We studied the histological and clinicopathological findings in 42 patients who had lymphomatoid granulomatosis (LYG). In addition to small round lymphocytes, small to intermediate lymphocytes with serpentine nuclei, large immature mononuclear lymphoid cells, abundant histiocytes, and vascular invasion by the cell infiltrate were observed in all cases. Fifty percent of lesions had occasional "atypical" cells with multi-lobed nuclei. Three of four follow-up autopsies showed large cell lymphoma, while one other autopsy and the single repeat biopsy showed increased numbers of large immature mononuclear lymphoid cells. Patients were most frequently men 40-60 yr old who had a history of pulmonary symptoms, such as cough or chest pain, and who showed multiple bilateral lung nodules without hilar adenopathy in the chest x-ray. Thirteen patients (38%) died of disease, 11 of them within 12 mth of initial diagnosis. The presence of neurological signs and symptoms, increased mitoses, or increased numbers of atypical multi-nucleated cells in the initial biopsy were not statistically significant predictors of survival.
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http://dx.doi.org/10.3109/00313028609059478 | DOI Listing |
Radiologia (Engl Ed)
December 2024
Servicio de Radiología, Hospital Universitario Doce de Octubre, Madrid, Spain.
Central nervous system (CNS) involvement by lymphoproliferative disorders is rare and associated with a poor prognosis. CNS involvement can be exclusive, primary or appear in a secondary manner as part of a systemic process. The spectrum of involvement that we encounter is varied and neuroimaging plays a key role in diagnosis.
View Article and Find Full Text PDFOphthalmic Plast Reconstr Surg
December 2024
Department of Ophthalmology & Visual Sciences.
Lymphomatoid granulomatosis, a rare lymphoproliferative disorder, was previously defined by categorical pulmonary involvement with possible invasion into the skin, central nervous system, liver, and kidneys. However, recent reports have documented confirmed cases of lymphomatoid granulomatosis without lung involvement. Here, the authors describe a 70-year-old male with rheumatoid arthritis on methotrexate who presented with an ulcerating lesion on the right lower eyelid, initially suspicious for a basal cell carcinoma.
View Article and Find Full Text PDFActas Dermosifiliogr
November 2024
Universidad de Zaragoza, Zaragoza, España; Servicio de Anatomía Patológica, Hospital Clínico Universitario Lozano Blesa, Zaragoza, España.
Epstein Barr virus (EBV) positive B lymphoproliferative disorders (LPD) with cutaneous involvement include a series of rare entities that go from indolent processes to aggressive lymphomas. B-cell EBV+ LPD mainly affect immunocompromised patients while T-cell EBV+ LPD are more prevalent in specific geographic regions such as Asia, Central America, and South America. Since the latest WHO-EORTC classification of cutaneous lymphomas in 2018, significant changes have been included in the new classifications of hematological malignancies.
View Article and Find Full Text PDFMod Rheumatol Case Rep
January 2025
Department of Nephrology and Rheumatology, Aichi Medical University, Nagakute, Aichi, Japan.
Lymphomatoid granulomatosis (LYG) is a rare, T-cell-rich Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative systemic disease. Only a few cases of LYG have been described in patients with autoimmune disorders, with only one case described in a patient with systemic lupus erythematosus (SLE). However, no cases of isolated central nervous system (CNS)-LYG have been reported in patients with autoimmune diseases.
View Article and Find Full Text PDFDermatol Reports
May 2024
Anatomic Pathology and Cytopathology Unit, G. Pascale Foundation National Cancer Institute IRCCS, Naples, Italy.
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