Abstract
Aplastic anemia (AA) has been reported to be associated with inflammatory bowel disease (IBD), but mostly with ulcerative colitis (UC). Little is known about the associations between AA and Crohn’s disease (CD). We aim to determine the portraits of patients with AA-CD. Among a total of 657 patients with CD registered in Xijing Hospital of Digestive Diseases IBD center from January 2008 to October 2018, the patients diagnosed with concurrent AA were reviewed. Clinical presentation, medical history, endoscopic features, response to treatment, and prognosis in this set of patients were collected. Six male patients confirmed as CD associated with AA were identified. The incidence rate was 0.91% for CD associated with AA in our series. Average age at diagnosis of CD and AA was 41.5 and 39.2 years old, respectively. Abdominal pain and hyperpyrexia were the most common symptoms. Endoscopic findings showed discontinued severe inflammation, and all these patients presented with deformed ileocecal valve. Conventional pharmacotherapy failed to achieve a favorable effect. Four of six patients died from CD progression and its complications. None of these patients received bone marrow transplantation treatment because of poverty. Concurrence of AA and CD is a relatively rare condition. Immunologic impairment may play an important pathogenic role and deserves further attention. Males are more susceptible to this condition. Patients with AA-CD are prone to a severe clinical course and poor prognosis. Conventional therapy achieves no potent effect, and allogeneic stem cell transplantation may be a potentially efficient therapy.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Takaku T, Calado RT, Kajigaya S, Young NS (2009) Interleukin-23 receptor (IL-23R) gene polymorphisms in acquired aplastic anemia. Ann Hematol 88(7):653–657. https://doi.org/10.1007/s00277-008-0666-6
Ghavidel A (2013) Ulcerative colitis associated with aplastic anemia; a case report. Middle East J Dig Dis 5(4):230–234
Shimada T, Maeda T, Ishikawa M, Okamura D, Ito Y, Wakimoto N, Nakamura Y, Kawai N, Ino H, Jinnai I, Mori S, Matsuda A, Bessho M (2012) Aplastic anemia complicated with ulcerative colitis. [Rinsho ketsueki] Jpn J Clin Hematol 53(2):224–228
Kishikawa H, Nishida J, Nakano M, Hirano E, Morishita T, Ishii H (2003) Ulcerative colitis associated with aplastic anemia. Dig Dis Sci 48(7):1376–1379
Gomollon F, Dignass A, Annese V, Tilg H, Van Assche G, Lindsay JO, Peyrin-Biroulet L, Cullen GJ, Daperno M, Kucharzik T, Rieder F, Almer S, Armuzzi A, Harbord M, Langhorst J, Sans M, Chowers Y, Fiorino G, Juillerat P, Mantzaris GJ, Rizzello F, Vavricka S, Gionchetti P, Ecco (2017) 3rd European Evidence-based Consensus on the Diagnosis and Management of Crohn's Disease 2016: part 1: diagnosis and medical management. J Crohn's Colitis 11(1):3–25. https://doi.org/10.1093/ecco-jcc/jjw168
Killick SB, Bown N, Cavenagh J, Dokal I, Foukaneli T, Hill A, Hillmen P, Ireland R, Kulasekararaj A, Mufti G, Snowden JA, Samarasinghe S, Wood A, Marsh JC, British Society for Standards in H (2016) Guidelines for the diagnosis and management of adult aplastic anaemia. Br J Haematol 172(2):187–207. https://doi.org/10.1111/bjh.13853
Camitta BM, Thomas ED, Nathan DG, Santos G, Gordon-Smith EC, Gale RP, Rappeport JM, Storb R (1976) Severe aplastic anemia: a prospective study of the effect of early marrow transplantation on acute mortality. Blood 48(1):63–70
Otsubo H, Kaito K, Sekita T, Shimada T, Kobayashi M, Hosoya T (1998) Mesalazine-associated severe aplastic anemia successfully treated with antithymocyte globulin, cyclosporin and granulocyte colony-stimulating factor. Int J Hematol 68(4):445–448
Sharma BC, Yachha SK, Mishra RN, Gupta D (1996) Hypoplastic anemia associated with ulcerative colitis in a child. J Pediatr Gastroenterol Nutr 23(3):326–328
Naithani R, Mahapatra M, Kumar R, Rai S (2005) Aplastic anemia and Crohn's disease - coincidence or association? Indian J Gastroenterol 24(4):183
Mori M, Tanaka T, Akifuji Y, Ueki J, Nakamoto S, Uwai M, Terui Y, Tomizuka H, Hatake K, Ozawa K, Miura Y (1999) Moderate aplastic anemia associated with Crohn's disease during antithymocyte globulin treatment. [Rinsho ketsueki] Jpn J Clin Hematol 40(10):1105–1109
Tyndall A (1997) Hematopoietic stem cell transplantation in rheumatic diseases other than systemic sclerosis and systemic lupus erythematosus. J Rheumatol Suppl 48:94–97
Ikehara S (2002) Bone marrow transplantation: a new strategy for intractable diseases. Drugs Today 38(2):103–111
Tajiri H, Tomomasa T, Yoden A, Konno M, Sasaki M, Maisawa S, Sumazaki R, Shimizu T, Toyoda S, Etani Y, Nakacho M, Ushijima K, Kobayashi A (2008) Efficacy and safety of azathioprine and 6-mercaptopurine in Japanese pediatric patients with ulcerative colitis: a survey of the Japanese Society for Pediatric Inflammatory Bowel Disease. Digestion 77(3-4):150–154. https://doi.org/10.1159/000140974
Gremse DA, Bancroft J, Moyer MS (1989) Sulfasalazine hypersensitivity with hepatotoxicity, thrombocytopenia, and erythroid hypoplasia. J Pediatr Gastroenterol Nutr 9(2):261–263
Chow DK, Leong RW, Lai LH, Wong GL, Leung WK, Chan FK, Sung JJ (2008) Changes in Crohn's disease phenotype over time in the Chinese population: validation of the Montreal classification system. Inflamm Bowel Dis 14(4):536–541. https://doi.org/10.1002/ibd.20335
Group ACIW (2006) Retrospective analysis of 515 cases of Crohn's disease hospitalization in China: nationwide study from 1990 to 2003. J Gastroenterol Hepatol 21(6):1009–1015. https://doi.org/10.1111/j.1440-1746.2006.04140.x
Lopez-Cubero SO, Sullivan KM, McDonald GB (1998) Course of Crohn's disease after allogeneic marrow transplantation. Gastroenterology 114(3):433–440
Author information
Authors and Affiliations
Contributions
Song Su: Conception and design of the study, Acquisition of data, Analysis and interpretation of data, Drafting the article. ZhenZhen Liu: Acquisition of data, Analysis and interpretation of data, Drafting the article. Fang Wang: Acquisition of data, Analysis and interpretation of data. YuJie Zhang: Conception and design of the study, Revising the article. Yi Chu: Acquisition of data. MingZuo Jiang: Acquisition of data. Nan Wu: Acquisition of data, Analysis and interpretation of data. JunChao Lin: Acquisition of data. Bing Xu: Conception and design of the study, Revising the article. XianMin Xue: Acquisition of data. YongQuan Shi: Conception and design of the study, Analysis and interpretation of data. Shaoqi Yang: Revising the article. KaiChun Wu: Conception and design of the study, Analysis and interpretation of data. Jie Liang: Conception and design of the study, Analysis and interpretation of data, Revising the article
Corresponding authors
Ethics declarations
Conflict of interest
The authors declare that they have no conflict of interest.
Ethical approval
This is a retrospective observational study. This article does not contain any studies with human participants or animals performed by any of the authors.
Informed consent
Although this is a retrospective study, informed consent was obtained from all individual participants included in the study during the follow-up process.
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
National Natural Science Foundation of China (Nos. 81772650, 81322037, 81572302, 81421003), National Excellent Doctoral Dissertation of PR China (No. 201182).
Rights and permissions
About this article
Cite this article
Su, S., Liu, Z., Wang, F. et al. Aplastic anemia associated with Crohn’s disease: a tertiary center retrospective study. Ann Hematol 98, 2053–2061 (2019). https://doi.org/10.1007/s00277-019-03729-4
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00277-019-03729-4