Consider systemic capillary leak syndrome in monoclonal gammopathy with shock

INTRODUCTION AND IMPORTANCE: The cappilary leacking syndrome is a very rare disease that can be idiopathic (clarkson syndrome) or secondary to other pathologys. CASE PRESENTATION: We report a case of 37-year-old women who was admitted in the emergency room for a hemodynamic shock of neither cardiac nor septic cause, and the patient wasn't presenting any bleeding. The investigations showed that the diagnosis was a Clarkson syndrome crisis and the patient was having supportive treatment containing fluid therapy, vasoactive drugs, and ECMO. And died after 48h of hospitalization. CLINICAL DISCUSSION: the cappilary leacking syndrome is a very fatal affection, its physiopathologis remains unknown. It evoluate by crisis made by hypotension and anasarca, in severe cases it is presented as fatal hypovolemic schock. Biological investigations show hemoconcentration associated with hypoalbunemia which is pathognomonic of the disease. The treatment is essentially based on crisis treatment support by fluid therapy, vasoactives drugs, some practicien report the use of theophilyn for prevention but without any proven efficiency. CONCLUSION: For all this reasons we are in the obligation of investing in fundamental studies to better understand this fatal disease.