Multicentric reticulohistiocytosis: response to alkylating agents in six patients

Article Abstract:

Multicentric reticulohistiocytosis is a rare systemic disease, which is characterized by the presence of histiocytes (tissue macrophages that contain lipids, or fatty acids), and giant cells, which contain more than one nucleus, in many tissues. The symptoms of the disease include polyarthritis of the hands and large joints, and the development of nodules in the skin, bone, and mucous and synovial membranes. The disease can be limited to symptoms of arthritis and skin disfigurement or, in approximately 25 percent of the cases, can be malignant, involving the membranes surrounding the body organs and cavities and leading to death. There have been some reports that alkylating agents can cause partial or complete remission of the disease, when other therapies have not been successful. The successful treatment of six patients with multicentric reticulohistiocytosis with alkylating agents - five treated with cyclophosphamide and one with chlorambucil - is reported. A response was seen in the five patients who were treated with cyclophosphamide within four months. Three of the patients remained in remission for 6, 6, and 12 22 months after stopping treatment, while one had a recurrence after six months. The other patient receiving cyclophosphamide had another complication which led to death, although the multicentric reticulohistiocytosis was in remission at the time. The patient who was treated with chlorambucil went into complete remission and remained in remission 32 months after stopping the treatment at 12 months. Therefore, treatment of aggressive multicentric reticulohistiocytosis with alkylating agents has been successful, and after treatment for six to 18 months, patients may be able to stop the treatment and stay in remission. However, further research is needed to see if this is true. The rarity of the disease makes the success of treatment difficult to assess, because double-blind studies cannot be performed. (Consumer Summary produced by Reliance Medical Information, Inc.)

Development and progression, Arthritis, Chlorambucil, Alkylating agents

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Successful treatment of acquired hemophilia with oral immunosuppressive therapy

Article Abstract:

Immunosuppressive drugs seem to be effective in people who develop hemophila rather than inherit it. This condition is rare and is caused by the production of antibodies against factor VIII. Nine patients with no history of hemophilia were hospitalized for severe spontaneous hemorrhage. All had elevated blood levels of autoantibodies against factor VIII. They were treated with cyclophosphamide and prednisone, which are both drugs that suppress the immune system. All nine patients recovered and the levels of factor VIII autoantibodies dropped.

Drug therapy, Hemophilia, Prednisone

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High-dose cyclophosphamide to treat severe aplastic anemia

Article Abstract:

High-dose cyclophosphamide can be used to treat aplastic anemia without also giving the patients a bone marrow transplant, according to a study of 19 patients. Most patients receive both treatments, but many patients do not have a suitable bone marrow donor.

Care and treatment, Aplastic anemia

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