Platelet Disorders

• Small platelets: Wiskott-Aldrich Syndrome (WAS) is X-linked and a dense granules deficiency. The result is due to "TIE" thrombocytopenia, immunodeficiency, eczema
• Small platelets causes TORCH Infections; Toxoplasma, other agents, rubella, CMV, Herpesvirus
• Bernard-Soulier Syndrome (BSS) is a autosomal recessive that has a complex deficiency GP Ib/IX/V results in thrombocytopenia and bleeding disorder.
• Large Platelets: Gray Platelet Syndrome (GPS) is an autosomal recessive with alpha granules deficiency that causes results in thrombocytopenia, mild bleeding tendencies and fibrosis of the marrow.
• Another large platelet is May-Hegglin Anomaly (MHA) it is an autosomal dominant with MYH9 gene mutations and result causes variable, thrombocytopenia, leukopenia, Dohle body-like inclusions in WBC.
• DISORDERS OF PRIMARY HEMOSTASIS

  Vascular Disorders (HE'S HoMeWorKS")

  Hereditary Hemorrhagic Telangiectasia/Osler-Weber-Rendu Disease

  Ehlers-Danios Syndrome/Cutis Hyperelastica

  Marfan's syndrome

  Kasabach-Meritt Syndrome/ Congenital Hemangiomata

  Senile Purpura

  Henoch Schonlein Purpura/Allergic Purpura/ Nonthrombocytopenic purpura

  Waterhouse Friderichsen Syndrome/ Hemorrhagic Adrenalitis

  Scurvy/Ascorbic Acid deficiency
• most common inherited vascular bleeding disorder is Hereditary Hemorrhagic Telangiectasia/Osler-Weber-Rendu Disease
• Localize dilation of capillary walls (skin and mucous membrane) is Hereditary Hemorrhagic Telangiectasia/Osler-Weber-Rendu Disease
• Hyperextensible joints and hyperelastic skin Ehlers-Danios Syndrome/Cutis Hyperelastica
• Skeletal defects (long extremities) and Arachnodactyly (spider fingers) is Ehlers-Danios Syndrome/Cutis Hyperelastica
• Tumors composed of vessels that commonly swell and bleed at the surface is Kasabach-Meritt Syndrome/ Congenital Hemangiomata
• Bruised areas on the forearms of elderly persons and due to degeneration of collagen is Senile Purpura
• Most common seen in children. Vascular abnormality is caused by immunologic damage to endothelial cells. Gastrointestinal hemorrhage and joint swelling is Henoch Schonlein Purpura/Allergic Purpura/ Nonthrombocytopenic purpura
• Severe capillary damage following meningococcal septicemia is Waterhouse Friderichsen Syndrome/ Hemorrhagic Adrenalitis
• Defects in synthesis of collagen and hyaluronic acid is Scurvy/Ascorbic Acid deficiency
• Platelet disorders; QUANTITATIVE PLATELET

  Thrombocytopenia ; Impaired/ Decrease PLT production

  "MT LeVi WAS MY BF"

  Megaloblastic anemia

  TAR syndrome

  Leukemia

  Viral Infections

  WAS

  MYH9 gene mutations

  BSS

  Fanconi anemia
• Platelet disorders; QUANTITATIVE PLATELET

  Thrombocytosis; Reactive/Secondary (Moderate Increase PLT)

  "RAM"

  Recover from splenectomy

  Acute blood loss

  Major surgery
• Platelet disorders: QUALITATIVE

  Adhesion is BSS and vWD
• Platelet disorders: QUALITATIVE

  Aggregations is Glanzmann's thrombasthenia, Hereditary afibrinogenemia
• Platelet disorders: QUALITATIVE

  Secretion is Alpha deficiency, GPS, Dense deficiency (HPS, CHS, TAR, WAS)
• Platelet disorders; QUANTITATIVE PLATELET

  Thrombocytopenia (decrease PLT production)

  Increase PLT destruction

  Increase splenic sequestration

  Massive blood transfusion
• plt rich plasma count of 200,000 to 300,000/uL
• To prepare PRP sodium citrate anticoagulated blood then centrifuge at 50 x g for 30 minutes. The tube stored at 18 to 24 degree celcius
• PRP based light transmittance aggregometry is initiated no less than 30 minutes after spx centrifuged and completed within 4 hours
• PRP original measure 9 to 12 mL of whole blood
• Ristocetin increases the expression of VWF
• measures platelet aggregation and ATP release?

  Platelet Lumi-aggregometry
• when ATP is release in PLT Lumiaggregometry; measured by addition of lucefirin
• bleeding time also called as Duke Method
• bleeding time (duke method) 2-4 mins
• Bleeding time (ivy method) 1-7 mins
• template bleeding time 2-9 mins
• Rumple-Leede test is 1+
• Clot retraction (hirschboeck/Castor oil) is 15-45 mins.
• MacFarlene Method id 44-67%
• In bleeding time (ivy method) inflate sphygmomanometer to 40 mmHg, cleanse are with 70% alcohol wipe dry with sterile gauze. Make 2 incisions (2mm deep and 2 mm long) blot with 2 separate filter paper every 30s and then report the average
• Modification of ivy method by C.H. Mielke Jr
• Uses template containing a standardized slit instead of lancets?

  template bleeding time
• simulate and surgicutt?


  template bleeding time
• uses capillary fragility test/tourniquet test?

  rumple-leede test