition is seen abnormally in _____ (AKA ____)
B: [Fibrinogen Degradation products] are actually _____ (pro vs. anti) coagulants.
B2: How do they do this? (3)
C: What does OD of thrombolytic agents cause?
A:
In primary fibrinolysis, fibrinogen is converted into [fibrinogen degradation products] by plasmin.
A2: This condition is seen in dead fetus syndrome (Abruptio Placenta)
B: [Fibrinogen Degradation products] are actually ANTI-coagulants by inhibiting:
- thrombin
- [platelet aggregation]
- Fibrin Cross-linking
C: OD of thrombolytic agents –> [1ºFibrinolytic State]—> Bleeding
SECONDARY FIBRINOLYSIS (Mostly Caused by _______)
A: What occurs?
B: What does This results in?
C: What is a good lab indiciator for this state?
SECONDARY FIBRINOLYSIS (Mostly caused by Disseminated intravascular coagulation)
In secondary fibrinolysis both fibrin and fibrinogen are digested by plasmin.
B: This results in simultaneous digestion of clotting factors and consumption of platelets
C: D-Dimer levels are products of Fibrin breakdown and good indicator of DIC or [DVT/PE]
Factor 5 Leiden
B: This Disease is the most common inherited cause of ________ state
Occurs when [Factor 5] is mutated to lack clevage site for Protein C and S DeActivation
B: This Disease is the most common inherited cause of hypercoagulable state
Prothrombin 20210
Inherited Point mutation in ProThrombin resulting in INC gene expression —> INC Thrombin —> INC Thrombosis
Hyperhomocysteinemia (4)
A: Etiology
B: Sx (4)
- CBS enzyme normally converts Homocysteine—> cystathionine
- When CBS is deficient, Homocysteine accumulates and causes:
B: “Fuck My Trashy Life…I have CBS problems smh”
- Fingers that are slender and long
- Mental Retardation
- Thrombosis
- Lens Dislocation
