Uveitis and Myositis as Immune Complications in Chemorefractory NK/T-Cell Nasal-Type Lymphoma Successfully Treated with Allogeneic Stem-Cell Transplant.
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.
Lymphoma Unit, Department of Hematology, Fundación Jiménez Díaz University Hospital, Health Research Institute IIS-FJD, Madrid, Spain.
Maria José
MJ
Gómez-Crespo
Lymphoma Unit, Department of Hematology, Fundación Jiménez Díaz University Hospital, Health Research Institute IIS-FJD, Madrid, Spain.
Aránzazu
0000-0003-4041-4438
A
García-Raso
Lymphoma Unit, Department of Hematology, Fundación Jiménez Díaz University Hospital, Health Research Institute IIS-FJD, Madrid, Spain.
Jose Luis
JL
López-Lorenzo
Lymphoma Unit, Department of Hematology, Fundación Jiménez Díaz University Hospital, Health Research Institute IIS-FJD, Madrid, Spain.
Teresa
T
Villaescusa
Department of Pathology, Fundación Jiménez Díaz University Hospital, Health Research Institute IIS-FJD, Madrid, Spain.
María
M
Rodríguez-Pinilla
Department of Pathology, Fundación Jiménez Díaz University Hospital, Health Research Institute IIS-FJD, Madrid, Spain.
José
J
Fortes
Lymphoma Unit, Department of Hematology, Fundación Jiménez Díaz University Hospital, Health Research Institute IIS-FJD, Madrid, Spain.
Cristina
C
Serrano
Department of Dermatology, Fundación Jiménez Díaz University Hospital, Health Research Institute IIS-FJD, Madrid, Spain.
Salma
S
Machan
Lymphoma Unit, Department of Hematology, Fundación Jiménez Díaz University Hospital, Health Research Institute IIS-FJD, Madrid, Spain.
Pilar
P
Llamas
Lymphoma Unit, Department of Hematology, Fundación Jiménez Díaz University Hospital, Health Research Institute IIS-FJD, Madrid, Spain.
Raúl
R
Córdoba