Abstract
BackgroundSystemic capillary leak syndrome is a rare disease with a high mortality rate. This syndrome is characterised by generalised edema, hypotension, hemoconcentration, and hypoproteinemia. The cause is the sudden onset of capillary hyperpermeability with extravasations of plasma from the intravascular to the extravascular compartment. We present the case of a patient who experienced two episodes of systemic capillary leak syndrome and pulmonary hypertension; the first after gemcitabine in an adjuvant setting and the second three years later after treatment with nab-paclitaxel for metastatic disease.Case presentationA 65-year-old patient underwent a pancreatectomy in January 2010 for ductal carcinoma (pT3 N0 M0, stage IIa), followed by adjuvant chemotherapy. Seven days after the last cycle, she developed dyspnea associated with orthopnea and cough. A transthoracic cardiac ecocolordoppler was performed, with evidence of pulmonary hypertension (58 mmHg). Blood tests showed an increase in creatinine, pro-BNP and D-Dimer. She began high-dose diuretic therapy combined with cortisone. After a month, the patient was eupneic and the anasarca had resolved. We decided gradually to reduce the steroid and diuretic therapy. After ten days of the reduction, the patient began to re-present the same symptoms after treatment with gemcitabine. Corticosteroid therapy was restored with rapid clinical benefit and decreased pro-BNP after a week of treatment. After two years, the disease returned. As a first line treatment, it was decided to use nab-paclitaxel 100 mg/m2 weekly. After two doses, followed by approximately 14 days of treatment, the patient developed acute respiratory distress syndrome. The clinical suspicion was a relapse of capillary leak syndrome and treatment with a high-dose diuretic (furosemide 250 mg daily) was started combined with cortisone (40 mg methylprednisolone). The patient showed a progressive clinical benefit.ConclusionsIn patients treated with gemcitabine and nab-paclitaxel who experience a sudden onset of diffuse edema with respiratory distress, capillary leak syndrome should be suspected. Immediate treatment with corticosteroids may be life-saving.
Highlights
Systemic capillary leak syndrome is a rare disease with a high mortality rate
In patients treated with gemcitabine and nab-paclitaxel who experience a sudden onset of diffuse edema with respiratory distress, capillary leak syndrome should be suspected
Capillary leak syndrome was suspected and the patient was started on high-dose diuretic therapy combined with cortisone (40 mg methylprednisolone)
Summary
Gemcitabine and nab-paclitaxel are generally well-tolerated but on rare occasions they may have particular toxicity. No case of capillary leak syndrome has been reported in literature with nab-paclitaxel. Capillary leak syndrome is a systemic process characterised by the hyperpermeability of the endothelium [10] In this disease there is a rapid extravasation of plasma from the intravascular compartment to the extravascular compartment. A diagnosis of vasculitis was unlikely as the indices of inflammation were negative (VES and PCR); there were high levels of rheumatoid factor and cryoglobulins, and hypocomplementemia was not present This confirms the direct damage caused by the drug and the absence of immune-mediated damage. In patients treated with gemcitabine and nab-paclitaxel, the sudden onset of diffuse oedema with respiratory distress tends to suggest this syndrome. Pulkkanen K, Kataja V, Johansson R: Systemic capillary leak syndrome resulting from gemcitabine treatment in renal cell carcinoma: a case report.
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
