MYCOSIS FUNGOIDES WITH SIGNET-RING CELLS AND MONOCLONAL GAMMOPATHY

A 73-year-old white woman was admitted to our hospital for evaluation of a chronic dermatitis. Personal history was remarkable only for arterial hypertension and noninsulin-dependent6 diabetes mellitus. Clinical and histopathologic findings were consistent with a diagnosis of mycosis fundoides (mf and staging procedures including bolld cell counts, serum biochemistry, urinalysis, bone marrow biopsy, Se zary cell counts in peripheral bolld, computerized tomography (ct scans, abdominal ultrasonography, chest roentgenograms, serum protein electrophoresis, and immunoelectrophoresis disclosed normal or negative results (T2 N0 M0 B0). After 2 years of standard treatment, the patient's condition had progressively worsened and she presented generalized erythematious plaques and a few, small, red-bluish tumors (Fig. 1). A biopsy specimen of a tumor showed an intense lymphoid infiltrate composed mainly of large and medium-sized cells, many of which exhibited clear interacytoplasmic vacuoles (Fig. 2). These vacuoles were negative for periodic acid-Schiff, alcian blue, and oil-red-O staining. Most of the neoplastic cells stained with uchl1 antibody, and none expressed B-cell markers. Some of the malignant cells reacted wih the Ber-h2 and mb3 monoclonal antibodies. No evidence of immunoglobulin libht chain production was observed. Negative staining with cd68 monoclonal antibody ruled out a monocyte-macrophage lineage of the malignant cells. On physical examination, no palpable lymph nodes were found. The quantitative immunoglobulin study showed an IgG level of 6,950 mg/dL (noral values, 800–1,800 mg/dL), IgA level of 126 mg/dL (normal, 90–450 mg/dL), IgM level of 130 mg/dk (normal, 70–280 gm/dL).Serum immunoelectrophoresis showed an IgG lambda monoclonal protein. Further studies including blood cell count, serum biochemistry, urinalysis, bone marrow biopsy, peripheral blood Sezary cell counts, Bence-Jones proteinuria, ct scans, abdominal ultrasonography, and chest roentgenograms yielded negaive or normal results. Thus, diagnosis of monoclonal gammopathy of unknown significance (mgus) was made. Despite treatment with systemic chemotherapy, the diesease followed an aggressive course and the patient died from Staphylococcus epidermidis septicemia.