Rare Diseases Symptoms Automatic Extraction

[Successful treatment with oral low-dose sobuzoxane and etoposide combined with rituximab in an elderly patient with HHV-8-negative primary effusion lymphoma-like lymphoma].

[primary effusion lymphoma]

Primary effusion lymphoma (PEL) is a rare B-cell lymphoma, characterized by human herpes virus 8 (HHV8) infection and serous effusions without detectable tumor masses. However, cases with HHV8 unrelated PEL have also been reported, mainly in Japan, and these are referred to as PEL-like lymphoma (PEL-LL). We describe a 70-year-old man with cardiac comorbidity who developed PEL-LL with pleural effusion. The patient achieved a complete response (CR) after treatment with oral low-dose sobuzoxane and etoposide combined with rituximab. To date, the patient has been in CR for about 7 months without chemotherapy. PEL-LL reportedly has a better prognosis than PEL. Because PEL-LL is positive for CD20, unlike PEL, combination therapy including rituximab may be effective. PEL-LL mainly affects elderly people, so that further investigation of tolerable and effective regimens is required.

Diseases presenting "b-cell lymphoma" symptom

  • adrenal incidentaloma
  • alpha-thalassemia
  • esophageal carcinoma
  • hodgkin lymphoma, classical
  • primary effusion lymphoma
  • severe combined immunodeficiency
  • systemic capillary leak syndrome
  • waldenström macroglobulinemia
  • wiskott-aldrich syndrome

This symptom has already been validated