Bleeding Disorders (Platelet & Vascular Endothelium) PDF

BLEEDING DISORDERS (PLATELET & VASCULAR ENDOTHELIUM HML 3003: HEMOSTASIS  Introduction Abnormal bleeding may result from (hemostatic - - disorders): - - 1. Vascular disorders - 2. Platelets disord...

BLEEDING DISORDERS (PLATELET & VASCULAR ENDOTHELIUM HML 3003: HEMOSTASIS  Introduction Abnormal bleeding may result from (hemostatic - - disorders): - - 1. Vascular disorders - 2. Platelets disorders - bleeding from mucus membranes & into skin - 3. Coagulation factors disorders - - bleeding into joints or soft tissues - - 2  The Type Of Haemorrhage - - tissues in bleeding petechiae purpura ecchymoses - haematoma = - - very minute bleeding look like dots on lighter skin tone 3  1) Vascular Disorders can be developed Inherited Acquired old age 1. Hereditary hemorrhagic telangiectasia - - 1. Senile Purpura = => 2. Connective Tissue disorders - - 2. Scurvy - ↓ Vitamin ( [swelling of th gums , frequent colds..... 3. Giant Hemangioma = 3. Steroid purpura - as a result of steroid use -I - to treat allergic reaction and asthma Petechiae in Scurvy Giant Hemangioma Senile Purpura 4  Hereditary Hemorrhagic Telangiectasia Uncommon, autosomal dominant disorder Pathogenesis: Presence of Telangiectasia (dilated superficial ~ i Permanently capillaries) in the skin, mucous membrane em - Telangiactasia maybe with pinpoint, nodular or spiderlike > - appearance = Moreover, it appears during childhood & becomes more - - numerous in adults = - 5  Hemorrhagic Telangiectasia -- = 6  Ehlers Danlos Syndrome Rare connective tissue disorder With abnormal collagen formation. Autosomal dominant trait. This syndrome characterized by : Extreme vascular fragility Skin hyper elasticity Hyper extensible joints very dangerous if bleeding begins 7  Ehlers Danlos Syndrome 8  Marfan’s Syndrome Ocular defect An inherited autosomal dominant associated with abnormal collagen formation Characterized by  Skeletal defect (long extremities)  Ocular defect  Cardiovascular defect 9  T Marfan’s Syndrome 10  Osteogenesis Imperfecta Autosomal dominant Collagen vascular disorder Characterized by brittle bone disease & lack of bone matrix fatal disease people with this , disease die early Poor quality of life 11  Giant Hemangioma A hereditary basis has not been established but the condition is present at birth. Characterized by tumorous masses composed of thin-walled blood vessels commonly found in the skin & subcutaneous tissue 12  Senile Purpura ‘age spots’ Occurs more commonly in elderly men than women. Due to a lack of collagen support for small vessels & loss of subcutaneous fat & elastic fibers, increase with advancing age. Result in characteristic red to purple irregular purpura (age spots) on the extensor surfaces of forearms & hands 13  2) Platelet Disorders Quantitative Plt disorders → (Thrombocytopenia) A) Failure of platelet production B) Increased platelet destruction Qualitative Plt disorders → (Abnormal plt function) A) Hereditary disorders B) Acquired disorders 14  2)Platelet Disorders Thrombocytopenia Defective function (qualitative) A) Failure of platelet production: A) Hereditary disorders: 1. Aplastic BM Can happen as an acquired disease den to benzene exposure 1. Bernad- Soulier Syndrome (↓ GPIb) 2. Megaloblastic anemia 2. Glanzmann`s thrombasthenia (↓GPIIb/IIIa) 3. Leukemia 3. Storage pool diseases: 4. Viral infection (HIV) a)↓ α-granules → Grey platelet syndrome 5. Radio & chemotherapy b)↓ dense granules→ β-storage pool deficiency or admesion no granules ↓ - aggregation B) Increased Plt destruction: unknown B) Acquired disorders: 1. Autoimmune idiopathic 1. Antiplatelet drugs (aspirin) thrombocytopenic purpura 2. Hyperglobulinemia→ Multiple Myeloma 2. Infections (HIV, Malarial) 3. Drug-induced immune thrombocytopenia 15  References Marques, M.,(2015) Quick Guide to Hemostasis, 3rd Ed. American Association for Clinical Chemistry (AACC). 9781594251818 Keohane, E., Smith, L., and Walenga, J. (2019). Rodak’s Hematology: Clinical Principles & Applications (6thed.).Philadelphia: Elsevier Saunders. Kaushansky/ et al, (2016) Williams Hematology, 9th Ed. McGraw-Hill. ISBN 9780071833011 Moore, Gary/ Knight, Gavin (2013) Essential Haematology, Oxford University Press ISBN: 9780191666711. Lewis, S., Bain, B., Bates, I. (2012) Dacie and Lewis, Practical Haematology, 11th Ed., Churchill Livingstone Elsevier. ISBN: 978-0-7020-3407-7. Hoffbrand, A., Moss, P. (2011) Essential Haematology, 6th Ed. Wiley Blackwell. ISBN: 978-1-4051-9890-5. Moss, Paul, Pettit, Hoffbrand. Essential Haematology, 5th Ed. Blackwell Science. 2006. ISBN: 978140513649 16