Lymphomatoid granulomatosis mimicking cancer and sarcoidosis.

Two cases of misdiagnoses of lymphomatoid granulomatosis are discussed here. Lymphomatoid granulomatosis is an Epstein-Barr virus-associated lymphoproliferative disorder with aggressive behavior. Due to its rarity and many presentations, delay in diagnosis and treatment is common. Its histological features including large atypical B-cells, T cell predominance, angiocentricity, necrosis, and evidence of EBV-positive cells should elicit the diagnosis of lymphomatoid granulomatosis. The settings that are described here have not yet been described in the literature.

A case of lymphomatoid granulomatosis-like lung lesions with abundant infiltrating IgG4-positive plasma cells whose serum IgG4 levels became high following the start of corticosteroid therapy.

A 59-year-old man with a history of prostate hyperplasia was admitted to our hospital for further examination of a lung mass and renal dysfunction. Lung biopsy specimens revealed that inflammatory cells had infiltrated into the blood vessel walls. We initially suspected lymphomatoid granulomatosis, but Epstein Barr virus-encoded small RNA was negative. However, 50% of the infiltrating plasma cells were positive for IgG4. Furthermore, the kidneys and prostate contained abundant IgG4-positive plasma cells. He was diagnosed with IgG4-related sclerosing disease even though serum IgG4 levels were not elevated (45.7 mg/dL). Prednisolone reduced the lung masses and ameliorated renal function, but the serum IgG4 level increased (377 mg/dL). Seronegative IgG4-related sclerosing disease should be considered when patients present with such symptoms and treatment responses, and the secretion of IgG4 might be blocked by its active synthesis.

Case report: lymphadenopathy, perinodal granulomatosis, and immunologic aberrations.

The authors report on a patient with an undescribed constellation of lymph nodal plasmacytosis, perinodal epithelioid cell granulomas and fibrosis as well as anti-Sm and anti-phospholipid antibodies. The illness does not meet the criteria of well-known nosologic entities, but it is thought to represent an unrecognized nosological entity within the group of undifferentiated immune disorders.

Hypercalcemia with excess serum 1,25 dihydroxyvitamin D in lymphomatoid granulomatosis/angiocentric lymphoma.

Hypercalcemia has been described in a variety of granulomatous and lymphoproliferative disorders in association with elevated serum levels of 1,25-dihydroxyvitamin D. In such cases, hypercalcemia appears to be the consequence of excessive production of 1,25(OH)2D by the lymphocyte/macrophage line. The authors report a patient with lymphomatoid granulomatosis/angiocentric lymphoma who developed hypercalcemia with extreme elevation in serum 1,25(OH)2D. Therapy with steroids reduced the serum calcium and 1,25(OH)2D levels to normal. Hypercalcemia has not previously been reported in lymphomatoid granulomatosis/angiocentric lymphoma. The distinctive features of this malignancy, and the derangement in the metabolism of 1,25(OH)2D in lymphoproliferative disorders in general, are discussed.

Granulomatosis de Wegener: presentacion de un caso tratado exitosamente con ciclofosfamida / Wegener's granulomatosis: report of one case succesfully treated with cyclophosphamide

Se presenta el caso de una mujer de 37 anos con enfermedad multisistemica caracterizada clinicamente por uveitis rinitis, otitis media cronica, nodulos pulmonares, ulcera genital, paralisis facial periferica, fiebre y perdida de peso, con evidencia por examenes paraclinicos de anemia, leucocitosis con neutrofilia, eritrosedimentacion elevada, aumento de la IgA, uroanalisis francamente patologico y rapido deterioro de la funcion renal. Estos hallazgos clinics y paraclinicos sumados a la confirmacion histopatologica de una glomerulonefritis necrotizante focal y segmentaria permitieron concluir que el cuadro correspondia a una granulomatosis de Wegener clasica, obteniendose excelente respuesta al tratamiento con ciclofosfamida y prednisolona. La granulomatosis de Wegener es una entidad en la cual es muy importante el diagnostico temprano, ya que la iniciacion precoz del tratamiento ofrece posibilidades de remision de la enfermedad.

[Lymphomatoid granulomatosis and hypersensitivity pneumopathy. Clinical and biological study (serum and alveolar) toward a new pathogenic hypothesis of the disease]. / Granulomatose lymphomatoïde et pneumopathie d'hypersensibilité. Etude clinique et biologique (sérique et alvéolaire) vers de nouvelles hypothèsses pathogéniques de la maladie.

The authors discussed the malign form of lymphomatoid granulomatosis on the basis of two cases and consider the possible role of an aerocontaminant which is usually responsible for the extrinsic allergic type of alveolitis. A detailed biochemical and cytological study of the alveolar fluid from one of these patients is presented in parallel with immunofluorescent histology and electron microscopy of the lung. The observed biochemical abnormalities in the alveolar fluid could be superimposed on those formed in certain types of hypertensive lung disease. On the other hand the cytology was different, the cytofluorimetric technique revealed a mononuclear cell population rich in RNA whose role in the genesis of the disease of malign nature are discussed. The hypothesis of a hypersecretion of IgA by this malign process is raised by immunofluorescent studies of the lung. The role of IgA in lymphomatoid granulomatosis and bird fanciers lung is discussed in the light of recent work on pigeon IgA.