Summary
A case is reported of a 72 year old man suffering from classical seropositive RA for 10 years. Two months before admission he experienced general illness, fever and itching. Lymphnodes enlargement and hepatosplenomegaly were found. Histologic features of lymphnode biopsy were compatible with angioimmunoblastic lymphadenopathy and Lennert Lymphoma. Rapidly progressive pulmonary deterioration followed with hilar invasion and honeycombing. Sternal punction, bone biopsy, bronchus biopsy and blind lung biopsy however, did not reveal lymphomatous invasion. On the contrary, lung biopsy, showed bronchiolitis obliterans, an often fatal, small airway disease whose connection with RA is discussed. Plasmapheresis induced a correction of leucopenia and thrombocytopenia. A fatal evolution evolved within six months. Autopsy revealed diffuse invasion by immunoblastic sarcoma. The clinicopathological entities angioimmunoblastic lymphadenopathy and Lennert lymphoma are referred to. The relationship with autoimmune disorders is stressed.
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References
Anderson L.G. and Talal N. The spectrum of benign to malignant lymphoprofileration in Sjögren's syndrome. Clin. Exp. Immunol. 1972, 10, 119.
Schleissner L.A., Sheenhan W.W. and Orselli R.C. Lupus erythematosus in a patient with amyloidosis, adrenal insufficiency and subsequent immunoblastic sarcoma. Arthritis Rheum. 1976, 19, 249.
Talal N. and Schnitzer B. Extra-articular manifestations in rheumatoid arthritis. Haematological changes. Clin. Rheum. Dis. 1977, 3(3), 421.
Walker W.C. and Wright V. Pulmonary lesions and Rheumatoid Arthritis. Medecine 1968, 47, 501.
Geddes D.M., Corrin B., Brewerton D.A., Davies R.J, and Turner-Warwick M. Progressive airway obliteration in adults and its association with rheumatoid arthritis. Q.J. Med. 1977, 184, 427–444.
Gosink B.B., Friedman P.J. and Liebow A.A. Bronchiolitis obliterans: A review. Am. J. Röntgenol. 1973, 117, 816–832.
Beroft D.M. Bronchiolitis obliterans, bronchiectasis and other sequela of adenovirus type 21 infection in young children. J. Clin. Pathol. 1971, 24, 72.
Moskowitz R.L., Lyons H.A. and Cottle H.R. Silo-filler' disease: clinical, physiologic and pathologic study of patient. Am. J. Med. 1964, 36, 457.
Chebat J., Seigneur F., Lechien J., Menkes C.J., Simon F. and Marin J.L. Bronchiolitis obliterans au cours des traitements par la D-Penicillamine. Nouv. Press. Med. 1980, 9, 2655.
Epler G.R., Snider G.L., Gaensler E.A., Cathart E.S., Fitzgerald M.X., and Carrington C.B. Bronchiolitis and bronchitis in connective tissue disease. A possible relationship to the use of penicillamine. JAMA 1979, 242, 528–532.
Herne N., Jaubert D., Cannileri G. and Prieur J. Bronchiolite oblitérante au cours de la polyarthrite rhumatoide. Rev. Rhum. Mal. osteoartic 1981, 48, 744.
Lyle W.H. D-Penicillamine and fatal obliterative bronchiolitis. Br. Med. J. 1977, 1, 105.
Siame J.L., Cocheteur P. and Delcambre B. Bronchiolite sévère au cours d'une polyarthrite rhumatoide traitée par la D-Pénicillamine. Rev. Rhum. Mal osteoartic 1981, 48, 605–606.
Neiman R.S., Dervan P., Path, Haudenschild C. and Jaffe R. Angioimmunoblastic lymphadenopathy. Cancer 1978, 41, 507–518.
Flandrin G. Adénopathies angio-immunoblastiques avec anémie auto-immune et hyperglobulinémie polyclonale. Nouv. Press. Med. 1976, 5, 1521.
Bisson M., Massias P., Segond P. and Jacquot J.M. Association d'une lymphadénopathie angioimmunoblastique et d'un syndrome Gougerot-Sjögren. Nouv. Presse Med. 1978, 27, 2393.
Watanabe H. Association of immunoblastic lymphadenopathy and Hashimoto's thyroiditis. Ann. Intern. Med. 1977, 87, 61.
Nathwani B.N., Rappaport H., Moran E.M., Pangalis G.A., and Kim H. Malignant lymphoma arising in angio-immunoblastic lymphadenopathy. Cancer 1978, 41, 578–604.
Kim H., Jacobs C., Warnke R.A. and Dorfman R.F. Malignant lymphoma with a high content of epithelioid histiocytes. Cancer 1978, 41, 620.
Branda R.F., Molodow C.F., Mc Collough J.J., and Jacob H.S. Plasma-exchange in the treatment of Immune disease. Transfusion 1976, 5, 570.
Dequeker J., Geusens P. and Wielandts L. Short and longterm experience with plasmapheresis in connective tissue diseases. Biomedicine 1980, 32, 189–194.
Lockwood C.M., Worlledge S., Nicholas A., Cotton C. and Peters D.K. Reversal of impaired splenic function in patients with nephritis or vasculitis (or both) by plasma-exchange. N. Engl. J. Med. 1979, 300, 524.
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Pieters, R., Martens, J. & Dequeker, J. Rheumatoid arthritis associated with bronchiolitis obliterans and immunoblastic sarcoma. Clin Rheumatol 1, 35–40 (1982). https://doi.org/10.1007/BF02032474
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DOI: https://doi.org/10.1007/BF02032474