Alfa heavy chain disease : a case report and evaluation of the treatment.

Year 1988, Volume: 41 Issue: 1, 131 - 138, 31.03.1988

Nahide Konuk

https://izlik.org/JA44ET85KK

Abstract

Alfa heavy chain disease (alfa-HCD) is an uncommon form of monoclonal ganımapaothy which involves freguenily gastrointestinal iractus. Our case has most of the clinical, immünologicel and epidemiological characteristics of this disease, Having been given 3 times COPP and 4 times CHOP regimens, it was obtained a dramatical remission. Because of the patient's refusing the therapy fora period of 3-4 months, the disease relapsed and didn t respond to CHOP regimen, but it responded to prednisolon 60 mg/m'/d as a single agent. The patient has been still alive for 31 months and he has been in remission for 12 months without therapy.

Keywords

immünologicel , epidemiological , gastrointestinal iractus

BİR ALFA-AĞIR ZİNCİR HASTALIĞI VAKASI VE TEDAVİNİN DEĞERLENDİRİLMESİ

https://izlik.org/JA44ET85KK

Abstract

Alfa-AZH seyrek görülen, gastrointestinal sistemi tutan bir monoklonal gamopati türüdür. Vakamız bu hastalığın klinik, epidemiyolojik, immünolojik özelliklerinin çoğunu taşımaktadır. Kendisine 3 kür COPP, 4 kür CHOP tedavisi verilmiş ve dramatik bir iyilik sağlanmıştır. Hastanın tedaviye ara vermesi nedeniyle hastalık nüksetmiş ve CHOP kürüne yanıt alınamamıştır. Buna karşılık tek alan olarak prednizolon (60 mg/m7 ile klinik tam remisyon elde edilmiştir. On iki aydır önemli bir şikayeti olmadan izlenen hastanın toplam takip süresi 31 aydır.

Keywords

gastrointestinal sistem , epidemiyoloji , immünoloji

References

• 1. Crow J, Asselah F : Immunoproliferative Small Intestinal Disease in Algerains Cancer 54 : 1908-1913. 1984.
• 2. Florin-Christensen A, et al : Alpha chain disease with pulmonary manifestations. Br Med J 20413, 1974.
• 3. Franklin EC, et al : Heavy chain disease. A new disorder of serum gammaglobulins. Am J Med 37: 332, 1964.
• 4. Galian A, et al : Pathological study of alphe-chain disease, with special emphasis on evolution. Cancer 59 : 2081. 1977.
• 5. Monous ON, et al : Alpha chain disease with clinical, İmmunological and histological recovery. Br Med J 2 : 409, 1974.
• 6. Novis BH, etal : Long survival in a patient with alpha-chain disease. Cancer 53 : 970-973, 1984.
• 7. Paykoç Z, Uzunalimoğlu Ö : Primer Proksimal intestinal Lenfoma. Türkiye Klinikleri 3 : 2, 1983.
• 8. Rambaud JC, Seligmann M : Alpha heavy chain disease. Lancet 1 : 1430, 1973.
• 9. Rambaud JCandet al: Alpha heavy chain disease without gualitative serum IgA abnormality. Cancer 51 : 686-693, 1983.
• 10.Rambaud JC et al : Natural history of Alpha-chain disease and the so-called Mediteranean ylmphoma, Recent Results Cancer Res 44 : 271, 1978.
• 11.Seligmann M, et al, : Heavy chain disease : current findings and concepts. Immunol Rev 48 : 145, 1979.
• 12.Seligman M, Rambaud JC : Alpha chain disease : a possible model for the pathogenesis of human Iymphomas. In the Immunopathology of Lymphoreticular Neoplasms, Comperehensive Immunology, Vol 4, Twomey JJ and Good RA (eds). New York : Plenum Mediça IBook Company, p 425, 1978.
• 13.Seligmann M. et al : Alpha chain disease : a new immunoglobulin abnormality. Science 162 : 1396, 1968 . 14.Who report : Alpha chain disease and related small intestinal Iymphoma : a meimorandum. Bull WHO 54; 615, 1976,
• 15.Zlotnick A, Levy M. : Alpha chain disease, a variant ot Mediterranean Iymphoma. Arch Int Med 128 : 432, 1971.