Abstract
NK/T-cell lymphomas are a group of clonal proliferations of NK- or, rarely, T-cell types and have peculiar clinicopathologic features. Most common site of involvement is the upper aerodigestive tract (nasal cavity, nasopharynx, paranasal sinuses, and palate). Association of autoimmune paraneoplastic disorders with NK/T-cell lymphomas is not well studied. Our patient was diagnosed with NK/T-cell lymphoma stage IV with skin involvement and treated frontline with CHOEP regimen. While he was under treatment, two immune complications presented: anterior uveitis of autoimmune origin refractory to steroids and myositis in lower limbs muscles. Autologous transplantation was rejected due to confirmed early relapse after first-line treatment, and the patient received second-line treatment according to the SMILE scheme, reaching complete response after four cycles. The patient underwent allogeneic transplantation and at the time of manuscript preparation is alive despite multiple complications. The disease should be suspected in patients with rhinitis or recurrent sinusitis, and early biopsy is recommended for all patients to avoid a delay in diagnosis. Our patient also presented symptoms of disease progression after first-line treatment, representing a paraneoplastic process, a very rare phenomenon in T-type lymphomas. This case is novel for the appearance of an inflammatory myositis, a histologically verified paraneoplastic phenomenon that responded to treatment for lymphoma.
Highlights
NK/T-cell lymphomas are a group of clonal proliferations of NK- or, rarely, T-cell types and have peculiar clinicopathologic features
The most common hematologic malignancies associated with dermatomyositis/myositis are B-cell lymphomas [15]
Extranodal NK/T-cell nasal lymphoma is rarely associated with skin lesions mimicking inflammatory or reactive disorders with a similar histopathologic pattern
Summary
NK/T-cell lymphomas are a group of clonal proliferations of NK- or, rarely, T-cell types and have peculiar clinicopathologic features. Extranodal NK/T-cell lymphomas can present in other sites such as the skin, testis, lung, or gastrointestinal tract [2]. Nasal-type lymphoma is clinically less aggressive, is more localized, and has better prognosis [6]. The ENK/T-NT category accounts for less than 2% of nonHodgkin’s lymphomas in Europe and North America but is more frequent in Asia and South and Central America [7]. The association of malignancy with paraneoplastic events is well known, and sometimes the clinical and radiologic features can mimic cellulitis, panniculitis, or fasciitis [2, 6, 9]
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