Bleeding disorders Doc. MUDr. L. Boudová, Ph.D.. Bleeding disorders I. Vessels - increased...
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Transcript of Bleeding disorders Doc. MUDr. L. Boudová, Ph.D.. Bleeding disorders I. Vessels - increased...
Bleeding disordersI. Vessels - increased fragilityII. Platelets - deficiency or dysfunctionIII.Coagulation disordersIV.Combination I-III
• I. Vessels • infections, drugs (hypersensitivity) • poor vascular wall: Ehlers-Danlos, scurvy;
Cushing syndrome, old people• hereditary hemorrhagic teleangiectasia• amyloid
Thrombocytes150-300 000/mm3 normal
100 000/mm3 thrombocytopenia
50 000/mm3 posttraumatic bleeding
20 000/mm3 spontaneous bleeding
Splenomegaly
a sign of an underlying diseasea problem per se
bigger spleen~ bigger function = destruction of blood cellsHypersplenism1. splenomegaly2. (pan) cytopenia3. correction of blood cytopenia by
splenectomy
Immune thrombocytopenic purpura
Idiopathic TP – but autoimmune!
Antibodies against platelets
Primary or secondary
ITP• Acute – children
• Abrupt onset, both sexes, 2 weeks after virosis, resolves within 6 mo, 20% chronic
• Chronic – adults (F:M 3:1)
• Petechial hemorrhages and ecchymoses
• Easy bruising, skin, mucosa, nose, gums, soft tis., melena, hematuria, menstruation
• Clin.: nonspecific – reflective of thrombocytopenia
ITP - organs
• Spleen - normal size
• PB: megathrombocytes
• BM:+ /- more megs
• Therapy: splenectomy
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
Thrombotic microangiopathiesthrombotic thrombocytopenic purpura (TTP)hemolytic-uremic syndrome (HUS)
VersusDisseminated intravascular coagulation
Common: hyaline thrombi
!!Differences: DIC: primary importance:
activation of clotting system
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
Thrombotic microangiopathies
TTP
neurological deficits (transient)
renal failureadult women
HUS
mostly no neurol. sympt.acute renal failure DOMINANT!children; E. coli O157:H7, verotoxin
Common: thrombocytopenia, microangiopathic hemolytic anemia, fever
Thrombotic microangiopathiesthrombotic thrombocytopenic purpura (TTP)hemolytic-uremic syndrome (HUS)
VersusDisseminated intravascular coagulation
Common: hyaline thrombi
!!Differences: DIC: primary importance:
activation of clotting system
secondary complication
of some serious condition consumption coagulopathy
thrombohemorrhagic diathesis
acute, subacute, chronic
Disseminated intravascular coagulation (DIC)
DIC: thrombotic and hemorrhagic diathesis
Microthrombi hypoxia, infarctions
Consumption →depletion of platelets and clotting factors
+ secondary activation of fibrinolysis
hemorrhages
activation of coagulation sequence
Mechanisms of DIC trigger
1. Release of tissue factor or thromboplastic substances
2. Widespread endothelial injury
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.
DIC
• Morphology microthrombi
• Kidneys hemorrhages
Microinfarcts, bilat. renal necrosis
• lungs
• Brain - microinfarcts, hemorrhage
• Placenta
• Spleen , liver
• Endocrine organs: pituitary, adrenals
DIC - clinical
• microangiopathic hemolytic anemia, RDS – dyspnea, cyanosis
• neurologic symptoms, oliguria, acute renal and circulatory failure,
• Convulsions, coma
• SHOCK