BACKGROUND: Alemtuzumab (MabCampath; ILEX Pharmaceuticals, Geneva, Switzerland) is a humanised monoclonal antibody directed against CD52. It belongs to a new group of monoclonal antibodies with anti-neoplastic effects used in chronic lymphocytic leukaemia (CLL) either as first-line treatment or in those cases resistant to alkylating drugs. Paraneoplastic pemphigus (PNP) is a severe mucocutaneus disease mostly associated with B-cell lymphoproliferative disorders. Independent of the course of the underlying malignancy, this disease is often resistant to conventional immunosuppressive treatment and may lead to death as a result of infectious complications. CASE PRESENTATION: We report a case where an ongoing long-term remission of PNP has been induced by alemtuzumab in a patient with an underlying B-CLL. A 68-yr-old male with a 4-yr history of B-CLL presented with a widespread blistering eruption on the extremities and trunk and a severe stomatitis. The diagnosis of PNP relied on the clinical, histological and direct immunofluorescence findings. Despite intensive treatment strategies with various immunosuppressive drugs and antibiotics, blisters continued to develop and the patient was deteriorating. When treated with alemtuzumab the mucocutaneous lesions healed almost completely within a few weeks and the patients' general condition improved significantly. After 12 wk of treatment with alemtuzumab, the CLL infiltration of the bone marrow previously quantified at 75-80% remitted completely. Twelve months later, the patient was still in remission with only a small residual ulceration on the lip and one on the penis. CONCLUSIONS: Based on this case report we recommend treatment with alemtuzumab to severe cases of PNP in CLL. However, further follow-up of this case is needed in order to assess the long-term effect of alemtuzumab treatment in PNP. Copyright Blackwell Munksgaard 2004.
BACKGROUND:Alemtuzumab (MabCampath; ILEX Pharmaceuticals, Geneva, Switzerland) is a humanised monoclonal antibody directed against CD52. It belongs to a new group of monoclonal antibodies with anti-neoplastic effects used in chronic lymphocytic leukaemia (CLL) either as first-line treatment or in those cases resistant to alkylating drugs. Paraneoplastic pemphigus (PNP) is a severe mucocutaneus disease mostly associated with B-cell lymphoproliferative disorders. Independent of the course of the underlying malignancy, this disease is often resistant to conventional immunosuppressive treatment and may lead to death as a result of infectious complications. CASE PRESENTATION: We report a case where an ongoing long-term remission of PNP has been induced by alemtuzumab in a patient with an underlying B-CLL. A 68-yr-old male with a 4-yr history of B-CLL presented with a widespread blistering eruption on the extremities and trunk and a severe stomatitis. The diagnosis of PNP relied on the clinical, histological and direct immunofluorescence findings. Despite intensive treatment strategies with various immunosuppressive drugs and antibiotics, blisters continued to develop and the patient was deteriorating. When treated with alemtuzumab the mucocutaneous lesions healed almost completely within a few weeks and the patients' general condition improved significantly. After 12 wk of treatment with alemtuzumab, the CLL infiltration of the bone marrow previously quantified at 75-80% remitted completely. Twelve months later, the patient was still in remission with only a small residual ulceration on the lip and one on the penis. CONCLUSIONS: Based on this case report we recommend treatment with alemtuzumab to severe cases of PNP in CLL. However, further follow-up of this case is needed in order to assess the long-term effect of alemtuzumab treatment in PNP. Copyright Blackwell Munksgaard 2004.