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Complete and durable remission in a patient with life-threatening scleromyxedema treated with high-dose melphalan and BU with auto-SCT

Bone Marrow Transplantation volume 42, pages 215–217 (2008)Cite this article

Scleromyxedema is a rare paraneoplastic fibromucinous connective tissue disorder characterized by papular mucinosis, monoclonal gammopathy and extracutaneous involvements.1 It tends to have a chronic and progressive course, with a high mortality rate from extracutaneous involvements.2 Response to alkylator-based chemotherapy or other therapies tends to be short.2, 3 Chronic oral low-dose melphalan is the most commonly used chemotherapy, but rarely results in durable remissions, and is associated with a 30% TRM rate.2 A few reports suggest significant potential benefit of high-dose chemotherapy (HDCT) and auto-SCT for scleromyxedema patients.4, 5, 6, 7, 8 We report a case of life-threatening scleromyxedema, refractory to prior oral melphalan, but successfully treated with HD melphalan, BU and auto-SCT, with complete clinical remission at 5 years following treatment.

A 51-year-old male presented in March of 2002 with progressive and treatment-refractory scleromyxedema for consideration of auto-SCT. He had a history of pruritic ‘hardened shiny lumps’ over diffusely thickened skin since 1998. Medical conditions included diabetes, recurrent deep venous thrombosis, hypertension and gout. He had no thyroid disorder. He had a monoclonal IgG-λ paraproteinemia and skin biopsy revealed dermal deposition of acidic mucin, diffuse fibroblastic proliferation and focal chronic inflammation. He had received therapies with prednisone, colchicine, penicillamine, methotrexate, melphalan with prednisone and pulse methylprednisolone. His melphalan treatment was started at 10 mg and escalated to 24 mg with prednisone at 100 mg, daily for 4 days per month for 9 months.

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Authors and Affiliations

  1. Division of Medical Oncology, Department of Medicine, Tom Baker Cancer Centre, University of Calgary, Calgary, Alberta, Canada

    T Cheng & D A Stewart

  2. Medical School, University of Calgary, Calgary, Alberta, Canada

    V Gnanakumar

  3. Department of Pathology, Chinook Regional Hospital Laboratory, Chinook Health, Lethbridge, Alberta, Canada. E-mail: tinachen@cancerboard.ab.ca,

    C Hegedus

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  1. T Cheng
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  2. V Gnanakumar
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  3. C Hegedus
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  4. D A Stewart
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Cheng, T., Gnanakumar, V., Hegedus, C. et al. Complete and durable remission in a patient with life-threatening scleromyxedema treated with high-dose melphalan and BU with auto-SCT. Bone Marrow Transplant 42, 215–217 (2008). https://doi.org/10.1038/bmt.2008.148

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