Идиопатическая мультицентрическая болезнь Кастлемана

Васильев В.И., Пальшина С.Г., Павловская А.И. и др. Идиопатическая мультицентрическая болезнь Кастлемана. Терапевтический архив. 2020; 92 (5): 78–84. DOI: 10.26442/00403660.2020.05.000440

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Vasilyev V.I., Palshina S.G., Pavlovskaya A.I., et al. Idiopathic multicentric Castleman's disease. Therapeutic Archive. 2020; 92 (5): 78–84. DOI: 10.26442/00403660.2020.05.000440

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Аннотация

Идиопатическая мультицентрическая болезнь Кастлемана (ИМБК) – редкое лимфопролиферативное заболевание, имеющее множество сходных лабораторных, рентгенорадиологических и клинико-патологических проявлений с различными состояниями, включая и IgG4-cвязанное заболевание. Повышение активности цитокинов, особенно интерлейкина-6, приводит к системным воспалительным симптомам с развитием лимфаденопатии и редко – экстранодальных поражений. Гистологические изменения в лимфатических узлах демонстрируют картину гиалиноваскулярного, плазмоклеточного или смешанного вариантов болезни Кастлемана и бывают трудноотличимы от картины реактивной, опухолевой и IgG4-связанной лимфаденопатии. ИМБК может быть диагностирована только при исключении инфицирования человеческим герпес-вирусом 8-го типа и вирусом иммунодефицита человека. В статье авторы приводят описание двух случаев ИМБК, включая первое в мировой литературе описание экстранодального поражения в мышце бедра при данной нозологии. Приведен обзор литературы по основным клинико-лабораторным и морфологическим проявлениям, позволяющим подтвердить диагноз ИМБК.

Ключевые слова: идиопатическая мультицентрическая болезнь Кастлемана, лимфопролиферативные заболевания, IgG4-связанные заболевания, экстранодальные поражения при болезни Кастлемана.

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Idiopathic multicentric Castleman's disease is a rare lymphoproliferative disorder that has many similar laboratory, radiological, clinical and pathological manifestations with various conditions, including IgG4-related disease. Increased activity of cytokines, especially interleukin-6, leads to systemic inflammatory symptoms with the development of lymphadenopathy and rarely extranodal lesions. Histological changes in the lymph nodesin hyaline vascular and plasma cell variants of Castleman's disease are hardly distinguishable from the pattern of reactive, tumor and IgG4-related lymphadenopathy. Idiopathic multicentric Castleman's disease can be diagnosed only when infection with human herpesvirus-8 type and human immunodeficiency virus is excluded. In the article, the authors describe two cases of idiopathic multicentric Castleman's disease, including the first world literature description of extranodal damage of the hip muscle in this disorder. In addition, the authors gave a review of the literature on the main clinical, laboratory and morphological manifestations, which allow confirming the diagnosis of Castleman's disease.

Keywords: idiopathic multicentric Castleman's disease, lymphoproliferative diseases, IgG4-related disease, extranodal lesions in Castleman's disease.

Список литературы

1. Castleman D, Towne VW. Case records of the Massachusets General Hospital weekly clinicopathologic exercises: case 40011. N Engl Med. 1954;250(1):26-30. doi: 10.1056/NEJM195401072500107
2. Castleman B, Iverson L, Menendez VP. Localized mediastinal lymphonode hyperplasia resembling thymoma. Cancer. 1956;9(4):822-30.
doi: 10.1002/1097-0142(195607/08)9:4<822:aid-cncr2820090430>
3.0.co;2-4
3. Flendrig JA. Benign giant lymphoma: clinicopathologic correlation study. In: Clark RL, Cumley RW (Eds.) The year book of cancer. Year Book Medical, Chicago; 1970; p. 296–9.
4. Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of mediastinum and other locations. Cancer. 1972;29(3):670–83. doi: 10.1002/1097-0142(197203)29:3<670::AID-CNCR2820290321>3.0.CO;2-#
5. Frizzera G, Banks PM, Massarelli G, Rosai J. A systemic lymphoproliferative disorder with morphologic features of Castleman’s disease. Pathological findings in 15 patients. Am J Surg Pathol. 1983;7(3):211-31. doi: 10.1200/jco.1985.3.9.1202
6. Lachant NA, Sun NC, Leong LA, et al. Multicentricangiofollicular lymph node hyperplasia (Castleman’s disease) followed by Kaposi’s sarcoma in two homosexual males with the acquired immunodeficiency syndrome (AIDS). Am J Clin Pathol. 1985;83(1):27-33. doi: 10.1093/ajcp/83.1.27
7. Oksenhendler T, Duarte M, Soulier J, et al. Multicentric Castelman’s disease in HIV infection: a clinical and pathological study of 20 patients. AIDS. 1996;10(1):61-7. doi: 10.1097/00002030-199601000-00009
8. Soulier J, Grollet L, Oksenhendler E, et al. Kaposi’s sarcoma-associated herpesvirus-like DNA sequences in multicentric Castleman’s disease. Blood. 1995;86(4):1276-80.
9. Wang Hao-Wey, Pittaluga S, Jaffe ES. Multicentric Castleman’s disease: Where are we now? Semin Diagn Pathol. 2016;33:294-306. doi: 10.1053/j.semdp.2016.05.006
10. Liu AY, Nabel CS, Finkelman BS, et al. Idiopathic multicentric Castleman’s disease: a systematic literature review. Lancet Hematol. 2016;3(4):e163-75. doi: 10.1016/s2352-3026(16)00006-5
11. Kawabata H, Takai K, Kojima M, et al. Castleman-Kojima disease (TAFRO syndrome): a novel systemic inflammatory disease characterized by a constellation of symptoms, namely, thrombocytopenia, ascites (anasarca), microcytic anemia, myelofibrosis, renal dysfunction, and organomegaly. J Clin Exp Hematop. 2013;53(1):57-61. doi: 10.3960/jslrt.53.57
12. Fajgenbaum DC, Uldrick TS, Bagg A, et al. International, evidence-based consensus diagnostic criteria for HHV-8-nеgative/idiopathic multicentric Castleman disease. Blood. 2017. doi: 10.1182/blood-2016-10-746933
13. Гласко Е.Н., Капланская И.Б., Лукина Е.А. Ангиофолликулярная гиперплазия лимфатических узлов. Арх. пат. 1986;1:51-5 [Glas-
ko EN, Kaplanskaya IB, Lukina EA. Angiofollikulyarnaya giperplaziya limfaticheskikh uzlov. Arkh. pat. 1986;1:51-5 (In Russ.)].
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kyan AL, Kaplanskaya IB, Korneva EP, Frank GA. Bolezn' Kastlemana. Therapeutic Archive. 2005;77(7):48-53 (In Russ.)].
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17. Venizelos I, Papathomas TG, Papathanasiou M, et al. Orbital involvement in Castleman Disease. Clinical Pathologic reviews. Eds. Seregard S, Boni-
uk M. Surv Ophthalmol. 2010;85(3):247-55. doi: 10.1016/j.survophthal.2009.09.003
18. Kang D, Lee J, Lee H, Baek S. Unicentric Castleman’s disease in orbit: A case report. Indian J Ophthalmol. 2015;63(6):555-7. doi: 10.4103/0301-4738.162645
19. Fiel-Gan MD, Voytek TM, Weiss RG, et al. Castleman’s disease of the left triceps in a child suspected to be a small round cell tumor of childhood. Pediatr Dev Pathol. 2000;3(3):286-9. doi: 10.1007/s100249910038
20. Rooney RS, Pitcher JD. Castleman’s disease in extremity. Am J Orthopsychiatry. 1998;27:373-4.
21. Dargent JL, Delplace J, Roufosse C, et al. Development of a calcifying fibrous pseudotumour within a lesion of Castleman disease, hyaline-vascular subtype. J Clin Pathol. 1999;52:547-9. doi: 10.1136/jcp.52.7.547
22. Skelton HG, Smith KJ. ExtranodalmulticentricCastleman’s disease with cutaneous involvement. Mod Pathol. 1998;11:983-9.
23. Cоhen H. Tumor-like proliferations of lymphoid tissue-occurrence in deltoid muscle and mediastinum. J Mount Sinai Hosp. 1957;24:750-60.
24. Eward WC, DeWitt SB, Brigman BE, et al. Extranodal Castleman disease of the extremities: a case report and review of the literature. Skeletal Radiol. 2014;43:1627-31. doi: 10.1007/s00256-014-145-z
25. Kazakov DV, Fanburg-Smith JC, Suster S, et al. Castleman disease of the Subcutis and underlying skeletal muscle. Report of cases. Am J Surg Pathol. 2004;28(5):569-77. doi: 10.1097/00000478-200405000-00002
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shev SKh, Vasil'ev VI, Kovrigina AM, et al. Zabolevanie, svyazannoe s IgG4: kharakteristika gruppy bol'nykh i terapiya rituksimabom. Therapeutic Archive. 2013;85(2):48-53 (In Russ.)].
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31. Сокол Е.В. Клинико-лабораторные особенности IgG4-связанного заболевания в ревматологии. Автореф. дис. … канд. мед. наук. М., 2016; c. 1-22 [Sokol EV. Kliniko-laboratornye osobennosti IgG4-svyazannogo zabolevaniya v revmatologii. Avtoref. dis. … kand. med. nauk. Moscow, 2016; p. 1-22 (In Russ.)].
32. Егорова Е.К. Клинико-морфологические особенности различных вариантов болезни Кастлемана. М., 2015; с. 1-23 [Egorova EK. Kliniko-morfologicheskie osobennosti razlichnykh variantov bolezni Kastlemana. Moscow, 2015; p. 1-23 (In Russ.)].
33. Cheuk W, Yuen HKL, Chu SYY. Lymphadenopathy of IgG4-related sclerosing disease. Am J Surg Pathol. 2008;32:671-81. doi: 10.1097/PAS.0b013e318157c068
34. Grimm KE, Barry TS, Chizhevsky V, et al. Histopathological findings in 29 lymph node biopsies with increased IgG4 plasma cells. Modem Pathol. 2012;25:480-91. doi: 10.1038/modpathol.2011.177
35. Sato Y, Kojima M, Takata K, et al. Systemic IgG4-related lymphadenopathy: a clinical and pathologic comparison to multicentric Castleman’s disease. Modem Pathol. 2009;22:589-99. doi: 10.1038/modpathol.2009.17
36. Umehara H, Okazaki K, Kawano M, et al. How diagnose IgG4-related disease. Ann Rheum Dis. 2017. doi: 10.1136/annrheumdis-2017-211330
37. Sato Y, Kojima M, Takata K, et al. Multicentric Castleman’s disease with abundant IgG4-positive cells: a clinical and pathological analysis of six cases. J Clin Pathol. 2010;63:1084-9. doi: 10.1136/jcp.2010.082958
38. Васильев В.И., Сокол Е.В., Родионова Е.Б. и др. Связанные с IgG-4 поражения слюнных желез. Терапевтический архив. 2015;87(8):92-102 [Vasil'ev VI, Sokol EV, Rodionova EB, et al. Svyazannye s IgG-4 porazheniya slyunnykh zhelez. Therapeutic Archive. 2015;87(8):92-102 (In Russ.)]. doi: 10.17116/terarh201587892-102
39. Сheuk W, Chan JKS. IgG4-related sclerosing disease. A critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol. 2010;17:303-32. doi: 10.1097/PAP.0b013e3181ee63ce
40. Егорова Е.К., Суборцева И.Н., Плискунова Ю.В. и др. Болезнь Кастлемана и IgG4-ассоциированная лимфаденопатия. Гематол. и трансфузиол. 2014;59(4):25-30 [Egorova EK, Subortseva IN, Pliskunova YuV, et al. Bolezn' Kastlemana i IgG4-assotsiirovannaya limfadenopatiya. Gematol. i Transfuziol. 2014;59(4):25-30 (In Russ.)].
41. Chan JK, Fletcher CD, Hicklin GA, et al. Glomeruloid hemangioma: a distinctive cutaneous lesion of multicentric Castleman’s disease associated with POEMS syndrome. Am J Surg Pathol. 1990;14:1036-46. doi: 10.1097/00000478-199011000-00005
42. Foss HD, Araujo I, Demel G, et al. Expression of vascular endothelial growth factor in lymphomas and Castleman’s disease. J Pathol. 1997;183:44-50. doi: 10.1002/(sici)1096-9896(199709)183:1<44::aid-path1103>3.0.co;2-i

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1. Castleman D, Towne VW. Case records of the Massachusets General Hospital weekly clinicopathologic exercises: case 40011. N Engl Med. 1954;250(1):26-30. doi: 10.1056/NEJM195401072500107
2. Castleman B, Iverson L, Menendez VP. Localized mediastinal lymphonode hyperplasia resembling thymoma. Cancer. 1956;9(4):822-30.
doi: 10.1002/1097-0142(195607/08)9:4<822:aid-cncr2820090430>
3.0.co;2-4
3. Flendrig JA. Benign giant lymphoma: clinicopathologic correlation study. In: Clark RL, Cumley RW (Eds.) The year book of cancer. Year Book Medical, Chicago; 1970; p. 296–9.
4. Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of mediastinum and other locations. Cancer. 1972;29(3):670–83. doi: 10.1002/1097-0142(197203)29:3<670::AID-CNCR2820290321>3.0.CO;2-#
5. Frizzera G, Banks PM, Massarelli G, Rosai J. A systemic lymphoproliferative disorder with morphologic features of Castleman’s disease. Pathological findings in 15 patients. Am J Surg Pathol. 1983;7(3):211-31. doi: 10.1200/jco.1985.3.9.1202
6. Lachant NA, Sun NC, Leong LA, et al. Multicentricangiofollicular lymph node hyperplasia (Castleman’s disease) followed by Kaposi’s sarcoma in two homosexual males with the acquired immunodeficiency syndrome (AIDS). Am J Clin Pathol. 1985;83(1):27-33. doi: 10.1093/ajcp/83.1.27
7. Oksenhendler T, Duarte M, Soulier J, et al. Multicentric Castelman’s disease in HIV infection: a clinical and pathological study of 20 patients. AIDS. 1996;10(1):61-7. doi: 10.1097/00002030-199601000-00009
8. Soulier J, Grollet L, Oksenhendler E, et al. Kaposi’s sarcoma-associated herpesvirus-like DNA sequences in multicentric Castleman’s disease. Blood. 1995;86(4):1276-80.
9. Wang Hao-Wey, Pittaluga S, Jaffe ES. Multicentric Castleman’s disease: Where are we now? Semin Diagn Pathol. 2016;33:294-306. doi: 10.1053/j.semdp.2016.05.006
10. Liu AY, Nabel CS, Finkelman BS, et al. Idiopathic multicentric Castleman’s disease: a systematic literature review. Lancet Hematol. 2016;3(4):e163-75. doi: 10.1016/s2352-3026(16)00006-5
11. Kawabata H, Takai K, Kojima M, et al. Castleman-Kojima disease (TAFRO syndrome): a novel systemic inflammatory disease characterized by a constellation of symptoms, namely, thrombocytopenia, ascites (anasarca), microcytic anemia, myelofibrosis, renal dysfunction, and organomegaly. J Clin Exp Hematop. 2013;53(1):57-61. doi: 10.3960/jslrt.53.57
12. Fajgenbaum DC, Uldrick TS, Bagg A, et al. International, evidence-based consensus diagnostic criteria for HHV-8-nеgative/idiopathic multicentric Castleman disease. Blood. 2017. doi: 10.1182/blood-2016-10-746933
13. Гласко Е.Н., Капланская И.Б., Лукина Е.А. Ангиофолликулярная гиперплазия лимфатических узлов. Арх. пат. 1986;1:51-5 [Glas-
ko EN, Kaplanskaya IB, Lukina EA. Angiofollikulyarnaya giperplaziya limfaticheskikh uzlov. Arkh. pat. 1986;1:51-5 (In Russ.)].
14. Меликян А.Л., Капланская И.Б., Корнева Е.П., Франк Г.А. Болезнь Кастлемана. Терапевтический архив. 2005;77(7):48-53 [Meli-
kyan AL, Kaplanskaya IB, Korneva EP, Frank GA. Bolezn' Kastlemana. Therapeutic Archive. 2005;77(7):48-53 (In Russ.)].
15. Melikyan AL, Egorova EK, Kovrigina AM, et al. Kliniko-morfologicheskie osobennosti razlichnykh variantov bolezni Kastel'mana. Therapeutic Archive. 2015;87(7):64-71 (In Russ.) doi: 10.17116/terarkh201587764-71
16. Casper C. The aetiology and management of Castleman disease at 50 years: translating pathophysiology to patient care. Br J Haematol. 2005;129(1):3-17. doi: 10.1111/j.1365-2141.2004.05311.x
17. Venizelos I, Papathomas TG, Papathanasiou M, et al. Orbital involvement in Castleman Disease. Clinical Pathologic reviews. Eds. Seregard S, Boni-
uk M. Surv Ophthalmol. 2010;85(3):247-55. doi: 10.1016/j.survophthal.2009.09.003
18. Kang D, Lee J, Lee H, Baek S. Unicentric Castleman’s disease in orbit: A case report. Indian J Ophthalmol. 2015;63(6):555-7. doi: 10.4103/0301-4738.162645
19. Fiel-Gan MD, Voytek TM, Weiss RG, et al. Castleman’s disease of the left triceps in a child suspected to be a small round cell tumor of childhood. Pediatr Dev Pathol. 2000;3(3):286-9. doi: 10.1007/s100249910038
20. Rooney RS, Pitcher JD. Castleman’s disease in extremity. Am J Orthopsychiatry. 1998;27:373-4.
21. Dargent JL, Delplace J, Roufosse C, et al. Development of a calcifying fibrous pseudotumour within a lesion of Castleman disease, hyaline-vascular subtype. J Clin Pathol. 1999;52:547-9. doi: 10.1136/jcp.52.7.547
22. Skelton HG, Smith KJ. ExtranodalmulticentricCastleman’s disease with cutaneous involvement. Mod Pathol. 1998;11:983-9.
23. Cоhen H. Tumor-like proliferations of lymphoid tissue-occurrence in deltoid muscle and mediastinum. J Mount Sinai Hosp. 1957;24:750-60.
24. Eward WC, DeWitt SB, Brigman BE, et al. Extranodal Castleman disease of the extremities: a case report and review of the literature. Skeletal Radiol. 2014;43:1627-31. doi: 10.1007/s00256-014-145-z
25. Kazakov DV, Fanburg-Smith JC, Suster S, et al. Castleman disease of the Subcutis and underlying skeletal muscle. Report of cases. Am J Surg Pathol. 2004;28(5):569-77. doi: 10.1097/00000478-200405000-00002
26. Sedyshev SKh, Vasil'ev VI, Kovrigina AM, et al. Zabolevanie, svyazannoe s IgG4: kharakteristika gruppy bol'nykh i terapiya rituksimabom. Therapeutic Archive. 2013;85(2):48-53 (In Russ.)
27. Logvinenko OA, Vasil'ev VI. Nekhodzhkinskie limfomy pri revmaticheskikh zabolevaniyakh. Rheumatology Science and Practice. 2011;49(5):71-82 (In Russ.) doi: 10.14412/1995-4484-2011-1464
28. Sedyshev SK, Vasiliev VI, Kovriguina AM, Nasonov EL. IgG4-linked systemic disease. Modern look on "old" disease. Rheumatology Science and Practice. 2012;50(5):64-72 (In Russ.) doi: 10.14412/1995-4484-2012-1184
29. Sokol EV, Vasilyev VI. Treatment of IgG4-related disease. Rheumatology Science and Practice. 2016;54(3):352-60 (In Russ.) doi: 10.14412/1995-4484-2016-352-360
30. Sedyshev SKh. Zabolevaniya, proyavlyayushchiesya simmetrichnym uvelicheniem slyunnykh zhelez I tkanei orbit v revmatologicheskoi praktike. Avtoref. dis. … kand. med. nauk. Moscow, 2012; p. 1-23 (In Russ.)
31. Sokol EV. Kliniko-laboratornye osobennosti IgG4-svyazannogo zabolevaniya v revmatologii. Avtoref. dis. … kand. med. nauk. Moscow, 2016; p. 1-22 (In Russ.)
32. Egorova EK. Kliniko-morfologicheskie osobennosti razlichnykh variantov bolezni Kastlemana. Moscow, 2015; p. 1-23 (In Russ.)
33. Cheuk W, Yuen HKL, Chu SYY. Lymphadenopathy of IgG4-related sclerosing disease. Am J Surg Pathol. 2008;32:671-81. doi: 10.1097/PAS.0b013e318157c068
34. Grimm KE, Barry TS, Chizhevsky V, et al. Histopathological findings in 29 lymph node biopsies with increased IgG4 plasma cells. Modem Pathol. 2012;25:480-91. doi: 10.1038/modpathol.2011.177
35. Sato Y, Kojima M, Takata K, et al. Systemic IgG4-related lymphadenopathy: a clinical and pathologic comparison to multicentric Castleman’s disease. Modem Pathol. 2009;22:589-99. doi: 10.1038/modpathol.2009.17
36. Umehara H, Okazaki K, Kawano M, et al. How diagnose IgG4-related disease. Ann Rheum Dis. 2017. doi: 10.1136/annrheumdis-2017-211330
37. Sato Y, Kojima M, Takata K, et al. Multicentric Castleman’s disease with abundant IgG4-positive cells: a clinical and pathological analysis of six cases. J Clin Pathol. 2010;63:1084-9. doi: 10.1136/jcp.2010.082958
38. Vasil'ev VI, Sokol EV, Rodionova EB, et al. Svyazannye s IgG-4 porazheniya slyunnykh zhelez. Therapeutic Archive. 2015;87(8):92-102 (In Russ.) doi: 10.17116/terarh201587892-102
39. Сheuk W, Chan JKS. IgG4-related sclerosing disease. A critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol. 2010;17:303-32. doi: 10.1097/PAP.0b013e3181ee63ce
40. Egorova EK, Subortseva IN, Pliskunova YuV, et al. Bolezn' Kastlemana i IgG4-assotsiirovannaya limfadenopatiya. Gematol. i Transfuziol. 2014;59(4):25-30 (In Russ.)
41. Chan JK, Fletcher CD, Hicklin GA, et al. Glomeruloid hemangioma: a distinctive cutaneous lesion of multicentric Castleman’s disease associated with POEMS syndrome. Am J Surg Pathol. 1990;14:1036-46. doi: 10.1097/00000478-199011000-00005
42. Foss HD, Araujo I, Demel G, et al. Expression of vascular endothelial growth factor in lymphomas and Castleman’s disease. J Pathol. 1997;183:44-50. doi: 10.1002/(sici)1096-9896(199709)183:1<44::aid-path1103>3.0.co;2-i

Авторы

В.И. Васильев1, С.Г. Пальшина1, А.И. Павловская2, Н.В. Кокосадзе2, Б.Д. Чальцев1, Л.А. Шорникова3

1 ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой», Москва, Россия;
2 ФГБУ «Национальный медицинский исследовательский центр онкологии им. Н.Н. Блохина» Минздрава России, Москва, Россия;
3 ФГБОУ ВО «Московский государственный медико-стоматологический университет им. А.И. Евдокимова» Минздрава России, Москва, Россия

________________________________________________

V.I. Vasilyev1, S.G. Palshina1, A.I. Pavlovskaya2, N.V. Kokosadze2, B.D. Chaltsev1, L.A. Shornikova3

1 Nasonova Research Institute of Rheumatology, Moscow, Russia;
2 Blokhin National Medical Research Center of Oncology, Moscow, Russia;
3 Yevdokimov Moscow State University of Medicine and Dentistry, Moscow, Russia

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