2021, Number 4
Rev Hematol Mex 2021; 22 (4)
Neurological involvement as manifestation of thrombotic thrombocytopenic purpura
Pérez-Corrales LL, Zaragoza-Sandoval VM, López-Martínez DA
Full text
Language: Spanish
References: 5
Page: 203-206
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ABSTRACT
Background: Thrombotic thrombocytopenic purpura is a rare thrombotic microangiopathy with annual prevalence of 10 cases/million people and annual incidence of one new case/million people. It is defined by a severe deficiency in ADAMTS13. Rapid recognition of its etiology is decisive for the correct treatment of patients, since it is associated with a misdiagnosis in about 30% of cases. Likewise, the use of clinical scores in conjunction with the measurement of ADAMTS13 activity is crucial to confirm or exclude the diagnosis of thrombotic thrombocytopenic purpura. The historical pentad of fever, thrombocytopenia, microangiopathic hemolytic anemia, neurological symptoms, and renal failure that used to define thrombotic thrombocytopenic purpura seems outdated, since several studies have shown that these five symptoms were present in less than 10% of patients with an acute illness. Plasmapheresis remains the cornerstone of current thrombotic thrombocytopenic purpura treatment.Clinical case: A 47-year-old male patient with no significant medical history with a clinical picture suggestive of thrombotic thrombocytopenic purpura.
Conclusions: In an emergency setting, the diagnosis of thrombotic thrombocytopenic purpura is a challenge because the disease is rare, in addition, it needs an early differential diagnosis to initiate appropriate treatment in a timely manner, otherwise it may lead to a delay in treatment, which can affect the prognosis of the patient.
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