OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press61410.17650/1818-8346-2007-0-1-24-30RARE AND DIFFICULT HEMATOLOGIC SYNDROMESРЕДКИЕ И СЛОЖНЫЕ ГЕМАТОЛОГИЧЕСКИЕ СИНДРОМЫParaneoplastic pemphigus - a rare autoimmune complication in patients with chronic lymphocytic leukemiaПаранеопластическая пузырчатка — редкое аутоиммунное осложнение при хроническом лимфолейкозеVolkovaM. A.ВолковаМ. А.
Russian Federation
KitchiginaM. Yu.КичигинаМ. Ю.
Russian Federation

Moscow

PotekaevN. S.ПотекаевН. С.
Russian Federation
TeplyukN. P.ТеплюкН. П.
Russian Federation
KupriyanovaA. G.КуприяноваА. Г.
Russian Federation
N.N. Blokhin Russian Cancer Research Centre, Russian Academy of Medical SciencesРОНЦ им. Н.Н. Блохина, РАМНI.M. Sechenov Moscow Medical AcademyММА им. И.М.СеченоваNational Research Institute of Transplantology and Artificial OrgansНИИ трансплантологии и искусственных органов220220071243022112022221120222007https://oncohematology.abvpress.ru/ongm/article/view/614

This is a case report of the 64-year-old male patient who suffered of previously untreated chronic lymphocytic leukemia (CLL), and paraneoplastic pemphigus. He was treated with prednisolone, MabThera, chlorambucil and cyclophosphamide. The patient achieved remission of CLL, and all symptoms of paraneoplastic pemphigus disappeared. During the year after the treatment he has good performance status, and receives only low doses of chlorambucil and prednisolone.

Приводится описание больного 64 лет с паранеопластической пузырчаткой, развившейся на фоне циклофосфаном илейкераном привело к ремиссии ХЛЛ и исчезновению всех симптомов пузырчатки. В течение года после лечения больной находится в хорошем состоянии и получает только малые дозы лейкерана и преднизолона.

paraneoplastic pemphigusCLLprednisoloneleukeranmabtheraпаранеопластическая пузырчаткаХЛЛпреднизолонлейкеранмабтера1. Anhalt G.J., Chan K.S., Stanley J.R. et al. Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med 1990;393:1729—35.Anhalt G.J., Chan K.S., Stanley J.R. et al. Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med 1990;393:1729—35.2. Korman N.J., Eyre R.W., Klaus-Kovtun V., Stanly J.R. Demonstration of an adhering-junction molecule (plakoglobin) in the autoantigens of pemphigus foliacens and pemphigus vulgaris. N Engl J Med 1989;321:631 — 5.Korman N.J., Eyre R.W., Klaus-Kovtun V., Stanly J.R. Demonstration of an adhering-junction molecule (plakoglobin) in the autoantigens of pemphigus foliacens and pemphigus vulgaris. N Engl J Med 1989;321:631 — 5.3. Ruhrberg Ch., Hajibagheri M.A.N., Parry D.A.D., Watt F.M. Periplakin, a novel component of cornified envelopes and desmosomes that belongs to the plakin family and forms complexes with envoplakin. J Cell Biology 1997;139:1835—49.Ruhrberg Ch., Hajibagheri M.A.N., Parry D.A.D., Watt F.M. Periplakin, a novel component of cornified envelopes and desmosomes that belongs to the plakin family and forms complexes with envoplakin. J Cell Biology 1997;139:1835—49.4. Ашмарин Ю.Я., Буров Г.П., Жгун А.А. О сочетании вульгарной пузырчатки и рака желудка. Клин мед 1959;(4):142—4.Ашмарин Ю.Я., Буров Г.П., Жгун А.А. О сочетании вульгарной пузырчатки и рака желудка. Клин мед 1959;(4):142—4.5. Потекаев Н.С., Кечкер В.И., Николаева Л.П. Злокачественные новообразования и везикуло-буллезные дерматозы. Труды 1 ММИ. Т. 31. Гистохимические и биохимические исследования в дерматологии. М.; 1964. с. 227—32.Потекаев Н.С., Кечкер В.И., Николаева Л.П. Злокачественные новообразования и везикуло-буллезные дерматозы. Труды 1 ММИ. Т. 31. Гистохимические и биохимические исследования в дерматологии. М.; 1964. с. 227—32.6. Mahoney M.G., Aho S., Uitto J., Stanley J. The Members of the plakin family of proteins recognized by paraneoplastic pemphigus antibodies include periplakin. J Invest Dermatol 1998;111:308—13.Mahoney M.G., Aho S., Uitto J., Stanley J. The Members of the plakin family of proteins recognized by paraneoplastic pemphigus antibodies include periplakin. J Invest Dermatol 1998;111:308—13.7. Amagai M., Nishikawa T., Nousari H.C. et al. Antibodies against desmoglein 3(pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantolysis in vivo in necnatal mice. J Clin Invest 1998;102:775—8.Amagai M., Nishikawa T., Nousari H.C. et al. Antibodies against desmoglein 3(pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantolysis in vivo in necnatal mice. J Clin Invest 1998;102:775—8.8. Reich K., Brinck U., Letschart M. et al. Graft-versus host disease —like immunophenotype and apoptotic ker-atinocyte death in paraneoplastic pemphigus. Br J Dermatol 1999;141:739—46.Reich K., Brinck U., Letschart M. et al. Graft-versus host disease —like immunophenotype and apoptotic ker-atinocyte death in paraneoplastic pemphigus. Br J Dermatol 1999;141:739—46.9. Vu Thuong N., Assane N., Bassler K. Classification, clinical manifestations, and immunopatological mechanism of the epithelial variant of paraneoplastic autoimmune multiorgan syndrome: a reappraisal of paraneoplastic pemphigus. Arch Dermatol 2001;137:193—206.Vu Thuong N., Assane N., Bassler K. Classification, clinical manifestations, and immunopatological mechanism of the epithelial variant of paraneoplastic autoimmune multiorgan syndrome: a reappraisal of paraneoplastic pemphigus. Arch Dermatol 2001;137:193—206.10. Kim S.C., Kwon Y.D., Lee Y.J. et al. CDNA cloning of the 210 paraneoplastic pemphigus antigen revels that envoplakin is component of the antigen complex. J Invest Dermatol 1997;109:365—9.Kim S.C., Kwon Y.D., Lee Y.J. et al. CDNA cloning of the 210 paraneoplastic pemphigus antigen revels that envoplakin is component of the antigen complex. J Invest Dermatol 1997;109:365—9.11. Mahler V., Antoni C., Anhalt G.J. et al. Graft-versus-host-like mucocutaneous eruptions with serological features of paraneoplastic pemphigus and sistemic lupus erythematosus in a patient with non-hodgrin lymphoma. Dermatology 1998;197:78—83.Mahler V., Antoni C., Anhalt G.J. et al. Graft-versus-host-like mucocutaneous eruptions with serological features of paraneoplastic pemphigus and sistemic lupus erythematosus in a patient with non-hodgrin lymphoma. Dermatology 1998;197:78—83.12. Nousari H.C,, Deterling R., Wojtczack H. et al. The mechanism of respiratory failure in paraneoplastic pemphigus. N Tngl J Med 1999;340:1406—10.Nousari H.C,, Deterling R., Wojtczack H. et al. The mechanism of respiratory failure in paraneoplastic pemphigus. N Tngl J Med 1999;340:1406—10.13. Wolff H., Kunte C., Messer G. et al. Paraneoplastic pemphigus with fetal pulmonary involvement in a woman with a mesenteric castleman tumor. Br J Dermatol 1999;140:313—6.Wolff H., Kunte C., Messer G. et al. Paraneoplastic pemphigus with fetal pulmonary involvement in a woman with a mesenteric castleman tumor. Br J Dermatol 1999;140:313—6.14. Chin A.C., Stich D., White F.V. et al. Paraneoplastic pemphigus and bronchiolitis obliterans associated with a mediastinal mass: a rare case of castle-man’s disease with respiratory failure requiring lung transplantation. J Pediatric Surgery 2001;36:1—5.Chin A.C., Stich D., White F.V. et al. Paraneoplastic pemphigus and bronchiolitis obliterans associated with a mediastinal mass: a rare case of castle-man’s disease with respiratory failure requiring lung transplantation. J Pediatric Surgery 2001;36:1—5.15. Потекаев Н.С., Теплюк Н.П., Ястребова Р.И. и др. Паранеопластическая пузырчатка в послеоперационном периоде. Росс журн кожн венерич бол 2003;(2):23—5.Потекаев Н.С., Теплюк Н.П., Ястребова Р.И. и др. Паранеопластическая пузырчатка в послеоперационном периоде. Росс журн кожн венерич бол 2003;(2):23—5.16. Шеклаков Н.Д. Одновременное поражение пищевода пузырчаткой и раковой опухолью. Сов мед 1966;(3):122—6.Шеклаков Н.Д. Одновременное поражение пищевода пузырчаткой и раковой опухолью. Сов мед 1966;(3):122—6.17. Теплюк Н.П. Истинная пузырчатка и буллезный пемфигоид как паранеопластические процессы. Альманах клинической медицины. Т. IX. Пролиферативные заболевания кожи. М.; 2006. с. 134—7.Теплюк Н.П. Истинная пузырчатка и буллезный пемфигоид как паранеопластические процессы. Альманах клинической медицины. Т. IX. Пролиферативные заболевания кожи. М.; 2006. с. 134—7.18. Krumic A.L., Kokai D., Basetic B. et al. Retroperitoneal round-cell liposarcoma associated with paraneoplastic pemphigus presenting as lichen planus pemphigoidеs-Цke eruption. Int J Dermatol 1997;36:526—9.Krumic A.L., Kokai D., Basetic B. et al. Retroperitoneal round-cell liposarcoma associated with paraneoplastic pemphigus presenting as lichen planus pemphigoidеs-Цke eruption. Int J Dermatol 1997;36:526—9.19. Anhalt G.J. Paraneoplastic pemphigus: the role of tumors and drugs. Br J Dermatol 2001;144:1101—4.Anhalt G.J. Paraneoplastic pemphigus: the role of tumors and drugs. Br J Dermatol 2001;144:1101—4.20. Nikolskaia O.V., Nousari C.H., Anhalt G.J. Paraneoplastic pemphigus in association with castleman’s disease. Br J Dermatol 2003;149:1143—51.Nikolskaia O.V., Nousari C.H., Anhalt G.J. Paraneoplastic pemphigus in association with castleman’s disease. Br J Dermatol 2003;149:1143—51.21. Kaplan Y., Hodak E., Ackerman L. et al. Neoplasms associated with paraneoplastic pemphigus: a review with emphesis on non-hematologic malignancy and oral mucosal manifestation. Oral Oncol 2004;40:553—62.Kaplan Y., Hodak E., Ackerman L. et al. Neoplasms associated with paraneoplastic pemphigus: a review with emphesis on non-hematologic malignancy and oral mucosal manifestation. Oral Oncol 2004;40:553—62.22. Healy C.M., Thornhill M.H. An association between recurrent oro-geni-tal ulceration and non-steroidal antiinflammatory drugs. J Oral Pathol Med 1995;24:46—8.Healy C.M., Thornhill M.H. An association between recurrent oro-geni-tal ulceration and non-steroidal antiinflammatory drugs. J Oral Pathol Med 1995;24:46—8.23. Gooptu C., Littlewood T.J., Frith P. et al Paraneoplastic pemphigus: an association with fludarabine? Br J Dermatol 2001;144:1255—61.Gooptu C., Littlewood T.J., Frith P. et al Paraneoplastic pemphigus: an association with fludarabine? Br J Dermatol 2001;144:1255—61.24. Valsecchi R., Resengheti A., Cainelli A. Bullous and erosive stomatitis induced by nimesulide. Dermatology 1992;185:74—5.Valsecchi R., Resengheti A., Cainelli A. Bullous and erosive stomatitis induced by nimesulide. Dermatology 1992;185:74—5.25. Kirsner R.S., Anhalt G.J., Kerdel F.A. Treatment with alpha interferon associated with the development of paraneoplastic pemphigus. Br J Dermatol 1995;132:474—8.Kirsner R.S., Anhalt G.J., Kerdel F.A. Treatment with alpha interferon associated with the development of paraneoplastic pemphigus. Br J Dermatol 1995;132:474—8.26. Bronmmann M., von Felbert V., Streit M. et al. Progressive respiratory failure in paraneoplastic pemphigus associated with chronic lymphocytic leukemia. Dermatology 2004;208:251—4.Bronmmann M., von Felbert V., Streit M. et al. Progressive respiratory failure in paraneoplastic pemphigus associated with chronic lymphocytic leukemia. Dermatology 2004;208:251—4.27. Berradori L., Lombardi T., Samson J. et al. Anti-CD20 monoclonal antibody (rituximab) for refractory erosive stomatitis secondary to cd20(+) follicular lymphoma-associated paraneoplastic pemphigus. Arch Dermatol 2001;137:269—72.Berradori L., Lombardi T., Samson J. et al. Anti-CD20 monoclonal antibody (rituximab) for refractory erosive stomatitis secondary to cd20(+) follicular lymphoma-associated paraneoplastic pemphigus. Arch Dermatol 2001;137:269—72.28. Hohwy T., Bang K., Steimche T. et al. Alemtuzumab-induced remission of both severe paraneoplastic pemphigus and leukemic bone morrow infiltration in a case of treatment-resistant b-cell chronic lymphocytic leukemia. Eur J Haematol 2004;73:206—9.Hohwy T., Bang K., Steimche T. et al. Alemtuzumab-induced remission of both severe paraneoplastic pemphigus and leukemic bone morrow infiltration in a case of treatment-resistant b-cell chronic lymphocytic leukemia. Eur J Haematol 2004;73:206—9.29. Camisa C., Helm T.N. Paraneoplastic pemphigus is a distinct neoplasia-induced autoimmune disease. Arch Dermatol 1993;129:883—6.Camisa C., Helm T.N. Paraneoplastic pemphigus is a distinct neoplasia-induced autoimmune disease. Arch Dermatol 1993;129:883—6.30. Yuaoki M., Kodera M., Fujimoto A. et al. Paraneoplastic pemphigus without antidesmoglein 3 or antidesmoglein 1 autoantibodies. Br J Dermatol 2001;144:610—3.Yuaoki M., Kodera M., Fujimoto A. et al. Paraneoplastic pemphigus without antidesmoglein 3 or antidesmoglein 1 autoantibodies. Br J Dermatol 2001;144:610—3.31. Cerroni L., Zenahlik P., Hofler G. et al. Specific cutaneous infiltrates of b-cell chronic lymphocytic leukemia. a clinicopathologic and prognostic study of 42 patients. Am J Surgical Pathol 1996;20:1000—10.Cerroni L., Zenahlik P., Hofler G. et al. Specific cutaneous infiltrates of b-cell chronic lymphocytic leukemia. a clinicopathologic and prognostic study of 42 patients. Am J Surgical Pathol 1996;20:1000—10.32. Hamblin T. Autoimmune disease and its management in chronic lymphocytic leukemia. In: Chronic lymphoid leukemias. B.D. Cheson (ed). N Y, Marsel Dekker; 2001. p. 435—58.Hamblin T. Autoimmune disease and its management in chronic lymphocytic leukemia. In: Chronic lymphoid leukemias. B.D. Cheson (ed). N Y, Marsel Dekker; 2001. p. 435—58.