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

• Published in: Intensive care medicine Vol. 24; no. 9; pp. 981 - 983
• Main Authors: LASSOUED, K, CLAUVEL, J.-P, SIMILOWSKI, T, AUTRAN, B, BENGOUFA, D, OKSENHENDLER, E
• Format: Journal Article
• Language: English
• Published: Heidelberg Springer 01.09.1998; Berlin Springer Nature B.V
• 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; Case study; Human; Infection; Immunopathology; Lung disease; Concomitant disease; Respiratory disease; Capillary leak syndrome; Lung; Cardiovascular disease; Globulin γ; Immune deficiency
• ISSN: 0342-4642; 1432-1238
• DOI: 10.1007/s001340050700

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.