Extensive Combined Surgeries in Patients with Blood Diseases and Gastric Cancer

S.R. Karagyulyan, K.I. Danishyan, A.V. Grzhimolovskii, V.S. Shavlokhov, S.A. Shutov, M.A. Silaev, L.G. Kovaleva, O.M. Sorkina, A.Yu. Bulanov

Hematology Research Center of RF MH, Moscow, Russian Federation

For citation: Karagyulyan S.R., Danishyan K.I., Grzhimolovskii A.V., Shavlokhov V.S., Shutov S.A., Silaev M.A., Kovalyova L.G., Sorkina O.M., Bulanov A.Yu. Extensive Combined Surgeries in Patients with Blood Diseases and Gastric Cancer. Klin. onkogematol. 2014; 7(3): 335–42 (In Russ.).


ABSTRACT

The second tumor in patients with myelo- or lymphproliferative diseases is not rare. However, the state of patients’ hemostasis and severely enlarged spleen can become a contraindication for a surgical intervention in a general surgery unit. Four cases of a successful gastrectomy with expanded lymphodissection are presented in a 53 year old woman with subleukemic myelosis (primary myelofibrosis according to WHO classification) and giant splenomegaly (38 ´ 21 ´ 13 cm, spleen weight: 4500 g) and in a 68 year old man with diffuse large В-cell lymphoma and massive splenomegaly (22 ´ 12 ´ 8 cm, spleen weight: 2850 g). Intraoperative compensated blood loss in both cases was 3800 ml and 3740 ml, respectively. Two patients had mild hemophilia A. Specially designed transfusional and anaesthetic strategy was required in all cases because of hemostatic disorders.


Keywords: stomach cancer, surgical treatment, hemophilia, myelofibrosis, lymphoma.

Address correspondence to: max-blood@mail.ru

Accepted: May 28, 2014

Read in PDF (RUS)pdficon


REFERENCES

  1. Климанский В.А., Бекназаров Я.Б. Опасности и осложнения спле- нэктомии у больных с заболеваниями системы крови. Хирургия 1986; 1: 88–92. [Klimanskii V.A., Beknazarov Ya.B. Hazards and complications of splenectomy in patients with blood diseases. Khirurgiya 1986; 1: 88–92. (In Russ.)].
  2. Климанский В.А. Хирургические аспекты лечения заболевания си- стемы крови. Клин. мед. 1989; 8: 3–8.  [Klimanskii V.A. Surgical aspects of treatment of blood diseases. Klin. med. 1989; 8: 3–8. (In Russ.)].
  3. Arnoletti J.P., Karam J., Brodsky J. Early Postoperative Complications of Splenectomy for Hematologic Disease. Am. J. Clin. Oncol. 1999; 22(2): 114–8.
  4. Nicholson I.A., Falk G.L., Mulligan S.C. Laparoscopically assisted massive splenectomy. A preliminary report of the technique of early hilar devascularization. Surg. Endoscopy 1998; 12: 73–5.
  5. Petroianu A. Subtotal splenectomy for the treatment of chronic lymphocytic leukemia. Ann. Hematol. 2003; 82(11): 708–9. Epub 2003 Aug 2.
  6. Smith L., Luna G., Merg A.R. et al. Laparoscopic splenectomy for treatment of splenomegaly. Am. J. Surg. 2004; 187(5): 618–20.
  7. Мещерякова Л.М., Ковалева Л.Г., Карагюлян С.Р. Патофизиологиче- ские основы лечения сублейкемического миелоза. В кн.: Патофизиология крови. Экстремальные состояния (сборник работ). Под ред. А.И. Воро- бьева, Н.А. Горбуновой. М., 2004: 122–34. [Meshcheryakova L.M., Kovaleva L.G., Karagyulyan S.R. Pathophysiological principles of treatment of subleukemic myelosis. In: Vorob’ev A.I., Gorbunova N.A., eds. Patofiziologiya krovi. Ekstremal’nye sostoyaniya (sbornik rabot). (Blood pathophysiology. Urgent conditions (collection of papers)). Moscow, 2004. pp. 122–34].
  8. Ковалева Л.Г., Карагюлян С.Р., Колосова Л.Ю. и др. Спленэктомия при сублейкемическом миелозе. Гематол. и трансфузиол. 2004; 49(5): 14–21. [Kovaleva L.G., Karagyulyan S.R., Kolosova L.Yu. et al. Splenectomy in subleukemic myelosis. Gematol. i transfuziol. 2004; 49(5): 14–21. (In Russ.)].
  9. Демидова А.В., Хорошко Н.Д. Сублейкемический миелоз. В кн.: Руко- водство по гематологии. Под ред. А.И. Воробьева. М.: Ньюдиамед, 2003; 2: 16–20. [Demidova A.V., Khoroshko N.D. Subleukemic myelosis. In: Vorob’ev A.I., ed. Rukovodstvo po gematologii. (Handbook in hematology). Moscow: N’yudiamed Publ., 2003. Т. 2. рр. 16–20.].
  10. Ковалева Л.Г., Карагюлян С.Р., Колосова Л.Ю. и др. Спленэктомия при сублейкемическом миелозе. Гематол. и трансфузиол. 2004; 5: 14–21. [Kovaleva L.G., Karagyulyan S.R., Kolosova L.Yu. et al. Splenectomy in subleukemic myelosis. Gematol. i transfuziol. 2004; 5: 14–21. (In Russ.)].
  11. Вуд М.Э., Бан П.А. Секреты гематологии и онкологии: Пер. с англ. Под ред. Ю.Н. Токарева, А.Е. Бухны. М.: Бином, 1997. [Wood M.E., Bun P.A. Hematology/Oncology Secrets (Russ. Ed. Yu.N. Tokarev, A.E. Bukhny, eds. Wood M.E., Bun P.A. Sekrety gematologii i onkologii. Moscow: Binom Publ., 1997].
  12. Давыдов М.И., Тер-Ованесов М.Д., Полоцкий Б.Е., Туркин И.Н. Рак желудка. В кн.: Энциклопедия клинической онкологии. Под ред. М.И. Давыдова. М.: РЛС, 2004: 223–7. [Davydov M.I., Ter-Ovanesov M.D., Polotskii B.E., Turkin I.N. Gastric cancer. In: Davydov M.I. Entsiklopediya klinicheskoi onkologii. (Encyclopedia of clinical oncology). Moscow: RLS Publ., 2004. рр. 223–7.]. Горобец Е.С. Принципы анестезии при абдоминальных онкологиче- ских операциях. Регион. анест. и леч. боли 2009; 3(2): 32. [Gorobets E.S. Principles of anesthesia in abdominal oncological surgeries. Region. anest. i lech. boli 2009; 3(2): 32. (In Russ.)].
  13. Щербакова О.В., Шулутко Е.М., Буланов А.Ю. и др. Лапароскопи- ческая спленэктомия в условиях эндотрахеальной анестезии: динамика показателей гемостаза. Эндоскоп. хир. 2004; 1: 192–3. [Shcherbakova O.V., Shulutko E.M., Bulanov A.Yu. et al. Laparoscopic splenectomy with endotracheal anesthesia: changes in hemostatic parameters. Endoskop. khir. 2004; 1: 192–3. (In Russ.)].
  14. Chappell D., Heindl B., Jacob M. et al. Sevoflurane reduces leukocyte and platelet adhesion after ischemia-reperfusion by protecting the endothelial glycocalyx. Anesthesiology 2011; 115(3): 483–91.
  15. Буланов А.Ю., Городецкий В.М., Щербакова О.В. и др. Тромбо- эластографическая оценка системы гемостаза и эффективность ее коррекции перед оперативными вмешательствами у больных с заболеваниями системы крови. Гематол. и трансфузиол. 2012; 57(5): 36–42. [Bulanov A.Yu., Gorodetskii V.M., Shcherbakova O.V. et al. Thromboelastographic evaluation of the hemostatic system and the efficacy of its correction before surgical intervention in patients with blood diseases. Gematol. i transfuziol. 2012; 57(5): 36–42. (In Russ.)].
  16. Буланов А.Ю., Аграчева Н.С., Шулутко Е.М. и др. Т-клеточная лим- фома и рак желудка у пожилого пациента. Трансфузионная терапия при хирургическом вмешательстве. Клин. геронтол. 2009; 15(3): 47–50. [Bulanov A.Yu., Agracheva N.S., Shulutko E.M. et al. T-cell lymphoma and gastric in an elderly patient. Transfusion therapy in surgical intervention. Klin. gerontol. 2009; 15(3): 47–50. (In Russ).]
  17. Mendez D., De La Cruz, Arrebola M.M. et al. The effect of propofol on interaction of platelets with leukocytes and erythrocytes in surgical patients. Anesth. Analg. 2003; 96: 713–9.

Autoimmune lymphoproliferative syndrome: literature review and case report

I.B. Kovynev1, T.I. Pospelova1, A.V. Mishenin2, I.N. Nechunaeva2, and R.V. Tarnovsky1

1 Novosibirsk State Medical University, Novosibirsk, Russian Federation

2 Municipal Clinical Hospital #2, Novosibirsk, Russian Federation


ABSTRACT

The article presents the literature review and the rare case of autoimmune lymphoproliferative syndrome (ALPS). The disease is associated with blocked apoptosis of immune cells caused by mutations in Fas-protein genes. The clinical course is characterized by progressive generalized lymphadenopathy with the picture of reactive hyperplasia in lymph nodes biopsy specimens and immune 1- to 3-lineage cytopenia(s). Potential of transformation into lymphoma or systemic lupus erythematosus (SLE) and current treatment approaches are discussed


Keywords: autoimmune lymphoproliferative syndrome, cytopenias, autoimmune hemolytic anemia, autoimmune neutropenia, lymphadenopathy, lymphoproliferative disease, immune thrombocytopenic purpura, lymphoma

Read in  PDF (RUS)pdficon


REFERENCES

  1. Jackson C.E., Puck J.M. Autoimmune lymphoproliferative syndrome, impaired apoptosis. Curr. Opin. Pediatr. 1999; 11(6): 521–7.
  2. Fleisher T.A., Straus S.E., Bleesing J.J. et al. Genetic disorders lymphocyte apoptosis pathway involving FAS: autoimmune lymphoproliferative syndrome. Curr. Allergy Asthma Rep. 2001; 1(6): 534–40.
  3. Oren H., Ozkal S., Gulen H. et al. Autoimmune lymphoproliferative syndrome: report of two cases and review of the literature. Ann. Hematol. 2002; 81(11): 651–3.
  4. Sneller M.C., Wang J., Dale J.K. et al. Clincal, immunologic, and genetic features of an autoimmune lymphoproliferative syndrome associated with abnormal lymphocyte apoptosis. Blood 1997; 89(4): 1341–8.
  5. Sneller M.C., Dale J.K., Straus S.E. Autoimmune lymphoproliferative syndrome. Curr. Opin. Rheumatol. 2003; 15(4): 417–21.
  6. Straus S.E. et al. An inherited disorder of lymphocyte apoptosis: the autoimmune lymphoproliferative syndrome. Ann. Intern. Med. 1999; 130(7): 591–601.
  7. Canale V.C., Smith C.H. Chronic lymphadenopathy simulating malignant lymphoma. J. Paediatr. 1967; 70: 899.
  8. Sneller M.C., Straus S.E., Jaffe E.S. et al. A novel lymphoproliferative/ autoimmune syndrome resembling murine lpr/gld disease. J. Clin. Invest. 1992; 90(2): 334–41.
  9. Watanabe-Fukunaga R., Brannan C.I., Copeland N.G. et al. Lymphoproliferation disorder in mice explained by defects in Fas antigen that mediates apoptosis. Nature 1992; 356(6367): 314–7.
  10. Rieux-Laucat F., Le Deist F., Hivroz C. et al. Mutations in Fas associated with human lymphoproliferative syndrome and autoimmunity. Science 1995; 268(5215): 1347–9.
  11. Rieux-Laucat F., Le Deist F., Fischer A. Autoimmunelymphoproliferative syndromes: genetic defects of apoptosis pathways. Cell Death Differ. 2003; 10(1): 124–33.
  12. Rao V.K., Oliveira J.B. How I treat autoimmune lymphoproliferative syndrome. Blood 2011; 118(22): 5741–51.
  13. Lenardo M.J. Fas and the art of lymphocyte maintenance. J. Exp. Med. 1996; 183(3): 721–4.
  14. Ройт А., Бростофф Дж., Мейл Д. Иммунология. М.: Мир, 2000. [Royt A., Brostoff J., Meyl D. Immunologiya (Immunology). M.: Mir, 2000.]
  15. Щербина А.Ю., Продеус А.П., Румянцев А.Г. Иммунодефицитные состояния. Трудный пациент 2007; 2: 28–30. [Shcherbina A.Yu., Prodeus A.P., Rumyantsev A.G. Immunodefitsitnyye sostoyaniya (Immunodeficiency states). Difficult patient 2007; 2: 28–30.]
  16. Данилов И.П. Гематологические аспекты первичного иммунодефи- цита и аутоиммунных заболеваний. Мед. новости 2005; 4: 5–8. [Danilov I.P. Hematologicheskiye aspecty pervichnogo immunodefitsita i autoimmunnykh zabolevaniy (Hematological aspects of primary immunodeficiency and autoimmune disorders). Med. novosti 2005; 4: 5–8.]
  17. Teachey D.T., Seif A.E., Grupp S.A. Advances in the management and understanding of autoimmune lymphoproliferative syndrome (ALPS). Br. J. Haematol. 2010; 148(2): 205–16.
  18. Teachey D.T. New advances in the diagnosis and treatment of autoimmune lymphoproliferative syndrome. Curr. Opin. Pediatr. 2012; 24(1): 1–8.
  19. Ries F., Ferster A., Rieux-Laucat F. et al. Autoimmune lymphoproliferative syndrome (ALPS).Case report and family history. Bull. Soc. Sci. Med. Grand Duche Luxemb. 2010; 2: 271–8.
  20. Seif A.E., Manno C.S., Sheen C., Grupp S.A., Teachey D.T. Identifying autoimmune lymphoproliferative syndrome in children with Evans syndrome: a multi-institutional study. Blood 2010; 115(11): 2142–5.
  21. Madkaikar M., Mhatre S., Gupta M., Ghosh K. Advances in autoimmune lymphoproliferative syndromes. Eur. J. Haematol. 2011; 87(1): 1–9.
  22. Turbyville J.C., Rao V.K. The autoimmune lymphoproliferative syndrome: A rare disorder providing clues about normal tolerance. Autoimmun. Rev. 2010: 488–93.
  23. Kim Y.J., Dale J.K., Noel P., Brown M.R. et al. Eosinophilia is associated with a higher mortality rate among patients with autoimmune lymphoproliferative syndrome. Am. J. Hematol. 2007; 82: 615–24.
  24. Лисуков И.А., Ковынев И.Б., Лосева М.И. Изучение экспрессии дифференцировочных антигенов лимфопоэза на мембране мононуклеаров периферической крови при опухолевой прогрессии лимфосарком. Тер. арх. 1991; 7(63): 78–80. [Lisukov I.A., Kovynev I.B., Loseva M.I. Izucheniye ekspressii differentsirovochnykh antigenov limfopoeza na membrane mononuklearov perifericheskoy krovi pri opukholevoy progressii limfosarkom (Studies of lymphopoiesis differentiation antigens expression on the membrane of peripheral blood mononuclear cells in lymphosarcoma tumor progression). Ter. arkh. 1991; 7(63): 78–80.]
  25. Лисуков И.А., Ковынев И.Б., Лосева М.И. Экспрессия эритроидных дифференцировочных антигенов на мононуклеарных клетках перифе- рической крови при опухолевой прогрессии неходжкинской злокаче- ственной лимфомы. Гематол. и трансфузиол. 1991; 36(3): 9–10. [Lisukov I.A., Kovynev I.B., Loseva M.I. Ekspressiya eritroidnykh differentsirovochnykh antigenov na mononuklearnykh kletkah perefericheskoy krovi pri opukholevoy progressii nekhodzhkinskoy zlokachestvennoy limfomy (Expression of erythroid differentiation antigens on peripheral blood mononuclear cells in tumor progression of non-Hodgkin’s malignant lymphoma). Gematol. i transfuziol. 1991; 36(3): 9–10.]
  26. Ковынев И.Б., Поспелова Т.И., Лосева М.И. Значение иммуномор- фологических маркеров апоптоза при оценке опухолевой прогрессии лимфобластной неходжкинской лимфомы. Журн. клин. и экспер. мед. 2004; 1(2): 190–5. [Kovynev I.B., Pospelova T.I., Loseva M.I. Znacheniye immunomorfologicheskikh markerov apoptoza pri otsenke opukholevoy progressii limfoblastnoy nekhodzhkinskoy limfomy (Significance of immunomorphological markers of apoptosis in assessment of tumor progression of non-Hodgkin’s lymphoblastic lymphoma). Zhurn. klin. i eksper. med. 2004; 1(2): 190–5.]
  27. Oliveira J.B., Bleesing J.J., Dianzani U. et al. Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop. Blood 2010; 116(14): 35–40.
  28. Dowdell K.C., Niemela J.E., Price S. et al. Somatic FAS mutations are common in patients with genetically undefined autoimmune lymphoproliferative syndrome. Blood 2010; 115(25): 5164–9.
  29. Bleesing J.J., Brown M.R., Dale J.K. et al. TcR-alpha/beta(+) CD4(–) CD8(–) T cells in humans with the autoimmune lymphoproliferative syndrome express a novel CD45 isoform that is analogous to murine B220 and represents a marker of altered O-glycan biosynthesis. Clin. Immunol. 2001; 100: 314–24.
  30. Straus S.E., Jaffe E.S., Puck J.M. et al. The development of lymphomas in families with autoimmunelymphoproliferative syndrome with germline Fas mutations and defective lymphocyte apoptosis. Blood 2001; 98: 194–200.
  31. Strober W., Fuss I.J., Dale J.K. et al. Characteristic T helper 2 T cell cytokine abnormalities in autoimmune lymphoproliferative syndrome, a syndrome marked by defective apoptosis and humor autoimmunity. J. Immunol. 1997; 158(4): 1912–8.
  32. Levy Y., Brouet J.C. Interleukin-10 prevents spontaneous death of germinal center B cells by induction of the bc1-2 protein. J. Clin. Invest. 1994; 93(1): 424–8.
  33. Li L., Krajewski S., Reed J.C., Choi Y.S. The apoptosis and proliferation of SAC-activated B cells by IL-10 are associated with changes in Bcl-2, Bcl-xL, and Mcl-1 expression. J. Clin. Invest. 1994; 93424–8.
  34. Cohen S.B., Crawley J.B., Kahan M.C. et al. Interleukin-10 rescues T cells from apoptotic cell death: association with an upregulation of Bcl-2. Immunology 1997; 921–5.
  35. Horwitz D.A., Gray J.D., Behrendsen S.C. et al. Decreased production of interleukin-12 and other Th1-type cytokines in patients with recent-onset systemic lupus erythematosus. Arthritis Rheum. 1998; 41(5): 838–44.
  36. Tanaka H., Nagai H., Maeda Y. Effect of anti-IL-4 and anti-IL-5 antibodies on allergy airway hyperresponsiveness in mice. Life Sci. 1994; 62: PL169–74.
  37. Caminha I., Fleisher T.A., Hornung R.L. et al. Using biomarkers to predict the presence of FAS mutations in patients with features of the autoimmune lymphoproliferative syndrome. J. Allergy Clin. Immunol. 2010; 125: 946–9.
  38. Behrmann I., Walczak H., Krammer P.H. Structure of the human APO-1 gene. Eur. J. Immunol. 1994; 24(12): 3057–62.
  39. Infante A.J., Britton H.A., DeNapoli T. et al. The clinical spectrum in a large kindred with autoimmune lymphoproliferative syndrome due to a Fas mutation that impairs lymphocyte apoptosis. J. Pediatr. 1998; 133(5): 629–33.
  40. Huang B., Eberstadt M., Olejniczak E.T. et al. NMR structure and mutagenesis of the Fas (APO-1/CD95) death domain. Nature 1996; 384(6610): 638–41.
  41. Fisher G.H., Rosenberg F.J., Straus S.E. et al. Dominant interfering Fas gene mutations impair apoptosis in a human autoimmune lymphoproliferative syndrome. Cell 1995; 81(6): 935–46.
  42. Drappa J., Vaishnaw A.K., Sullivan K.E. et al. Fas gene mutations in the Canale-Smith syndrome, an inherited lymphoproliferative disorder associated with autoimmunity. N. Engl. J. Med. 1996; 335(22): 1643–9.
  43. Izeradjene K., Quemeneur L., Michallet M.C. et al. Mycophenolate mofetil interferes with interferon gamma production in T-cell activation models. Transplant. Proc. 2001; 33(3): 2110–1.
  44. Teachey D.T., Greiner R., Seif A. et al. Treatment with sirolimus results in complete responses in patients with autoimmune lymphoproliferative syndrome. Br. J. Haematol. 2009; 145(1): 101–6.
  45. Hsieh A.C., Liu Yi., Edlind M.P. et al. The translational landscape of mTOR signalling steers cancer initiation and metastasis. Nature 2012; 485: 55–61.
  46. Hartford C.M., Ratain M.J. Rapamycin: something old, something new, sometimes borrowed and now renewed. Clin. Pharmacol. Ther. 2007; 82(4): 381–8.
  47. Abdel-Karim I.A., Giles F.J. Mammalian target of rapamycin as a target in hematological malignancies. Curr. Probl. Cancer 2008; 32(4): 161–77.
  48. Brown V.I., Fang J., Alcorn K. et al. Rapamycin is active against B-precursor leukemia in vitro and in vivo, an effect that is modulated by IL-7-mediated signaling. Proc. Natl. Acad. Sci. USA 2003; 100(25): 15113–8.
  49. Fernandez D., Bonilla E., Mirza N., Niland B., Perl A. Rapamycin reduces disease activity and normalizes T cell activation-induced calcium fluxing in patients with systemic lupus erythematosus. Arthritis Rheum. 2006; 54(9): 2983–8.
  50. Bruyn G.A., Tate G., Caeiro F. et al.; RADD Study Group. Everolimus in patients with rheumatoid arthritis receiving concomitant methotrexate: a 3-month, double-blind, randomised; placebo-controlled; parallel-group; proofof-concept study. Ann. Rheum. Dis. 2008; 67(8): 1090–5.
  51. Bobe P., Bonardelle D., Benihoud K. et al. Arsenic trioxide: A promising novel therapeutic agent for lymphoproliferative and autoimmune syndromes in MRL/lpr mice. Blood 2006; 108(13): 3967–75.
  52. Dowdell K.C., Pesnicak L., Hoffmann V. et al. Valproic acid (VPA), a histone deacetylase (HDAC) inhibitor, diminishes lymphoproliferation in the Fas deficient MRL/lpr–/– murine model of autoimmune lymphoproliferative syndrome (ALPS). Blood (ASH Annual. Meeting Abstracts) 2006; 108: 2497.
  53. Saouaf S.J., Li B., Zhang G., Shen Y. et al. Deacetylase inhibition increases regulatory T cell function and decreases incidence and severity of collagen-induced arthritis. Exp. Mol. Pathol. 2009; 87(2): 99–104.
  54. Cooper N., Davies E.G., Thrasher A.J. Repeated courses of rituximab for autoimmune cytopenias may precipitate profound hypogammaglobulinaemia requiring replacement intravenous immunoglobulin. Br. J. Haematol. 2009; 146(1): 120–2.
  55. Rao V.K., Price S., Perkins K. et al. Use of rituximab for refractory cytopenias associated with autoimmune lymphoproliferative syndrome (ALPS). Pediatr. Blood Cancer 2009; 52(7): 847–52.
  56. Bleesing J.J., Straus S.E., Fleisher T.A. Autoimmune lymphoproliferative syndrome. A human disorder of abnormal lymphocyte survival. Pediatr. Clin. N. Am. 2000; 47(6): 1291–310.
  57. Cohen J.M., Sebire N.J., Harvey J. et al. Successful treatment of lymphoproliferative disease complicating primary immunodeficiency/immunodysregulatory disorders with reduced-intensity allogeneic stem-cell transplantation. Blood 2007; 110(6): 2209–14.
  58. Kahwash S.B., Fung B., Savelli S. et al. Autoimmune lymphoproliferative syndrome (ALPS): a case with congenital onset. Pediatr. Dev. Pathol. 2007; 10(4): 315–9.