Bleeding disorders

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Bleeding disorders

• Doc. MUDr. L. Boudová, Ph.D.

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Bleeding disordersI. Vessels - increased
fragilityII. Platelets - deficiency or
dysfunctionIII.Coagulation disordersIV.Combinati
on I-III

• I. Vessels
• infections, drugs (hypersensitivity)
• poor vascular wall Ehlers-Danlos, scurvy
  Cushing syndrome, old people
• hereditary hemorrhagic teleangiectasia
• amyloid

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Thrombocytes

• 150-300 000/mm3 normal
• 100 000/mm3 thrombocytopenia
• 50 000/mm3 posttraumatic bleeding
• 20 000/mm3 spontaneous bleeding

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Thrombocytopenia

• decreased production
• increased destruction
• sequestration
• dilution

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Splenomegaly

• a sign of an underlying disease
• a problem per se
• bigger spleen bigger function
• destruction of blood cells
• Hypersplenism
• splenomegaly
• (pan) cytopenia
• correction of blood cytopenia by splenectomy

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Immune thrombocytopenic purpura

• Idiopathic TP but autoimmune!
• Antibodies against platelets
• Primary or secondary

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ITP

• Acute children
• Abrupt onset, both sexes, 2 weeks after virosis,
  resolves within 6 mo, 20 chronic
• Chronic adults (FM 31)
• Petechial hemorrhages and ecchymoses
• Easy bruising, skin, mucosa, nose, gums, soft
  tis., melena, hematuria, menstruation
• Clin. nonspecific reflective of
  thrombocytopenia

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ITP - organs

• Spleen - normal size
• PB megathrombocytes
• BM /- more megs
• Therapy splenectomy

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Thrombotic microangiopathiesrelated clinical
syndromesthrombotic thrombocytopenic purpura
(TTP)hemolytic-uremic syndrome
(HUS)ENDOTHELIAL INJURY WIDESPREAD HYALINE
MICROTHROMBI

• OVERLAP - common features (TTP, HUS)
• thrombocytopenia
• microangiopathic hemolytic anemia
• fever

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Thrombotic microangiopathiesthrombotic
thrombocytopenic purpura (TTP)hemolytic-uremic
syndrome (HUS)

• Versus
• Disseminated intravascular coagulation
• Common hyaline thrombi
• !!Differences DIC primary importance
• activation of clotting system

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Thrombotic microangiopathiesrelated clinical
syndromesthrombotic thrombocytopenic purpura
(TTP)hemolytic-uremic syndrome
(HUS)ENDOTHELIAL INJURY WIDESPREAD HYALINE
MICROTHROMBI

• OVERLAP - common features (TTP, HUS)
• thrombocytopenia
• microangiopathic hemolytic anemia
• fever

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Thrombotic microangiopathies

• TTP
• neurological deficits (transient)
• renal failure
• adult women

• HUS
• mostly no neurol. sympt.
• acute renal failure DOMINANT!
• children E. coli O157H7, verotoxin

Common thrombocytopenia, microangiopathic
hemolytic anemia, fever
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Microangiopathic hemolytic anemia
hemolytic-uremic syndrome several fragmented red
cells
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Thrombotic microangiopathiesthrombotic
thrombocytopenic purpura (TTP)hemolytic-uremic
syndrome (HUS)

• Versus
• Disseminated intravascular coagulation
• Common hyaline thrombi
• !!Differences DIC primary importance
• activation of clotting system

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Disseminated intravascular coagulation (DIC)

• secondary complication
• of some serious condition
• consumption coagulopathy
• thrombohemorrhagic diathesis
• acute, subacute, chronic

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DIC thrombotic and hemorrhagic diathesis
activation of coagulation sequence

• Microthrombi hypoxia, infarctions
• Consumption ?depletion of platelets and clotting
  factors
• secondary activation of fibrinolysis

hemorrhages
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Mechanisms of DIC trigger1. Release of tissue
factor or thromboplastic substances2.
Widespread endothelial injury
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DIC

• 1. obstetrics 50 abruptio placentae, retained
  dead fetus, septic abortion, amniotic fluid
  embolism, toxemia
• 2. neoplasms 30 adenocarcinomas, AML
• 3. infections gram-negative sepsis
• 4. trauma, burns, extensive surgery
• 5. Other snakebite, heat stroke, giant
  hemangioma, aortic aneurysm etc.

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DIC

• Morphology microthrombi
• Kidneys hemorrhages
• Microinfarcts, bilat. renal necrosis
• lungs
• Brain - microinfarcts, hemorrhage
• Placenta
• Spleen , liver
• Endocrine organs pituitary, adrenals

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DIC - clinical

• microangiopathic hemolytic anemia, RDS dyspnea,
  cyanosis
• neurologic symptoms, oliguria, acute renal and
  circulatory failure,
• Convulsions, coma
• SHOCK

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Disseminated intravascular coagulation
hyaline thrombi
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Sy Waterhouse - Friedrichsen