Pulmonary infections associated with systemic capillary leak syndrome attacks in a patient with hypogammaglobulinemia

Systemic capillary leak syndrome (SCLS) is a rare disorder of unknown etiology, characterized by recurrent hypovolemic shock attacks associated in most cases with a serum monoclonal immunoglobulin. Prophylactic therapy is usually disappointing and the outcome is often fatal. We report on a patient w...

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Bibliographic Details
Published in:Intensive care medicine 1998-09, Vol.24 (9), p.981-983
Main Authors: LASSOUED, K, CLAUVEL, J.-P, SIMILOWSKI, T, AUTRAN, B, BENGOUFA, D, OKSENHENDLER, E
Format: Article
Language:English
Subjects:
Adult
Agammaglobulinemia - complications
Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy
Biological and medical sciences
Bronchoalveolar Lavage Fluid - microbiology
Capillary Leak Syndrome - diagnosis
Capillary Leak Syndrome - etiology
Cell proliferation
Diagnosis, Differential
Emergency and intensive cardiocirculatory care. Cardiogenic shock. Coronary intensive care
Etiology
Humans
Hypogammaglobulinemia
Infections
Intensive care
Intensive care medicine
Lymphocytes B
Male
Medical sciences
Mycoplasma pneumoniae - isolation & purification
Parainfluenza Virus 3, Human - isolation & purification
Pneumonia - complications
Pneumonia - microbiology
Pneumonia, Mycoplasma - complications
Pneumonia, Viral - complications
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Summary:Systemic capillary leak syndrome (SCLS) is a rare disorder of unknown etiology, characterized by recurrent hypovolemic shock attacks associated in most cases with a serum monoclonal immunoglobulin. Prophylactic therapy is usually disappointing and the outcome is often fatal. We report on a patient with recurrent hypovolemic shocks consistent with the diagnosis of SCLS associated with severe serum panhypogammaglobulinemia but no detectable monoclonal immunoglobulin or B cell proliferation. Attacks were often preceded by severe respiratory infections. Both infections and attacks were successfully prevented by i.v. gammaglobulin replacement. Further evaluation is needed to assess the efficacy of i.v. gammaglobulins in patients with SCLS but without hypogammaglobulinemia.
ISSN:0342-4642
1432-1238
DOI:10.1007/s001340050700