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Lymphomatoid granulomatosis with splenomegaly and pancytopenia

Abolhasan Halvani1, Mohammad Bagher Owlia, Ramin Sami

  • 1Department of Internal Medicine, Shaheed Sadoughi Hospital, Shaheed Sadoughi University of Medical Sciences, Safaeieh, Yazd, Iran. halvani47@yahoo.com

Zhongguo Fei AI Za Zhi = Chinese Journal of Lung Cancer
|August 3, 2010
PubMed

Insights

Lymphomatoid granulomatosis (LG) is a rare angiocentric lymphoproliferative disease. This case highlights its unusual presentation with neurological symptoms and pancytopenia in a young patient.

Area of Science:

  • Pathology
  • Oncology
  • Neurology

Background:

  • Lymphomatoid granulomatosis (LG) is an uncommon angiocentric lymphoproliferative disorder.
  • Typically affects the lungs, skin, and central nervous system.

Observation:

  • A 15-year-old male presented with prolonged fever, cough, and dyspnea.
  • Developed nodular skin lesions, hepatosplenomegaly, and neurological deficits including seizures and hemiplegia.
  • Imaging revealed bilateral pulmonary nodules; laboratory tests showed pancytopenia.

Findings:

  • Histopathological and immunohistochemical analysis of skin biopsy confirmed lymphomatoid granulomatosis.
  • The patient exhibited rare manifestations including splenomegaly and pancytopenia.

Implications:

  • This case underscores the diverse clinical presentations of LG, including rare neurological involvement.
  • Highlights the importance of considering LG in young patients with unexplained pulmonary, skin, and neurological symptoms.
  • Emphasizes the aggressive nature of the disease, even with treatment, as seen in the fatal neurological progression.

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