Appeal type: Experimental/Investigational
Decision: Upheld Decision of Health Plan

Physician 1The patient has HIV infection, extensive Kaposis sarcoma and Castlemans disease, which has not responded to standard therapy consisting of three cycles of Cytoxan and Rituxan. The patient has requested authorization for Velcade. The Health Plan has denied this request indicating that the efficacy of Velcade for the patients diagnosis has not been shown.Castlemans disease is a rare condition with few clinical trials available. Velcade has been used successfully in controlled clinical trials for the treatment of refractory multiple myelomas (FDA indication) as well as a variety of other tumor types. Because Castlemans disease involves uncontrolled growth of lymphoid tissues, it resembles in many ways multiple myeloma, for which Velcade has proven effective. While it is plausible that Velcade may have some positive results for Castlemans disease, there have been no clinical trials, case reports, or protocols using Velcade for treatment of Castlemans disease. Therefore, there is no evidence that Velcade will result in any benefit in the patients condition. Furthermore, it is unknown what harm Velcade could cause the patient.Based upon the information set forth above, I have determined that the requested medication is not likely to be more beneficial for treatment of the patients medical condition than any available standard therapy. Therefore, the Health Plans denial should be upheld.Physician 2The patient is a 34-year-old man with Castlemans disease, Kaposis sarcoma, and HIV infection. Although the exact date is not clear from the provided information, there was a lymph node biopsy done which led to the diagnosis of Castlemans disease. In August 2003, the patient was treated with Cytoxan for Castlemans disease without benefit. He was on high-dose prednisone at the time; the treatment note suggested therapy with Cytoxan and CD20 antibody (Rituxan). In December 2003, the patient was discharged after a two-day hospitalization. Examination before admission revealed an enlarged liver and a markedly enlarged spleen. He was symptomatic with two weeks of fever. Other symptoms included nausea, leg and abdominal pain. He was treated with Cytoxan and Rituxan with apparent resolution of his fever. In January 2004, treatment with Velcade was prescribed. The patient was again hospitalized in February 2004 because of fever and chills. Recently, the patients oncologist noted the patient was neutropenic and febrile. The patient received a second dose of Velcade and his provider noted hepatosplenomegaly. He also noted the patient received four courses of Rituxan without benefit.The patient presumably has the multicentric variant of Castlemans disease. This is an extremely uncommon lymphoproliferative disorder of uncertain cause. It may be caused in some cases by human herpesvirus 8. As was recently noted in the Journal of Clinical Oncology, there is no uniform treatment for multicentric Castlemans disease. The article goes on to point out that the infrequency of the disease is the reason that there is no established regimen for its management. Another article also notes that the optimal therapy for this disease remains uncertain.Velcade is a representative of a new class of chemotherapeutic agents. The drug is Food and Drug Administration approved as an agent for multiple myeloma when other treatments have been ineffective. It is being used on an investigational basis in a number of types of malignancies. There is no standard therapy available for Castlemans disease. Therefore, the use of Velcade is medically appropriate in the case of this patient, especially given his non-response to Cytoxan and Rituxan. Based upon the information set forth above, I have determined that the requested medication is likely to be more beneficial for treatment of the patients medical condition than any available standard therapy. The Health Plans denial should be overturned.Physician 3This is a young man with multiple medical problems, notably HIV and Castlemans disease with limited options in treatment. The patients provider has recommended treatment with Velcade. The Health Plan has denied authorization for the recommended treatment on the basis that it is considered experimental in nature.There is no scientific evidence to suggest that Velcade will benefit the patient. There is no data available on the efficacy of the requested medication for the treatment of Castlemans disease. Thus, there is no evidence that the patient is likely to receive greater benefit from Velcade than from standard therapy. Therefore, I have determined that the requested medication is not likely to be more beneficial for treatment of the patients medical condition than any available standard therapy. The Health Plans denial should be upheld.