The terminal complement-inhibitor eculizumab has dramatically changed the management of patients with atypical hemolytic uremic syndrome (aHUS), and has also shown promise for treating certain forms of secondary HUS (sHUS), including that caused by drugs and solid-organ/hematopoietic stem cell transplant. While effective, eculizumab is costly and inconvenient. In this review, we evaluate the literature on eculizumab cessation in these diseases to better inform clinicians who consider stopping therapy. Reported relapse rates in aHUS after stopping eculizumab are as high as 30%, suggesting indefinite therapy is reasonable and that patients who choose to stop should be closely monitored. In sHUS, relapse is rare, justifying short courses of eculizumab.
Objective Evans syndrome, the combination of immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA) or autoimmune neutropenia, is associated with a high rate of relapsed/refractory disease. There are limited data on the efficacy of splenectomy for this condition. We reviewed patient outcomes after splenectomy for Evans syndrome compared to ITP at our institution. Methods We performed a retrospective analysis of patients who underwent splenectomy for autoimmune cytopenias over a 23‐year period with the intention of comparing disease relapse rates after splenectomy in patients with Evans syndrome and in those with ITP. Results During the study period, 77 patients underwent splenectomy for ITP and seven underwent splenectomy for Evans syndrome. In the Evans cohort, splenectomy led to an 85.7% initial response rate with a 42.8% rate of relapse within one year and a long‐term (one‐year) response rate of 42.8%. In the ITP cohort, the initial response rate was 90.9% with a long‐term response rate of 70.1%. Conclusion Our data suggest that long‐term remission rates after splenectomy are lower in adults with Evans syndrome compared to those with ITP, although splenectomy may still be an acceptable treatment for certain patients with Evans syndrome. Our findings underscore the need for further research and development of additional therapeutic strategies for this patient population.
Background: Only 32 cases of prostate cancer with peritoneal carcinomatosis and ascites have currently been reported in the literature. We present the first reported case of prostate cancer with peritoneal carcinomatosis and malignant ascites whose treatment was complicated by tumor lysis syndrome along with a literature review evaluating similar cases. Case: We present a rare case of a 78-year-old retired Caucasian male with recurrent metastatic prostate cancer and malignant ascites. He had previously received definitive radiotherapy for localized prostate cancer but presented with bilateral hydronephrosis and right-sided bladder wall thickening 10 years later. He had imaging evidence of locoregional recurrence with elevated prostate specific antigen (PSA) and was initiated on combined androgen blockade with intramuscular leuprolide and oral bicalutamide. After being transferred to Scripps Clinic, he was found to have a solitary right upper lobe pulmonary lesion for which he completed stereotactic body radiation therapy. Then, 10 months later, he developed new onset malignant ascites and was treated with two cycles of intravenous docetaxel. Treatment resulted in improvement in his ascites and reduction in PSA. Unfortunately, his course was complicated by tumor lysis syndrome, encephalopathy, Escherichia coli bacteremia, and a fatal saddle pulmonary embolism. Conclusions: (1) Occurrence of ascites in prostate cancer patients is associated with worse prognosis than non-ascitic variants. (2) More common etiologies of ascites must be evaluated for. (3) Ascitic fluid prostate specific antigen (PSA) measurement may aid in diagnosis of malignant ascites in cases with diagnostic uncertainty. (4) Patients with advanced disease may benefit from combined hormonal and cytotoxic therapies. (5) Awareness of the occurrence of tumor lysis syndrome (TLS) in patients treated for prostate cancer can help identify patients in whom prophylaxis would be beneficial.
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