0521779407-10 CUNY1086/Karliner 0 521 77940 7 June 6, 2007 17:44
Intravascular, Non-immune Hemolytic Anemia 861■Disseminated intravascular coagulation (DIC)
■abnormal consumption of coagulation factors with secondary acti-
vation of fibrinolysis
■sepsis (e.g., gram-negative bacteria, encapsulated gram-positive
bacteria, viral infections), obstetric complications, trauma, neo-
plasms, snakebites, burns, heat stroke, shock, hemolytic transfusion
reactions, severe liver cirrhosis, malignant hypertension, cavernous
hemangiomas
■Thrombotic thrombocytopenic purpura (TTP)
■congenital deficiency or acquired inhibitor to a serum metallopro-
tease responsible for normal cleavage of unusually large multimers
of von Willebrand’s factor; uncleaved large von Willebrand’s multi-
mers lead to agglutination of platelets
■most cases idiopathic
■associated with infection, pregnancy, transplant, AIDS, and drugs
(e.g. mitomycin C, ticlopidine, cyclosporine, and tacrolimus)
■Hemolytic uremic syndrome (HUS)
■primarily a disease of childhood
■may be sporadic or associated with pregnancy (adult), epidemic
(pediatric), or endemic
■onset of adult cases often as gastroenteritis or upper respiratory
tract infection followed a week later by malaise, fatigue and oli-
guria
■pediatric cases typically develop following a febrile illness or infec-
tions with verotoxin-secreting strains of Escherichia coli (strain
O157:H7) or Shigella dysenteriaeSigns & Symptoms
■Disseminated intravascular coagulation (DIC)
■often obscured by clinical features associated with primary initiating
illness
■bleeding, thrombosis, fever, pallor
■Thrombotic thrombocytopenic purpura (TTP)
■pentad: fever, thrombocytopenia, microangiopathic hemolytic ane-
mia, neurologic abnormalities, renal dysfunction
■pallor, purpura, petechiae, mucosal bleeding, jaundice, nau-
sea/vomiting, abdominal pain, arthralgias, myalgias, hep-
atospenomegaly, delirium, seizures, coma, paralysis, aphasia,
visual field defects
■Hemolytic uremic syndrome (HUS)
■triad: microangiopathic hemolytic anemia, thrombocytopenia,
acute renal failure