Парциальная красноклеточная аплазия костного мозга в сочетании с тимомой. Обзор литературы и собственные данные

Петренко А.А., Пивник А.В., Дудина Г.А., Дубницкая М.Г. Парциальная красноклеточная аплазия костного мозга в сочетании с тимомой. Обзор литературы и собственные данные. Терапевтический архив. 2019; 91 (7): 121–126. DOI: 10.26442/00403660.2019.07.000326

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Petrenko A.A., Pivnik A.V., Dudina G.A., Dubnitskaya M.G. Pure red cell aplasia of the bone marrow in combination with thymoma. A literature review and own data. Therapeutic Archive. 2019; 91 (7): 121–126. DOI: 10.26442/00403660.2019.07.000326

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Аннотация

Наблюдали 8 пациентов с редким сочетанием тимомы и парциальной краcноклеточной аплазии (ПККА), из них 7 женщин в возрасте от 44 до 68 лет. Диагноз ПККА установлен до выявления тимомы у 1 пациента, одновременно – у 3 пациентов, после выявления – у 4. Семи пациентам произведена тимомэктомия, резекция опухоли – одному пациенту. Вес удаленных тимом составил от 200 до 780 г. Морфологический вариант тимомы тип А (веретеноклеточная) установлен у 2 пациентов, тип В1 – у 2, тип В2 – у 2, тип В3 – у 2. Полные ремиссии получены в результате применения циклофосфамида и циклоспорина у 5 пациентов продолжительностью от 6 мес до 7 лет. Приведены результаты иммунологических исследований с выявлением негемолитических антител к протеолитическому антигену (Pr1d) на мембране эритроцитов у 4 пациентов. Из них у двух исследованных пациентов одновременно выявлены антитела к Pr1d-антигену и межвидовому антигену эритробластов млекопитающих. Показано, что продолжительность жизни эритроцитов не изменена. Прямая проба Кумбса была отрицательной у 5 пациентов, но при помощи агрегат-гемагглютинационной пробы и иммуноферментного анализа антитела на поверхности эритроцитов обнаруживались. Патогенез указанного сочетания заболеваний остается неясным и требует изучения.

Ключевые слова: парциальная красноклеточная аплазия костного мозга, анемия, ретикулоцитопения, тимома.

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Eight patients were observed with a rare combination of thymoma and pure red cell aplasia of bone marrow (PRCA), of which seven women were between 44 to 68 years old. The diagnosis of PRCA was established before the detection of thymoma in 1 patient, simultaneously in 3, after – in 4. Seven patients underwent timomectomy. The weight of removed thymomas was from 200 to 780 grams. Morphological type A thymoma variant (spindle cell) was installed in 2 patients, type B1 – in 2, type B2 – in 2, type B3 – in 2. Complete remissions were obtained using cyclophosphamide and cyclosporin in 5 patients, lasting from 6 months to 7 years. The results of immunological studies with the identification of non-hemolytic antibodies to the proteolytic antigen (Pr1d) on the erythrocyte membrane in 4 patients are presented. Of these, two studied patients simultaneously detected antibodies to the Pr1d antigen and the interspecific antigen of mammalian erythroblasts (IAME). It is shown that the lifespan of red blood cells are not changed. The direct Coombs test was negative in 5 patients, but with the help of aggregate hemaglutination test and enzyme immunoassay, antibodies were detected on the surface of erythrocytes. The pathogenesis of this combination of diseases remains unclear and needs to be elucidated.

Keywords: partial red cell aplasia of the bone marrow, anemia, reticulocytopenia, thymoma.

Список литературы

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8. Silik H. Polayes, Max Lederer. A recipient of many blood transfusions. JAMA. 1930;95(6):407–409. doi:10.1001/jama.1930.27210060002011a
9. Means R. Pure Red Cell Aplasia: Diagnosis and Management. Anemia in the Young and Old. 2019:167-78. doi: 10.1007/978-3-319-96487-4_10
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16. Hashimoto K, Harada M, Kamijo Y. Pure red cell aplasia induced by anti-erythropoietin antibodies, well-controlled with tacrolimus. Intern J Hematology. 2016;104(4):502-5. doi: 10.1007/s12185-016-2047-6
17. Идельсон Л.И., Пивник А.В., Тер-Григоров В.С., Маневич В.Л., Момостюк С.В. Клиника, иммунологические особенности аутоиммунной гемолитической анемии с антителами против антигена эритрокариоцитов, сочетающейся с доброкачественной тимомой. Терапевтический архив. 1981;53(9):99-102 [Idel'son LI, Pivnik AV, Ter-Grigorov VS, Manevich VL, Momostiuk SV. Clinical manifestations, immunological characteristics and therapy of autoimmune hemolytic anemia with antibodies against erythrokaryocyte antigens associated with benign thymoma. Therapeutic Archive. 1981;53(9):99-102 (In Russ.)].
18. Пивник А.В. Аутоиммунные депрессии красного ростка кроветворения: клиника, диагностика, лечение: дис. … докт. мед. наук. М.,1997 [Pivnik AV. Autoimmune depressions of the red sprout of hematopoiesis: clinic, diagnosis, treatment. Thesis for the degree of Doctor of Medical Sciences. Moscow,1997 (In Russ.)].
19. Murakawa T, Nakajima J, Sato H, Tanaka M, Takamoto S and Fukayama M. Thymoma associated with pure red-cell aplasia: clinical features and prognosis. Asian Cardiovasc Thorac Ann. 2002;10:150-4. doi: 10.1177/021849230201000213
20. Thompson CA. Pure red cell aplasia and thymoma. J Thorac Oncol. 2007;2:263-4. doi: 10.1097/01.JTO. 0000263705.23579.ee
21. Balasubramanian SK, Sadaps M, Thota S, Aly M, Przychodzen BP, Hirsch CM, Visconte V, Radivoyevitch T, Maciejewski JP. Rational management approach to pure red cell aplasia. Haematologica. 2018 Feb;103(2):221-30. doi: 10.3324/haematol. 2017.175810
22. Fisch P, Handgretinger R, Schaefer HE. Pure red cell aplasia. Brit J Haematology. 2000 Dec;111(4):1010-22.
23. Kawakami T, Sekiguchi N, Kobayashi J, Imi T, Matsuda K, Yamane T, Nishina S, Senoo Y, Sakai H, Ito T, Koizumi T, Hirokawa M, Nakao S, Nakazawa H, Ishida F. Frequent STAT3 mutations in CD8+ T-cells from patients with pure red cell aplasia. Blood Adv. 2018 Oct 23;2(20):2704-12. doi: 10.1182/bloodadvances.2018022723
24. Wintrobe’s Clinical Hematology. 9th edition, 1993:934.
25. Lipton J, Glader B, Means RT. Red cell aplasia: acquired and congenital disorders. In: Greer JP, Arber DA, Glader BE, List A, Means RT, Paraskevas F, Rodgers GM, eds. Wintrobe’s Clinical Hematology. 13th ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2013:975-89.
26. Zeok JV, Todd EP, Dillon M, DeSimone P, Utley JR. The role of thymectomy in red cell aplasia. Ann Thorac Surg. 1979 Sep;28(3):257-60.
27. Thompson CA, Steensma DP. Pure red cell aplasia associated with thymoma: clinical insights from a 50-year single-institutionexperience. Br J Haematol. 2006 Nov;135(3):405-7. doi: 10.1111/j.1365-2141.2006.06295.x
28. Li P, Du F, Gong Z, Hu B, Chi C, Chu H, Chen J. Cyclosporin A Reversed Chemoresistance of a Patient with Pure Red Cell Aplasia Secondary to Thymoma. Anticancer Res. 2017 Aug;37(8):4611-4. doi: 10.21873/anti canres.11861
29. Moriyama S, Yano M, Haneda H, Okuda K, Kawano O, Sakane T, Oda R, Watanabe T, Nakanishi R. Pure red cell aplasia associated with thymoma: a report of a single-center experience. J Thorac Dis. 2018 Aug;10(8):5066-72. doi: 10.21037/jtd.2018.07.14
30. Fu R, Zhang T, Liu B, Song J, Wang G, Li L, Wang H, Xing L, Wu Y, Guan J, Shao Z. The clinical characteristics and therapy response of patients with acquired pure red cell aplasia. Hematology. 2018 Oct;23(9):639-45. doi: 10.1080/10245332.2018.1470068
31. Wu J, Fang W, Chen G. The enlightenments from ITMIG Consensus on WHO histological classification of thymoma and thymic carcinoma: refined definitions, histological criteria, and reporting. J Thorac Dis. 2016 Apr;8(4):738-43. doi: 10.21037/jtd.2016.01.84

________________________________________________

1. Balasubramanian SK, Sadaps M, Thota S, Aly M, Przychodzen BP, Hirsch CM, Visconte V, Radivoyevitch T, Maciejewski JP. Rational management approach to pure red cell aplasia. Haematol. 2018;103:221-30. doi: 10.3324/haematol.2017.175810
2. Robert T, Means Jr. Pure red cell aplasia. Blood. 2016;128:2504-9. doi: 10.1182/blood-2016-05-717140
3. Sawada K, Fujishima N, Hirokawa M. Acquired pure red cell aplasia: updated review of treatment. Br J Haematol. 2008;142:505-14. doi: 10.1111/j.1365-2141.2008.07216.x
4. Charles RJ, Sabo KM, Kidd PG, Abkowitz JL. The pathophysiology of pure red cell aplasia: implications for therapy. Blood. 1996;87(11):4831-8.
5. Lacy MQ, Kurtin PJ, Tefferi A. Pure red cell aplasia: association with large granular lymphocyte leukemia and the prognostic value of cytogenetic abnormalities. Blood. 1996;87:3000-6.
6. Kaznelson P. Zur Entstehung der Blutplattehen. Verh dtsch Ges inn Med. 1922;34:557.
7. Matraz A. und Priezel A. Uber einige Gewachse des Thymus. Beitr path Anot. 1928 Aug 1;80:207-306.
8. Silik H. Polayes, Max Lederer. A recipient of many blood transfusions. JAMA. 1930;95(6):407–409. doi:10.1001/jama.1930.27210060002011a
9. Means R. Pure Red Cell Aplasia: Diagnosis and Management. Anemia in the Young and Old. 2019:167-78. doi: 10.1007/978-3-319-96487-4_10
10. Krantz SB, Kao V. Studies on Red Cell aplasia. I. Demonstration of a plasma inhibitor to heme synthesis and an antibody to erythroblast nuclei. Proc Nat Acad Sci USA. 1967;58:493-500. doi: 10.1073/pnas.58.2.493
11. Jepson J, Lowenstein L. Hormonal control of erythropoiesis during pregnancy in the mouse. Br J Haematol. 1968 Jun;14(6):555-62.
12. Krantz SB, Moore WH, Zaentz SD. Studies on red cell aplasia. V. Presence of erythroblast cytotoxicity in G-globulin fraction of plasma. J Clin Invest. 1973 Feb;52(2):324-36. doi: 10.1172/JCI107188
13. [Ter-Grigorov VS, Graf IA, Ievleva ES, Pivnik AV, Idel'son LI. Antibodies against interspecies erythrokaryocyte antigen in patients with partial red cell aplasia of the bone marrow. Biull Eksp Biol Med. 1980 Oct;90(10):59-61 (In Russ.)].
14. [Mechetner EB. Analysis of populations of mouse and human erythroblastic cells using monoclonal antibodies. Author's abstract Candidate of Biological Sciences. Moscow, 1985 (In Russ.)].
15. [Idelson LI, Koĭfman MM, Gorina LG, Olovnikov AM. Use of the aggregate-hemagglutination test and other methods for the XX diagnosis of autoimmune hemolytic anemia by the negative direct Coombs' test. Probl Gematol Pereliv Krovi. 1975;20(8):56-61 (In Russ.)].
16. Hashimoto K, Harada M, Kamijo Y. Pure red cell aplasia induced by anti-erythropoietin antibodies, well-controlled with tacrolimus. Intern J Hematology. 2016;104(4):502-5. doi: 10.1007/s12185-016-2047-6
17.[Idel'son LI, Pivnik AV, Ter-Grigorov VS, Manevich VL, Momostiuk SV. Clinical manifestations, immunological characteristics and therapy of autoimmune hemolytic anemia with antibodies against erythrokaryocyte antigens associated with benign thymoma. Therapeutic Archive. 1981;53(9):99-102 (In Russ.)].
18. [Pivnik AV. Autoimmune depressions of the red sprout of hematopoiesis: clinic, diagnosis, treatment. Thesis for the degree of Doctor of Medical Sciences. Moscow,1997 (In Russ.)].
19. Murakawa T, Nakajima J, Sato H, Tanaka M, Takamoto S and Fukayama M. Thymoma associated with pure red-cell aplasia: clinical features and prognosis. Asian Cardiovasc Thorac Ann. 2002;10:150-4. doi: 10.1177/021849230201000213
20. Thompson CA. Pure red cell aplasia and thymoma. J Thorac Oncol. 2007;2:263-4. doi: 10.1097/01.JTO. 0000263705.23579.ee
21. Balasubramanian SK, Sadaps M, Thota S, Aly M, Przychodzen BP, Hirsch CM, Visconte V, Radivoyevitch T, Maciejewski JP. Rational management approach to pure red cell aplasia. Haematologica. 2018 Feb;103(2):221-30. doi: 10.3324/haematol. 2017.175810
22. Fisch P, Handgretinger R, Schaefer HE. Pure red cell aplasia. Brit J Haematology. 2000 Dec;111(4):1010-22.
23. Kawakami T, Sekiguchi N, Kobayashi J, Imi T, Matsuda K, Yamane T, Nishina S, Senoo Y, Sakai H, Ito T, Koizumi T, Hirokawa M, Nakao S, Nakazawa H, Ishida F. Frequent STAT3 mutations in CD8+ T-cells from patients with pure red cell aplasia. Blood Adv. 2018 Oct 23;2(20):2704-12. doi: 10.1182/bloodadvances.2018022723
24. Wintrobe’s Clinical Hematology. 9th edition, 1993:934.
25. Lipton J, Glader B, Means RT. Red cell aplasia: acquired and congenital disorders. In: Greer JP, Arber DA, Glader BE, List A, Means RT, Paraskevas F, Rodgers GM, eds. Wintrobe’s Clinical Hematology. 13th ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2013:975-89.
26. Zeok JV, Todd EP, Dillon M, DeSimone P, Utley JR. The role of thymectomy in red cell aplasia. Ann Thorac Surg. 1979 Sep;28(3):257-60.
27. Thompson CA, Steensma DP. Pure red cell aplasia associated with thymoma: clinical insights from a 50-year single-institutionexperience. Br J Haematol. 2006 Nov;135(3):405-7. doi: 10.1111/j.1365-2141.2006.06295.x
28. Li P, Du F, Gong Z, Hu B, Chi C, Chu H, Chen J. Cyclosporin A Reversed Chemoresistance of a Patient with Pure Red Cell Aplasia Secondary to Thymoma. Anticancer Res. 2017 Aug;37(8):4611-4. doi: 10.21873/anti canres.11861
29. Moriyama S, Yano M, Haneda H, Okuda K, Kawano O, Sakane T, Oda R, Watanabe T, Nakanishi R. Pure red cell aplasia associated with thymoma: a report of a single-center experience. J Thorac Dis. 2018 Aug;10(8):5066-72. doi: 10.21037/jtd.2018.07.14
30. Fu R, Zhang T, Liu B, Song J, Wang G, Li L, Wang H, Xing L, Wu Y, Guan J, Shao Z. The clinical characteristics and therapy response of patients with acquired pure red cell aplasia. Hematology. 2018 Oct;23(9):639-45. doi: 10.1080/10245332.2018.1470068
31. Wu J, Fang W, Chen G. The enlightenments from ITMIG Consensus on WHO histological classification of thymoma and thymic carcinoma: refined definitions, histological criteria, and reporting. J Thorac Dis. 2016 Apr;8(4):738-43. doi: 10.21037/jtd.2016.01.84

Авторы

А.А. Петренко 1, А.В. Пивник 1,2, Г.А. Дудина 1, М.Г. Дубницкая 1

1 ГБУЗ «Московский клинический научный центр им. А.С. Логинова» Департамента здравоохранения г. Москвы, Москва, Россия;
2 Российский университет дружбы народов, Москва, Россия

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A.A. Petrenko 1, A.V. Pivnik 1,2, G.A. Dudina 1, M.G. Dubnitskaya 1

1 Loginov Moscow Clinical Scientific Center, Moscow, Russia;
2 Peoples’ Friendship University of Russia (RUDN University), Moscow, Russia

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