Rare Diseases Symptoms Automatic Extraction

A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma-A Case Report and Review of the Literature.

[primary effusion lymphoma]

Primary effusion lymphoma (PEL) or body cavity lymphoma is a rare type of extra nodal lymphoma of B-cell origin that presents as lymphomatous effusion(s) without any nodal enlargement or tumor masses. It belongs to the group of AIDS related non-Hodgkin's lymphomas. First described in 1996 in HIV infected individuals who were coinfected with Kaposi's sarcoma-associated herpesvirus (KSHV) or HHV-8 virus, it was included as a separate entity in WHO classification of tumors of hematopoietic and lymphoid tissue in the year 2001. The definition included association with HHV-8 virus as a mandatory diagnostic criterion. However, cases were later reported where PEL-like disease process was diagnosed in HHV-8 negative patients. This was eventually recognized as a rare but distinct entity termed as "HHV-8-unrelated PEL-like lymphoma". Herein, we are reporting a case of an elderly patient who presented with a large pleuropericardial effusion and was eventually diagnosed with this entity. Till date, only around 50 cases of HHV-8-unrelated PEL-like lymphoma have been reported and our case being EBV, HIV, and Hepatitis C negative makes it very unique and rare occurrence. We are also presenting a review of relevant literature focused mainly on comparing outcomes in patients treated with and without chemotherapy.

Diseases presenting "hepatitis" symptom

  • benign recurrent intrahepatic cholestasis
  • cholangiocarcinoma
  • congenital toxoplasmosis
  • dracunculiasis
  • esophageal squamous cell carcinoma
  • hirschsprung disease
  • inclusion body myositis
  • legionellosis
  • neuralgic amyotrophy
  • papillon-lefèvre syndrome
  • primary effusion lymphoma
  • pyomyositis
  • scrub typhus
  • severe combined immunodeficiency
  • typhoid
  • waldenström macroglobulinemia

This symptom has already been validated