IMPROVEMENT OF SYSTEMIC SCLEROSIS-LIKE SYMPTOMS AFTER THERAPY FOR MULTIPLE MYELOMA

Publication date: November 2018

Source: Annals of Allergy, Asthma & Immunology, Volume 121, Issue 5, Supplement

Author(s): S. Davuluri, A. Tieng, G. Franchin, M. Niazi

Introduction

Systemic sclerosis is known to carry an increased risk of malignancy. In this report we describe a rare case of systemic sclerosis as the presenting sign of amyloidosis associated with multiple myeloma.

Case Description

A 59-year-old woman presented to the clinic with a 6-month history of bilateral hand pain and puffiness. Physical examination revealed melasma, multiple enlarged right supraclavicular lymph nodes, and sclerodactyly. Laboratory tests were positive for antinuclear antibodies (1:160) in a nucleolar pattern and nephrotic range proteinuria (>8g/day). The initial impression was systemic sclerosis associated arthritis and patient was treated with hydroxychloroquine, resulting in slight improvement in her range of motion. Computed tomography of the neck showed mildly enlarged right supraclavicular lymph nodes measuring up to 1.6 centimeters in diameter. Biopsy of a cervical lymph node revealed malignant lymphoma. Bone marrow biopsy showed plasma cell myeloma (>80%); serum immunoelectrophoresis revealed elevated levels of free kappa lambda light chains (8447 mg per liter; reference range, 3.3-19.4). Initial management included cyclophosphamide, bortezomib, and dexamethasone, resulting in significant improvement in joint pain. Furthermore, she developed multiple periorbital papules. A biopsy of her left upper eyelid lesion demonstrated apple-green birefringence after Congo red staining, compatible with a diagnosis of amyloidosis.

Discussion

This patient initially presenting with a picture suggestive of systemic sclerosis was subsequently diagnosed with multiple myeloma and amyloidosis. She developed significant improvement in the range of motion of her finger joints as well as the skin tautness after initiating chemotherapy.

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