Catastrophic antiphospholipid syndrome: Lessons from the "CAPS Registry".
Review published in Medicina clinica (2024)
Abstract
The catastrophic antiphospholipid syndrome (CAPS) is a rare life-threatening clinical condition that represents the most severe clinical presentation of the antiphospholipid syndrome (APS). It was first described in 1992 in a group of patients that presented with multiorgan involvement and microangiopathic features of APS. Most of the current knowledge of CAPS comes from the analysis of all cases collected at the "CAPS Registry" that was created in 2000 to perform studies on this condition. Most cases are triggered by a prothrombotic situation that leads to a multiorgan thrombosis and a cytokine storm. The analysis of cases included in the "CAPS Registry" has shown that the triple therapy with anticoagulation, glucocorticoids, and plasma exchange and/or intravenous immunoglobulins is associated to a better prognosis of CAPS. The improvement of the knowledge allowed a decrease from the 50% mortality rate reported in the first series to 25-30% in the most recent publications.
Abstract sourced from PubMed (NCBI) for the cited record. See the original publication for the authoritative version.
Resumen
The catastrophic antiphospholipid syndrome (CAPS) is a rare life-threatening clinical condition that represents the most severe clinical presentation of the antiphospholipid syndrome (APS). It was first described in 1992 in a group of patients that presented with multiorgan involvement and...
Por qué esto importa para la hirudoterapia
Esta revisión se basa en el Registro internacional CAPS para resumir el conocimiento sobre el síndrome antifosfolípido catastrófico, señalando que la triple terapia con anticoagulación, glucocorticoides y recambio plasmático y/o inmunoglobulinas intravenosas mejora el pronóstico, reduciendo la mortalidad de aproximadamente un 50% a un 25–30%. Es relevante para el dominio de ASH únicamente en el contexto amplio de la anticoagulación como parte del régimen terapéutico de la enfermedad trombótica. El resumen no menciona sanguijuelas, hirudoterapia ni agentes basados en la hirudina. Su relevancia es indirecta.
Citación
Catastrophic antiphospholipid syndrome: Lessons from the "CAPS Registry".
Rodriguez-Pintó et al. · Medicina clinica, 2024
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Añadido a la biblioteca ASH: May 28, 2026 · Última actualización del sitio: June 18, 2026