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COMMENT
The Guideline panel discussed a number of additional considerations to
support good clinical care of patients with TTP. These "GPSs" are not
evidence-based recommendations. Specifically, they are not based on
systematic search or formal review of the evidence. These statements
are intended to provide a "snapshot" of the care provided to patients
with TTP by expert health care providers. They address scenarios in
which there is very limited high certainty evidence, yet there is a body
of indirect evidence and/or clinical experience that suggests net benefit
of certain actions. GPSs are intended to guide health care providers
who have limited experience in treating TTP. They should be used with
caution; the provider's own clinical judgement, in combination with the
individual patient's clinical situation, values, and preferences, should
be considered.
SECTION I. Supportive Care
➤ The following statements pertain to initial emergency care and
general supportive care of patients with TTP.
Statement 1
➤ Clinicians generally consider a diagnosis of TTP in individuals
presenting with thrombocytopenia and microangiopathic hemolytic
anemia. Patients may be critically ill, or may have relatively minor
and nonspecific complaints. Importantly, the classic signs of
TTP (e.g., hemolytic anemia, thrombocytopenia, fever, neurologic
abnormalities, renal abnormalities; also known as "Raynaud's
Pentad" are present in only 40% of patients; therefore, the historical
pentad only reflects the more severe forms of the disease, or patients
left without appropriate treatment because of a delayed diagnosis.
The prevalence of this catastrophic presentation decreased in
last decades as a result of better awareness of the disease among
practitioners. In this way, the presence of thrombocytopenia and
microangiopathic hemolytic anemia without other explanation should
prompt a suspicion of TTP in the differential diagnosis.
