Hema

• Hemostatic Disorders


  • Concept of Normal Coagulation
• Vascular Disorders


  • Purpura
• Hereditary Vascular Disorders


  • Hereditary Hemorrhagic Telangiectasia
  • Kasabach-Merritt Syndrome
  • Ehlers-Danlos Syndrome
  • Other Inherited Disorders
• Acquired Vascular Disorders


  • Allergic purpura
  • Paraproteinemia and Amyloidosis
  • Senile purpura
  • Drug-induced vascular purpuras
  • Purpura associated with Infections
  • Scurvy
• Purpuras of Unknown Origin


  • Purpura simplex
  • Psychogenic purpura
• Hemostasis


  The process of maintaining balance in our blood; Making sure that blood all the time stays on its liquid state
• Plasmin


  Enzyme responsible for the removal of the clot
• In the absence of an injury, we should prevent the formation of a clot
• If you have a problem with this balance, expect that there will be a problem in hemostatic mechanism. That problem could either lead to bleeding or thrombosis
• Purpura


  Clinical manifestation of red blood cell extravasation into mucosa or skin, results from various conditions, including rheumatologic, infectious, dermatologic, traumatic, and hematologic disorders
• Purpuric lesions


  RBCs leave the vessels fast if you have: infections, allergies, blood disorders, exposed to trauma or pressure (e.g. Dengue)
• Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber syndrome)

  • Autosomal Dominant
  • Thin and dilated vessels
  • Most common inherited vascular disorder that leads to vascular malformation
  • Patients experience epistaxis (nose bleeding) and iron deficiency anemia
  • Coagulation tests (PT and APTT) are normal
• Kasabach-Merritt Syndrome


  • Vascular trauma
  • Thrombocytopenia
  • Bleeding diathesis
  • Corticosteroid or Surgery
  • Condition composed of tumors that surrounds the blood vessels, leading to thrombocytopenia and bleeding
• Ehlers-Danlos Syndrome


  • Autosomal Dominant, recessive, or X-linked trait
  • Manifestations: Hyperextensible skin, Hypermobile joints, Joint laxity, Fragile tissues, Bleeding tendency
  • Main problem is lack of collagen due to lack of peptidase enzyme
  • Coagulation tests and platelet count are normal
• Ehlers-Danlos syndrome


  • Hyperextensible skin
  • Hypermobile joints
  • Joint laxity
  • Fragile tissues
  • Bleeding tendency, primarily subcutaneous hematoma formation
• Collagen


  Main problem is: You don't have enough collagen
• Peptidase enzyme


  You lack peptidase enzyme thus not being able to convert procollagen to become collagen
• Your skin resembles the skin of an old individual
• Coagulation tests and platelet count: NORMAL
• No known therapy given
• Ehlers-Danlos syndrome


  May be transmitted as an autosomal dominant, recessive, or X-linked trait
• Varieties of Ehlers-Danlos syndrome


  • 11 distinct varieties are recognized
• Bleeding tendency in Ehlers-Danlos syndrome


  Ranges from easy bruisability to arterial rupture
• Cause of Ehlers-Danlos syndrome


  Defects in collagen production, structure, or crosslinking, with resulting inadequacy of the connective tissues
• Platelet abnormalities have been reported in some patients with Ehlers-Danlos syndrome
• Other inherited vascular disorders

  • Pseudoxanthoma Elasticum
  • Homocystinuria
  • Marfan syndrome
  • Osteogenesis Imperfecta
• Marfan syndrome


  • Elongated extremities and fingers which are not proportion to their bodies
  • Blood vessels in their eyes are thin and affected, may even lead to blindness
• Osteogenesis imperfecta


  • Imperfect formation of bone tissues
  • Patients may demonstrate bruising, epistaxis, and sometimes intracranial bleeding
  • Bones become soft and fragile, cannot walk
• Pseudoxanthoma elasticum


  • Autosomal recessive disorder affecting elastic fibers
  • Blood vessels should be both flexible and elastic
  • Elastic fibers are calcified, becomes harder and brittle, prone to damage
• Acquired vascular disorders are caused by exposure to something that damages the blood vessels
• Allergic purpura (Henoch-Schonlein purpura)

  Nonthrombocytopenic purpura: allergic manifestation - skin rash and edema
• Henoch-Schonlein purpura


  • Acute IgA-mediated disorder
  • Commonly in children 3-7 years old
  • Not common among less than 2 years old and 20 years old and above
  • Coagulation tests and platelets: NORMAL
  • ESR and WBC is high
  • If the kidneys are affected, there may be hematuria or proteinuria
  • Could be associated with certain food, drugs, insect bites, vaccinations, etc.
  • Not associated with thrombocytopenia
  • Results from allergic vasculitis which can involve skin, kidneys, CNS, and even lungs
• Allergic purpura


  Allergic manifestation - skin rash and edema
• Henoch-Schonlein purpura


  Accompanied by transient arthralgia, nephritis, abdominal pain, and purpuric skin lesions
• Pathophysiology of allergic purpura

  Autoimmune vascular injury, vasculitis mediated by immune complexes containing IgA antibodies
• Paraproteinemia


  Coating of the platelet membrane with paraproteins, inhibition of fibrin polymerization
• Amyloidosis


  Deposition of abnormal quantities of amyloid protein in tissues
• Paraproteinemia


  Accumulation of unwanted/excess proteins (paraproteins) in blood
• Amyloidosis


  Protein precipitates that get deposited in tissues and vessels, leading to damage and abnormal function
• Paraprotein effect on platelets

  • Inhibits platelet aggregation, secretion, and procoagulant activity
  • Inhibits assembly of clotting factors on the platelet surface