Track

Case Reports

Abstract

Waldenström macroglobulinemia (WM) is characterized by lymphoplasmacytic lymphoma (LPL) with an IgM monoclonal protein. Cutaneous manifestations are typically related to elevated IgM, which can cause hyperviscosity, cryoglobulinemia, or immunoglobulin deposition. However, rarely, LPL can infiltrate the skin itself.

A 64-year-old male with IgM kappa LPL/WM presented to dermatology with asymptomatic erythematous patches with telangiectasias on the chest for three years, emerging one year before WM diagnosis. By report, prior skin biopsy revealed dermal edema and perivascular lymphocytic infiltrate with eosinophils, consistent with urticarial tissue reaction. Cryoglobulin, cryofibrinogen, and ANA testing were negative. CRP and ESR were elevated; C4 was low. Serum monoclonal IgM kappa (1.583 g/dL) was detected. Schnitzler’s syndrome was considered; however the patient denied systemic symptoms. Repeat skin punch biopsies demonstrated sparse atypical perivascular lymphoid infiltrates composed of lymphoplasmacytoid CD20-positive, kappa-restricted B cells, consistent with cutaneous involvement by the patient’s low-grade LPL. No large cell transformation was appreciated. Congo red was negative for amyloid. Bone marrow biopsy showed 20% involvement by LPL that was positive for MYD88 L265P mutation and negative for CXCR4 mutation.

Cutaneous manifestations of WM are rare, and skin infiltration of neoplastic B-cells is rarer yet. When skin infiltration does occur, the literature describes the lesions as indurated papules and plaques. This is the first report of a patchy, telangiectatic rash associated with LPL/WM and highlights the importance of close examination of cytomorphology of even scant lymphocytes in patients with hematologic malignancies. Increased recognition of cutaneous LPL may expedite diagnosis and impact systemic management.