Multiple Myeloma and Sclerodermatous Disease: Cause and Consequence Emilie S. Chan, Samir Parekh, Ahmed Elshamy, and Peter D. Gorevic. Department of Medicine, Mount Sinai School of Medicine, New York, NY
Sunday, March 4, 2018
South Hall A2 (Convention Center)
Peter D. Gorevic, MD FAAAAI
RATIONALE: Scleroderma (Scl) has been linked to monoclonal gammopathy (MG), cryoglobulinemia, and Multiple Myeloma (MM). Malignancy may complicate RNA polymerase-positive Scl.

METHODS: We describe three patients with both scleroderma (SSc; CREST) or sclerodermatous disease (AL amyloidosis) associated with MG/MM.

RESULTS: Case 1: 54 yo woman presented with skin tightening, decreased oral aperture and dysphagia. Forearm biopsy showed Amyloidosis, with 40% lambda-positive plasma cells on bone marrow (BM) biopsy. Amyloid was typed as AL lambda3r (IGV3-1) by N-terminal sequencing of Bence Jones protein, and of tryptic peptides extracted from tissue. The full sequence of patient IgGV3-1 was obtained by amplifying from BM aspirate, comparing to published germ-line and PBMC lambda3r sequences, and AL data bases. Case 2: 75 yo man developed gastric antral vascular ectasia (GAVE), Raynauds, and sclerodactyly after being diagnosed with smoldering MM and an IgG lambda MG. Serologic evaluation was notable for antibodies to RNA polymerase I/III, further defined by whole exome sequence analysis of BM plasma cells. Case 3: 67 yo woman had been diagnosed with IgGk MM 9 years ago, with 10-20% CD138+ k-restricted plasma cells on serial BM biopsies. Two years ago, she developed raynauds and acral ulcerations, and was found to have anti-centromere antibodies. Further evaluation was notable for significantly low C4/C1q and the presence of a cryoglobulin.

CONCLUSIONS: AL Amyloidosis may present as a scleroderma mimic with an increased incidence of MM. The association of MM with Scleroderma rare, and is considered in the context of a broader incidence of gammopathy and cryoglobulinemia.