Hemorrhagic DiathesisCauses:Increased fragility of vesselsPlatelet deficiency or dysfunctionDerangement of coagulation
Vessel Wall AbnormalitiesCommon but bleeding is LESS SeriousNormal Coagulation test1. Microbial damage to Microvasculature or DIC2. Scurvy – Impaired formation of collagen needed for vessel wall support
3. Drugs – immune complex deposits in vessel wall4. Hemoch-Scheonleinpurpura –  	unknown systemic hypersensitivity 	disease	involve vessels throughout the 	body & glomerularmesangium5. Amyloid Infiltration of blood vessels –	weakens the wall Vessel Wall Abnormalities
R/T THROMBOCYTOPENIADecrease productionAplastic anemiaLeukemiasMegalobalstic anemiaDecrease platelet survivalImmune – mediatedNon-immune – mechanical
Sequestration – seen in marked splenomegalyDilutional – blood stored > 24hours has less viable plateletsR/T THROMBOCYTOPENIA
Idiopathic Immune Thrombocytopenic Purpura ( Autoimmune )1. AcuteSelf-limited HgicdiseaseResolve w/in 6mos.Children after Viral infxn 2 week later  Sudden petechiae, purpuraRisk for cerebral bleedSevere cases Tx w/ corticosteroids
2. ChronicAdults women < 40y/oAssociated withCollagen Vascular DiseaseCLLHIVRepeated episodes of BleedingTx Steroids. IgG, Splenectomy in severe casesIdiopathic Immune Thrombocytopenic Purpura ( Autoimmune )
Deposition of Ig fragments in Amyloidosis
Thrombotic MicroangipathiesI.  Thrombotic Thrombocytopenic Purpura	1. Fever	2. Thrombocytopenia	3. Microangioapthic hemolytic anemia	4. Transient Neurological damage	5. Renal FailureDeficiency of ADAMTS 13 enzymeResults to accumulation of VHMW  vWF promote widesopread platelet Microaggregation
II. Hemolytic –Uremic Syndrome	1. MicroangiopathicHemolytic anemia	2. Thrombocytopenia	3. Prominence of renal failure	4. No Neurological damageHx of Enteric infection E. coliRelease of Shiga –like toxin  Absorbed in GIT  Binds and Damage endothelial cells in Glomerulus & other sites  Platelet Activation & AggregationThrombotic Microangipathies
Widespread formation of hyaline thrombi  in microcircualtionPlatelet consumption & Intravascular thrombiMicroangiopathic hemolytic anemiaMultiple Organ failureActivation of coagualtion cascades is NOT OF PRIMARY IMPORTANCEPT/PTT – usually NormalThrombotic Microangipathies
Plasmin Act.Proteolysis of clotting facotrs
Bleeding

Bleeding

  • 1.
    Hemorrhagic DiathesisCauses:Increased fragilityof vesselsPlatelet deficiency or dysfunctionDerangement of coagulation
  • 2.
    Vessel Wall AbnormalitiesCommonbut bleeding is LESS SeriousNormal Coagulation test1. Microbial damage to Microvasculature or DIC2. Scurvy – Impaired formation of collagen needed for vessel wall support
  • 3.
    3. Drugs –immune complex deposits in vessel wall4. Hemoch-Scheonleinpurpura – unknown systemic hypersensitivity disease involve vessels throughout the body & glomerularmesangium5. Amyloid Infiltration of blood vessels – weakens the wall Vessel Wall Abnormalities
  • 4.
    R/T THROMBOCYTOPENIADecrease productionAplasticanemiaLeukemiasMegalobalstic anemiaDecrease platelet survivalImmune – mediatedNon-immune – mechanical
  • 5.
    Sequestration – seenin marked splenomegalyDilutional – blood stored > 24hours has less viable plateletsR/T THROMBOCYTOPENIA
  • 6.
    Idiopathic Immune ThrombocytopenicPurpura ( Autoimmune )1. AcuteSelf-limited HgicdiseaseResolve w/in 6mos.Children after Viral infxn 2 week later  Sudden petechiae, purpuraRisk for cerebral bleedSevere cases Tx w/ corticosteroids
  • 7.
    2. ChronicAdults women< 40y/oAssociated withCollagen Vascular DiseaseCLLHIVRepeated episodes of BleedingTx Steroids. IgG, Splenectomy in severe casesIdiopathic Immune Thrombocytopenic Purpura ( Autoimmune )
  • 9.
    Deposition of Igfragments in Amyloidosis
  • 13.
    Thrombotic MicroangipathiesI. Thrombotic Thrombocytopenic Purpura 1. Fever 2. Thrombocytopenia 3. Microangioapthic hemolytic anemia 4. Transient Neurological damage 5. Renal FailureDeficiency of ADAMTS 13 enzymeResults to accumulation of VHMW vWF promote widesopread platelet Microaggregation
  • 14.
    II. Hemolytic –UremicSyndrome 1. MicroangiopathicHemolytic anemia 2. Thrombocytopenia 3. Prominence of renal failure 4. No Neurological damageHx of Enteric infection E. coliRelease of Shiga –like toxin  Absorbed in GIT  Binds and Damage endothelial cells in Glomerulus & other sites  Platelet Activation & AggregationThrombotic Microangipathies
  • 15.
    Widespread formation ofhyaline thrombi in microcircualtionPlatelet consumption & Intravascular thrombiMicroangiopathic hemolytic anemiaMultiple Organ failureActivation of coagualtion cascades is NOT OF PRIMARY IMPORTANCEPT/PTT – usually NormalThrombotic Microangipathies
  • 16.