Contributions
Abstract: EP1788
Type: ePoster
Abstract Category: Therapy - disease modifying - 32 Others
Background: Capillary leak syndrome (CLS) is a rare condition characterized by unexplained episodic capillary hyperpermeability due to a shift of fluid and protein from the intravascular to the interstitial space with subsequent hypovolemia, hemoconcentration and hypoproteinemia. It can be idiopathic or secondary to some conditions like infection, malignant disease and some drugs like monoclonal antibodies.
Objectives: To report a 61 year-old woman diagnosed with NMO three years before and treated with rituximab (RTX), who suffered a fatal CLS one month after the last drug dose. To our knowledge, it is the first reported case of CLS in NMO patient after rituximab treatment.
Methods: 61-year-old woman with Neuromyelitis optica (NMO) diagnosis treated with rituximab was referred to our hospital with severe hypovolemic shock and anasarca. Laboratory data showed a progressive increase in haematocrit (max 56.2%), haemoglobin (max 18.6 g/dl) and white blood cell count with a normal fraction. Total serum protein showed a marked decrease (1.6 g/dl) without proteinuria. She developed a multiple organ failure and died three hours later.
Results: We diagnosed the patient as having capillary leak syndrome (CLS) because of the hypovolemic shock with hypoproteinemia without proteinuria and the lack of suggestive data of infective disease or anaphylaxis. The autopsy showed only findings of diffuse oedema with ascites and pericardial effusion, without evidence of infection or malignant disease, compatible with CLS.
Conclusions: RTX is currently considered to be an effective and safe treatment for NMO. Although the most frequent adverse events are postinfusion reactions and infections, the increasingly widespread and prolonged use of the drug could lead us to less known and potentially more serious adverse effects. Even this is the first CLS case in NMO patient treated with RTX, it is important to consider the diagnosis in any patient who presents sepsis syndrome, in particular, when no clear infectious process can be identified. Increased awareness of this problem will lead to a higher identification of cases and the better knowledge of monoclonal antibodies.
Disclosure:
Rocio Hernandez Clares:nothing to disclose
Ignacio Fuentes Fernandez: nothing to disclose
Ester Carreon Guarnizo:nothing to disclose
Ana E. Baidez Guerrero: nothing to disclose
Gabriel Valero Lopez: nothing to disclose
Adelaida Leon Hernandez: nothing to disclose
Francisca Velazquez Marin:nothing to disclose
Joaquin Zamarro Parra: nothing to disclose
Jose Meca Lallana:nothing to disclose
Abstract: EP1788
Type: ePoster
Abstract Category: Therapy - disease modifying - 32 Others
Background: Capillary leak syndrome (CLS) is a rare condition characterized by unexplained episodic capillary hyperpermeability due to a shift of fluid and protein from the intravascular to the interstitial space with subsequent hypovolemia, hemoconcentration and hypoproteinemia. It can be idiopathic or secondary to some conditions like infection, malignant disease and some drugs like monoclonal antibodies.
Objectives: To report a 61 year-old woman diagnosed with NMO three years before and treated with rituximab (RTX), who suffered a fatal CLS one month after the last drug dose. To our knowledge, it is the first reported case of CLS in NMO patient after rituximab treatment.
Methods: 61-year-old woman with Neuromyelitis optica (NMO) diagnosis treated with rituximab was referred to our hospital with severe hypovolemic shock and anasarca. Laboratory data showed a progressive increase in haematocrit (max 56.2%), haemoglobin (max 18.6 g/dl) and white blood cell count with a normal fraction. Total serum protein showed a marked decrease (1.6 g/dl) without proteinuria. She developed a multiple organ failure and died three hours later.
Results: We diagnosed the patient as having capillary leak syndrome (CLS) because of the hypovolemic shock with hypoproteinemia without proteinuria and the lack of suggestive data of infective disease or anaphylaxis. The autopsy showed only findings of diffuse oedema with ascites and pericardial effusion, without evidence of infection or malignant disease, compatible with CLS.
Conclusions: RTX is currently considered to be an effective and safe treatment for NMO. Although the most frequent adverse events are postinfusion reactions and infections, the increasingly widespread and prolonged use of the drug could lead us to less known and potentially more serious adverse effects. Even this is the first CLS case in NMO patient treated with RTX, it is important to consider the diagnosis in any patient who presents sepsis syndrome, in particular, when no clear infectious process can be identified. Increased awareness of this problem will lead to a higher identification of cases and the better knowledge of monoclonal antibodies.
Disclosure:
Rocio Hernandez Clares:nothing to disclose
Ignacio Fuentes Fernandez: nothing to disclose
Ester Carreon Guarnizo:nothing to disclose
Ana E. Baidez Guerrero: nothing to disclose
Gabriel Valero Lopez: nothing to disclose
Adelaida Leon Hernandez: nothing to disclose
Francisca Velazquez Marin:nothing to disclose
Joaquin Zamarro Parra: nothing to disclose
Jose Meca Lallana:nothing to disclose