Angiocentric lymphomatoid granulomatosis and severe hypogammaglobulinaemia

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Abstract

Angiocentric lymphomatoid granulomatosis is a rare lymphoproliferative disease, mainly associated with pulmonary manifestation. Its origin is unknown, but Epstein-Barr virus may be one of the etiological factors. A 51-year-old male had an abdominal laparotomy in 1994 and a large granulomatous mass was removed from behind the cecum. No specific therapy was administerred. In February 1998 multiple pulmonary lesions were found by X-ray and thoracoscopic biopsy was made. The histopathological diagnosis was angiocentric lymphomatoid granulomatosis. The patient received 6 cycles of CHOP chemotherapy, with which a complete remission was achieved. A consistent severe hypogammaglobulinaemia was detected, so the diagnosis of common variable immunodeficiency (CVID) was established. The diagnosed CVID was the probable causative factor of the angiocentric lymphomatoid granulomatosis. After the CHOP treatment, the patient is on intravenous immunoglobulin substitution and is well up to today.

Original languageEnglish
Pages (from-to)535-541
Number of pages7
JournalHaematologia
Volume32
Issue number4
Publication statusPublished - Dec 1 2002

Keywords

  • Angiocentric lymphomatoid granulomatosis
  • Common variable immunodeficiency
  • Hypogammaglobulinaemia
  • Intravenous immunoglobulin
  • Non-Hodgkin's lymphoma

ASJC Scopus subject areas

  • Hematology

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