Summary:We report the case of a 46-year-old male who developed dermatomyositis and a sarcoid-like reaction in association with testicular relapse of multiple myeloma. The myositis progressed despite chemotherapy directed at the underlying malignant disorder and immunosuppressive treatment. There was, however, a dramatic and sustained response to high-dose chemotherapy and autologous peripheral blood stem cell transplantation which resulted in resolution of the myopathy and partial resolution of the sarcoid-like reaction. This case report highlights the potential of autologous stem cell transplantation as treatment for para-neoplastic disorders associated with haematological malignancies. Bone Marrow Transplantation (2001) 27, 1215-1217. Keywords: multiple myeloma; autologous PBSCT; dermatomyositis; sarcoidosis The presenting clinical features of multiple myeloma are protean and reflect the direct effects of clonal plasma cell proliferation (eg bone marrow failure), the activity of monoclonal antibody (eg amyloid deposition), or the consequence of a complex inter-relationship between the abnormal clone and the host bone marrow micro-environment (eg bone disease). The pathogenesis of the various 'paraneoplastic' disorders which occur in association with myeloma, such as axonal or demyelinating neuropathy, is less certain although antibodies with specificity for proteins expressed by affected tissues, amyloid deposition or changes in cytokine production patterns have all been proposed as potential mechanisms. The optimal treatment for this group of disorders has not been defined and their impact upon prognosis is not known. We report for the first time the effectiveness of high-dose chemotherapy and peripheral blood stem cell transplantation (PBSCT) as treatment for a para-neoplastic disorder associated with multiple myeloma.
Case reportA 46-year-old man presented with a 3 month history of general malaise and 2 week history of vomiting in March 1998. There was no significant past medical history. On examination he appeared unwell and short of breath at rest. He had a raised venous pressure, peripheral oedema and bilateral inspiratory crepitations on chest examination. FBC at presentation was: Hb 8.2 g/dl, white cell count 5.2 × 10 9 /l and platelets 163 ϫ 10 9 /l. Renal function tests confirmed acute renal failure with a creatinine of 844 umol/l (normal range 62-130) and urea 46.4 mmol/l (normal range 2.8-7.6). Corrected serum calcium was 3.43 mmol/l. Chest Xray showed changes consistent with pulmonary oedema. Serum electrophoresis confirmed an IgG lambda paraprotein of 70 g/l. No urinary Bence-Jones protein was detected. Skeletal survey revealed no lytic lesions. Bone marrow trephine was diffusely fibrotic with deposition of collagen (grade 4) and heavy infiltration by neoplastic plasma cells (CD138 positive, lambda light chain restriction). Most plasma cells had the features of plasmblasts. Renal biopsy showed a cast nephropathy.The diagnosis was of IgG lambda myeloma complicated by hypercalcaemia and acute rena...