Dados do Trabalho

Título

Gemcitabine-induced hemolytic uremic syndrome with an atypical presentation: a Case Report

Apresentação do caso

A 70-year-old woman with Li-Fraumeni syndrome had a diagnosis of metastatic pancreatic adenocarcinoma and leiomyosarcoma. Immediately after infusion of gemcitabine and nab-paclitaxel chemotherapy, she had an acute episode of dyspnea and hypoxemia. Vital signs revealed low oxygen saturation, and respiratory exam showed wheezing. A chest computed tomography angiogram showed a new discrete interstitial infiltration and stable lung nodules. She was hospitalized for two weeks, requiring oxygen support. After initiation of corticosteroids, she had a gradual improvement of the pulmonary symptoms. Despite that, she presented with a worsening of laboratory tests, with rising serum creatinine, anemia, thrombocytopenia, and high lactate dehydrogenase.Diagnosis of a hemolytic uremic syndrome secondary to gemcitabine therapy (GiHUS) was suspected. Despite continuation of corticosteroids for 7 weeks, her renal function continued to deteriorate. Renal biopsy was performed and confirmed thrombotic microangiopathy (TMA). Plasmapheresis was performed with no improvement. She then received eculizumab for 5 weeks which resulted in significant improvement of hematological parameters. Nevertheless, her renal function did not recover, and she evolved with end stage renal disease (ESRD).

Discussão

GiHUS is a very rare pathology that carries a poor prognosis, being potentially fatal. The first signs of the disease are usually anemia and thrombocytopenia, which can mimic the myelotoxicity of gemcitabine, confounding and delaying GiHUS diagnosis. We described here an atypical presentation with exuberant pulmonary symptoms. Only weeks after her initial symptoms, she initiated gradual presentation of the classical signs. There is no consensus on the gold-standard treatment for GiHUS. In addition to discontinuing gemcitabine, treatment with corticosteroids, plasmapheresis, and the C5 inhibitor eculizumab can be considered, with variable response. Nearly half of the cases progress to ESRD. The present case improved hematological parameters after eculizumab, but renal function did not recover in spite of all measures.

Comentários finais

Physicians should maintain high suspicion for GiHUS, since early discontinuation of the drug and initiation of supportive therapies may impact outcomes. In addition to renal dysfunction and blood alterations, other signs and symptoms related to microangiopathy may occur. In this case report, first disease manifestations were pulmonary symptoms

Palavras-chave

chemotherapy, gemcitabine, hemolytic uremic syndrome