Atypical Evolution of Secondary Hemolytic Uremic Syndrome Defined as Paraneoplastic Syndrome under Eculizumab and Palbociclib Therapies

Abstract

Thrombotic microangiopathy (TMA) is most of the time caused by thrombotic thrombocytopenic purpura or hemolytic uremic syndrome. A 60-year-old female was diagnosed in 2014 with mammary breast adenocarcinoma treated by several-line therapy: mastectomy, docetaxel, cyclophosphamide, radiotherapy, doxorubicine, and capecitabine. By mid-November, the patient was admitted to the hospital with regenerative, mechanical, and hemolytic anemia, schistocytes at 3%, and thrombopenia (99 G/L), associated with high blood transfusion requirement. After 9 sessions of plasmapheresis, there was no significant improvement in the biological parameters, nor after 2 cycles of paclitaxel. The patient was then treated with eculizumab during 4 weeks, with a slight reduction in blood requirement, and simultaneously with palbociclib. Since being treated with palpociclib, she had a great reduction in blood requirement and a good clinical condition. To conclude, we reported an initial moderate improvement of paraneoplasm-related TMA syndrome under eculizumab therapy with a slight reduction in red blood cell requirement; however, palbociclib therapy achieved a very good response with a dramatic reduction in red blood cell requirement.

Gemcitabine-induced thrombotic microangiopathy treated with eculizumab: a case report

“…This could also explain why treatment with eculizumab is potentially successful in this setting. Apart from drug-induced TMA through direct endothelial damage (type 1, dose dependent) or immune-mediated through the development of drug-dependent autoantibodies (type 2, none-dose dependent), the malignancy itself can also cause TMA (paraneoplastic TMA) ( 19 ). This can arise from metastases in the microvasculature or direct invasion of tumor cells in the bone marrow ( 2 ).…”

“…It was only after discontinuation of chemotherapy and during eculizumab treatment that the patient showed disease progression. Furthermore, paraneoplastic TMA usually does not respond well to eculizumab and would more likely improve when there is a good response to chemotherapy ( 19 ). Also, paraneoplastic TMA is most frequently seen in metastatic disease (91.8%), which was not the case in our patient ( 20 ).…”