Mini Derleme: Soğuk Aglütinin Hastalığı

Kırcalı, Ekin; Toprak, Selami Kocak; Topcuoglu, Pervin

doi:10.5578/llm.20239701

Mini Derleme: Soğuk Aglütinin Hastalığı

Ekin KIRCALI, (Atatürk Sanatoryum Eğitim ve Araştırma Hastanesi, Hematoloji Kliniği, Ankara, Türkiye)

Selami Koçak TOPRAK, (Ankara Üniversitesi Tıp Fakültesi, İç Hastalıkları Anabilim Dalı, Hematoloji Bilim Dalı, Ankara, Türkiye)

Pervin TOPÇUOĞLU (Ankara Üniversitesi Tıp Fakültesi, İç Hastalıkları Anabilim Dalı, Hematoloji Bilim Dalı, Ankara, Türkiye)

Türkiye'de Lösemi Lenfoma Miyelom Araştırmaları

113 12

Yıl: 2023 Cilt: 7 Sayı: 3 Sayfa Aralığı: 87 - 91 Metin Dili: Türkçe DOI: 10.5578/llm.20239701 İndeks Tarihi: 02-11-2023

Mini Derleme: Soğuk Aglütinin Hastalığı

Öz:

Soğuk aglütinin hastalığı (SAH), eritrosit I/i antijenlerine karşı oluşan Ig M tipindeki antikorların, soğukta bağlanması nedeniyle görülen otoimmün bir hemolitik anemidir. SAH vücutta parmak uçları, kulak ve burun ucu gibi soğuk bölgelerde eritrosit aglütinasyonuna ve hemolitik anemiye bağlı semptomlara sebep olabilir. Altta yatan malign ya da benign bir hastalık olmadığında birincil SAH adını alır; oldukça nadirdir ve toplumda görülme sıklığı 16/1.000.000 olarak bildirilmiştir. Altta yatan enfeksiyonlara, otoimmün hastalıklara ya da lenfoproliferatif hastalıklara bağlı gelişirse hastalığa ikincil soğuk aglütinin sendromu (SAS) denmektedir. Hastalığın tanısında hemoglobin/hematokrit uyumsuzluğu, çevre kanı yayması ve soğuk aglütinin varlığının gösterilmesi yer alır. Tedavide, semptomları ve hemolizi azaltmak için soğuktan kaçınmak önemlidir. Antikor üretimini azaltmada en etkili ilaçlardan biri rituksimabtır ve rituksimab,tek başına ya da bendamustinle, steroidlerle, interferonla, fludarabinle birlikte uygulanabilir. Ağır hemoliz, acil tedavi ihtiyacı gibi durumlarda da intravenöz immünglobulinler, plazmaferez gündeme gelebilir. SAS’de tedavi, altta yatan sebebin tedavisidir.

Anahtar Kelime: Soğuk aglütinin hastalığı hemoliz lenfoma anemi

A Mini Review on Cold Agglutinin Disease

Öz:

Cold agglutinin disease (CAD) is an autoimmune hemolytic anemia that occurs due to the binding of IgM type antibodies to erythrocyte I/i antigens at colder temperatures. CAD may cause symptoms related to erythrocyte agglutination and hemolytic anemia in colder parts of the body such as fingertips, ear and nose tip. When no underlying malignant or benign disease is present, it is called primary CAD, which is quite rare. Prevalence of primary CAD has been reported to be 16/1.000.000. If CAD occurs due to underlying infections, autoimmune diseases or lymphoproliferative disorders, then it is called secondary cold agglutinin syndrome (CAS). Diagnosis of CAD requires hemoglobin/hematocrit discordance, eryhtrocyte agglutinins on peripheral blood smear, and the presence of cold agglutinin. It is important to avoid cold to reduce symptoms and hemolysis. One of the most effective drugs in reducing antibody production is rituximab, which can be administered alone or in combination with bendamustine, steroids, interferon, and fludarabine. In cases with severe hemolysis and need for urgent treatment, intravenous immunoglobulins, sutimlimab and plasmapheresis may be considered. Treatment of CAS consists of the underlying cause.

Anahtar Kelime: Cold agglutinin disease hemolysis lymphoma anemia

Belge Türü: Makale Makale Türü: Derleme Erişim Türü: Erişime Açık

1. Berentsen S. How I manage patients with cold agglutinin disease. Br J Haematol 2018;181(3):320-30. https://doi.org/10.1111/ bjh.15109
2. Zilow G, Kirschfink M, Roelcke D. Red cell destruction in cold agglutinin disease. Infusionsther Transfusionsmed 1994;21(6):410- 5. https://doi.org/10.1159/000223021
3. Berentsen S, Ulvestad E, Langholm R, Beiske K, Hjorth-Hansen H, Ghanima W, et al. Primary chronic cold agglutinin disease: A population based clinical study of 86 patients. Haematologica 2006;91(4):460-6.
4. García-Sanz R, Montoto S, Torrequebrada A, de Coca AG, Petit J, Sureda A, et al. Waldenström macroglobulinaemia: Presenting features and outcome in a series with 217 cases. Br J Haematol 2001;115(3):575-82. https://doi.org/10.1046/j.1365- 2141.2001.03144.x
5. Lind K, Benzon MW, Jensen JS, Clyde WA Jr. A seroepidemiological study of Mycoplasma pneumoniae infections in Denmark over the 50-year period 1946-1995. Eur J Epidemiol 1997;13(5):581-6. https://doi.org/10.1023/A:1007353121693
6. Karunarathne S, Weerasinghe S, Govindapala D, Fernando H, Jayaratne B. Cold autoimmune haemolytic anaemia secondary to Epstein Barr virus infection presenting with peripheral gangrene; case report. Thromb J 2012;10(1):4. https://doi. org/10.1186/1477-9560-10-4
7. Dourakis SP, Alexopoulou A, Stamoulis N, Foutris A, Pandelidaki H, Archimandritis AJ. Acute Epstein-Barr virus infection in two elderly individuals. Age Ageing 2006;35(2):196-8. https://doi. org/10.1093/ageing/afj021
8. Nixon CP, Sweeney JD. Facilitation of the clinical diagnosis of Mycoplasma pneumoniae by the blood bank. Transfusion 2017;57(11):2564. https://doi.org/10.1111/trf.14115
9. Khan FY, A yassin M. Mycoplasma pneumoniae associated with severe autoimmune hemolytic anemia: Case report and literature review. Braz J Infect Dis 2009;13(1):77-9. https://doi. org/10.1590/S1413-86702009000100018
10. Terada K, Tanaka H, Mori R, Kataoka N, Uchikawa M. Hemolytic anemia associated with cold agglutinin during chickenpox and a review of the literature. J Pediatr Hematol Oncol 1998;20(2):149- 51. https://doi.org/10.1097/00043426-199803000-00012
11. Kumar A, Shaaban H, Koduru K, Abo S, Sidhom I, Guron G. Citrobacter freundii-induced cold agglutinin hemolysis. Ann Hematol 2011;90(7):855-6. https://doi.org/10.1007/s00277-010-1096-9
12. Schoindre Y, Bollée G, Dumont MD, Lesavre P, Servais A. Cold agglutinin syndrome associated with a 2009 influenza A H1N1 infection. Am J Med 2011;124(2):e1-2. https://doi.org/10.1016/j. amjmed.2010.05.015
13. Oshima M, Maeda H, Morimoto K, Doi M, Kuwabara M. Low-titer cold agglutinin disease with systemic sclerosis. Intern Med 2004;43(2):139-42. https://doi.org/10.2169/internalmedicine.43.139
14. Kotani T, Takeuchi T, Kawasaki Y, Hirano S, Tabushi Y, Kagitani M, et al. Successful treatment of cold agglutinin disease with anti-CD20 antibody (rituximab) in a patient with systemic lupus erythematosus. Lupus 2006;15(10):683-5. https://doi. org/10.1177/0961203306070983
15. Srinivasan N, Oswal A, Garg S, Nahar J, Gosmonova A, Nahar R. Cold agglutinin induced hemolysis in a newly diagnosed systemic lupus erythematosus. Am J Med Sci 2010;339(3):270-3. https://doi.org/10.1097/MAJ.0b013e3181ac3bd5
16. Honne K, Nagashima T, Iwamoto M, Kamesaki T, Minota S. Glucocorticoid-responsive cold agglutinin disease in a patient with rheumatoid arthritis. Case Rep Rheumatol 2015;2015:823563. https://doi.org/10.1155/2015/823563
17. Crisp D, Pruzanski W. B-cell neoplasms with homogeneous cold-reacting antibodies (cold agglutinins). Am J Med 1982;72(6):915-22. https://doi.org/10.1016/0002- 9343(82)90852-X
18. Swiecicki PL, Hegerova LT, Gertz MA. Cold agglutinin disease. Blood 2013;122(7):1114-21. https://doi.org/10.1182/ blood-2013-02-474437
19. Hansen DL, Berentsen S, Fattizzo B, Hansen PL, Barcellini W, Frederiksen H. Seasonal variation in the incidence of cold agglutinin disease in Norway, Denmark, and Italy. Am J Hematol 2021;96(7):E262-E265. https://doi.org/10.1002/ajh.26196
20. Brugnara, C. and S.J.U.W. Berentsen, MA: UpToDate, Cold agglutinin disease. 2021.
21. Berentsen S, Barcellini W, D’Sa S, Randen U, Tvedt THA, Fattizzo B, et al. Cold agglutinin disease revisited: a multinational, observational study of 232 patients. Blood 2020;136(4):480-8. https:// doi.org/10.1182/blood.2020005674
22. Lyckholm LJ, Edmond MB. Images in clinical medicine. Seasonal hemolysis due to cold-agglutinin syndrome. N Engl J Med 1996;334(7):437. https://doi.org/10.1056/ NEJM199602153340705
23. Bylsma LC, Gulbech Ording A, Rosenthal A, Öztürk B, Fryzek JP, Arias JM, et al. Occurrence, thromboembolic risk, and mortality in Danish patients with cold agglutinin disease. Blood Adv 2019;3(20):2980-5. https://doi.org/10.1182/bloodadvances.2019000476
24. Broome CM, Cunningham JM, Mullins M, Jiang X, Bylsma LC, Fryzek JP, et al. Increased risk of thrombotic events in cold agglutinin disease: A 10-year retrospective analysis. Res Pract Thromb Haemost 2020;4(4):628-35. https://doi.org/10.1002/rth2.12333
25. Hill QA, Stamps R, Massey E, Grainger JD, Provan D, Hill A; British Society for Haematology. The diagnosis and management of primary autoimmune haemolytic anaemia. Br J Haematol 2017;176(3):395-411. https://doi.org/10.1111/bjh.14478
26. Röth A, Barcellini W, D’Sa S, Miyakawa Y, Broome CM, Michel M, et al. Sutimlimab in cold agglutinin disease. N Engl J Med 2021;384(14):1323-34. https://doi.org/10.1056/NEJMoa2027760
27. Berentsen S. Sutimlimab for the treatment of cold agglutinin disease. Hemasphere 2023;7(5):e879. https://doi.org/10.1097/ HS9.0000000000000879
28. Berentsen S, Ulvestad E, Gjertsen BT, Hjorth-Hansen H, Langholm R, Knutsen H, et al. Rituximab for primary chronic cold agglutinin disease: A prospective study of 37 courses of therapy in 27 patients. Blood 2004;103(8):2925-8. https://doi.org/10.1182/ blood-2003-10-3597
29. Berentsen S, Randen U, Vågan AM, Hjorth-Hansen H, Vik A, Dalgaard J, et al. High response rate and durable remissions following fludarabine and rituximab combination therapy for chronic cold agglutinin disease. Blood 2010;116(17):3180-4. https://doi.org/10.1182/blood-2010-06-288647
30. Berentsen S, Barcellini W. Autoimmune hemolytic anemias. N Engl J Med 2021;385(15):1407-19. https://doi.org/10.1056/NEJMra2033982
31. Rossi G, Gramegna D, Paoloni F, Fattizzo B, Binda F, D’Adda M, et al. Short course of bortezomib in anemic patients with relapsed cold agglutinin disease: A phase 2 prospective GIMEMA study. Blood 2018;132(5):547-50. https://doi.org/10.1182/ blood-2018-03-835413
32. Tomkins O, Berentsen S, Arulogun S, Sekhar M, D’Sa S. Daratumumab for disabling cold agglutinin disease refractory to B-cell directed therapy. Am J Hematol 2020. https://doi.org/10.1002/ ajh.25932
33. Zaninoni A, Giannotta JA, Gallì A, Artuso R, Bianchi P, Malcovati L, et al. The immunomodulatory effect and clinical efficacy of daratumumab in a patient with cold agglutinin disease. Front Immunol 2021;12:649441. https://doi.org/10.3389/fimmu.2021.649441

APA	Kırcalı E, Toprak S, Topcuoglu P (2023). Mini Derleme: Soğuk Aglütinin Hastalığı. , 87 - 91. 10.5578/llm.20239701
Chicago	Kırcalı Ekin,Toprak Selami Kocak,Topcuoglu Pervin Mini Derleme: Soğuk Aglütinin Hastalığı. (2023): 87 - 91. 10.5578/llm.20239701
MLA	Kırcalı Ekin,Toprak Selami Kocak,Topcuoglu Pervin Mini Derleme: Soğuk Aglütinin Hastalığı. , 2023, ss.87 - 91. 10.5578/llm.20239701
AMA	Kırcalı E,Toprak S,Topcuoglu P Mini Derleme: Soğuk Aglütinin Hastalığı. . 2023; 87 - 91. 10.5578/llm.20239701
Vancouver	Kırcalı E,Toprak S,Topcuoglu P Mini Derleme: Soğuk Aglütinin Hastalığı. . 2023; 87 - 91. 10.5578/llm.20239701
IEEE	Kırcalı E,Toprak S,Topcuoglu P "Mini Derleme: Soğuk Aglütinin Hastalığı." , ss.87 - 91, 2023. 10.5578/llm.20239701
ISNAD	Kırcalı, Ekin vd. "Mini Derleme: Soğuk Aglütinin Hastalığı". (2023), 87-91. https://doi.org/10.5578/llm.20239701

APA	Kırcalı E, Toprak S, Topcuoglu P (2023). Mini Derleme: Soğuk Aglütinin Hastalığı. Türkiye'de Lösemi Lenfoma Miyelom Araştırmaları, 7(3), 87 - 91. 10.5578/llm.20239701
Chicago	Kırcalı Ekin,Toprak Selami Kocak,Topcuoglu Pervin Mini Derleme: Soğuk Aglütinin Hastalığı. Türkiye'de Lösemi Lenfoma Miyelom Araştırmaları 7, no.3 (2023): 87 - 91. 10.5578/llm.20239701
MLA	Kırcalı Ekin,Toprak Selami Kocak,Topcuoglu Pervin Mini Derleme: Soğuk Aglütinin Hastalığı. Türkiye'de Lösemi Lenfoma Miyelom Araştırmaları, vol.7, no.3, 2023, ss.87 - 91. 10.5578/llm.20239701
AMA	Kırcalı E,Toprak S,Topcuoglu P Mini Derleme: Soğuk Aglütinin Hastalığı. Türkiye'de Lösemi Lenfoma Miyelom Araştırmaları. 2023; 7(3): 87 - 91. 10.5578/llm.20239701
Vancouver	Kırcalı E,Toprak S,Topcuoglu P Mini Derleme: Soğuk Aglütinin Hastalığı. Türkiye'de Lösemi Lenfoma Miyelom Araştırmaları. 2023; 7(3): 87 - 91. 10.5578/llm.20239701
IEEE	Kırcalı E,Toprak S,Topcuoglu P "Mini Derleme: Soğuk Aglütinin Hastalığı." Türkiye'de Lösemi Lenfoma Miyelom Araştırmaları, 7, ss.87 - 91, 2023. 10.5578/llm.20239701
ISNAD	Kırcalı, Ekin vd. "Mini Derleme: Soğuk Aglütinin Hastalığı". Türkiye'de Lösemi Lenfoma Miyelom Araştırmaları 7/3 (2023), 87-91. https://doi.org/10.5578/llm.20239701