P1: RLJ/OZN P2: KUF
0521779407-D-01 CUNY1086/Karliner 0 521 77940 7 June 13, 2007 7:41
Disseminated Intravascular Coagulation 491■Pregnancy (distinguish from HELLP syndrome=hemolysis, ele-
vated liver function tests and low platelets)
■Family history: Autosomal dominant and recessive
■Many cases without obvious inciting cause (idiopathic)Signs & Symptoms
■DIC/TTP/HUS may present with mucosal bleeding, petechiae,
and/or purpura.
■DIC manifests with bleeding in the setting of abnormal laboratory
parameters involving coagulation factors and platelets; signs and
symptoms may be suggestive of an underlying disorder.
■DIC more commonly presents with hemorrhage, but can also present
with thrombotic complications such as embolic phenomena – pur-
puric lesions may be manifest on the digits.
■TTP/HUS is of rapid onset, although the disease may become chronic
in nature; DIC may be either acute or chronic.
■TTP pentad consists of fever, neurologic manifestations, microan-
giopathy, thrombocytopenia and renal failure.
■All five features need not be present in order to make the diagnosis.
■HUS may present with symptoms of a diarrheal illness.Distinction of TTP from HUS
■TTP: Fever and neurologic manifestations
■HUS: Renal insufficiency prominent
➣Clinical distinction is often difficult or impossible – may simply
need to make diagnosis as TTP/HUS.tests
■When suspected, diagnosis of TTP/HUS should be expedited and
can be based on historical features and basic laboratory studies.
■TTP/HUS is distinguished from DIC as microangiopathic hemolytic
anemia with normal coagulation parameters.Basic Tests
■CBC, including platelet count: define degree of anemia, thrombocy-
topenia
■Review of peripheral smear
➣Schistocytes indicative of microangiopathy: should see at least a
few per high-power field
➣Presence of toxic granulation, vacuoles, or D ̈ohle bodies (light
bluish cytoplasmic inclusions) in neutrophils suggests infection
potentially associated with DIC.