RESUMO
Introduction: Idiopathic systemic capillary leak syndrome (ISCLS) or Clarkson's disease is unusual but potentially lethal, characterized by recurrent shock incidents and anasarca secondary to idiopathic increase of capillary permeability. In such a context, the use of venoarterial Extracorporeal Membrane Oxygenation (ECMO) as cardiorespiratory support is a rescue action that seeks hemodynamic stability generation until spontaneous disappearance of the capillary occurs with the objective of surpassing the complications of resorption phase. Case Report: A 42 year old patient presented ISCLS and required ECMO as hemodynamic support for 8 days. She remained 20 days in Intensive Care Unit and was given hospital release after 43 days. Conclusions: The use of ECMO in the reported case was a useful strategy in the ISCLS management as a bridge to recovery both in the leak stage and the fluid resorption phase. Notwithstanding its indication is limited to thoroughly selected patients and requires further debate between specialists about its risks and benefits.
Introducción: El Síndrome de Leak Capilar Idiopático (SLCI) o enfermedad de Clarkson es un entidad rara pero potencialmente letal, caracterizada por episodios recurrentes de shock y anasarca secundarios al aumento idiopático de la permeabilidad capilar. En este contexto, el uso de membrana de oxigenación extracorpórea (ECMO) venoarterial como soporte cardiorrespiratorio, es una medida de rescate que busca generar estabilidad hemodinámica a la espera de la desaparición espontánea del leak capilar con el fin de sortear las complicaciones de la fase de reabsorción. Caso Clínico: Se presenta el caso de un paciente un paciente de 42 años de edad que presentó SLCI y requirió ECMO durante 8 días como soporte hemodinámico. Cursó 20 días de internación en la unidad de terapia intensiva y se otorgó el alta hospitalaria luego de 43 días. Conclusiones: El uso de ECMO en el caso reportado significó una estrategia útil para el manejo del SLCI como puente a la recuperación, tanto en la etapa de leak como en la fase de reabsorción de fluidos. Sin embargo, su indicación se ve limitada a pacientes altamente seleccionados, y todavía requiere un mayor debate entre especialistas sobre los posibles riesgos y beneficios.
Assuntos
Síndrome de Vazamento Capilar , Oxigenação por Membrana Extracorpórea , Adulto , Síndrome de Vazamento Capilar/terapia , Feminino , Humanos , Unidades de Terapia IntensivaRESUMO
We report a case of capillary leak that developed during treatment of antibody-mediated rejection in a kidney transplant recipient. A 53-year-old female transplant recipient experienced an increase in serum creatinine from 1.1 to 1.8 mg/dL. Antibody-mediated rejection was diagnosed by graft biopsy. She was treated with five plasmapheresis sessions (on alternate days with albumin replacement), five doses of immunoglobulin (5 g/dose at 100 mg/kg), a single dose of rituximab (500 mg), and four doses of bortezomib on days 1, 4, 7, and 10 (1.72 mg/dose at 1.3 mg/m2 body surface area). During treatment, edema, slight diarrhea, pancytopenia, hypoalbuminemia, peripheral neuropathy, and postural hypotension were noted. Despite control of liquids, she presented with edema progressing to an increase of more than 10 kg body weight. Prerenal acute graft dysfunction associated with hypotension was diagnosed on day 12, heart failure or other infectious complications being discounted. On day 13, daily hemodialysis was prescribed, and a stable volume status was reached after five hemodialysis sessions. On day 20, the patient recovered diuresis and the edema and diarrhea abated, but she remained on chronic hemodialysis. After excluding other causes of distributive shock, the diagnosis of capillary leak syndrome was based on the presence of hypotension, generalized edema, and hypoalbuminemia in the absence of significant proteinuria. The concomitant presence of diarrhea, peripheral neuropathy, and pancytopenia, suggest a possible causal role for bortezomib. Awareness by clinicians of capillary leak syndrome associated with bortezomib-based treatment of AMR is paramount, despite its rarity.