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 "lymphoma" symptom

  • adrenal incidentaloma
  • alpha-thalassemia
  • carcinoma of the gallbladder
  • cushing syndrome
  • dedifferentiated liposarcoma
  • erdheim-chester disease
  • erythropoietic protoporphyria
  • esophageal adenocarcinoma
  • esophageal carcinoma
  • familial hypocalciuric hypercalcemia
  • focal myositis
  • hirschsprung disease
  • hodgkin lymphoma, classical
  • kabuki syndrome
  • liposarcoma
  • locked-in syndrome
  • monosomy 21
  • oculocutaneous albinism
  • primary effusion lymphoma
  • severe combined immunodeficiency
  • systemic capillary leak syndrome
  • waldenström macroglobulinemia
  • wiskott-aldrich syndrome

This symptom has already been validated