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CC BY 4.0 · Endoscopy 2023; 55(S 01): E854-E855
DOI: 10.1055/a-2098-0883
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Small-bowel B-cell lymphoma presenting as autoimmune hemolytic anemia and severe obscure gastrointestinal bleeding

Authors

  • Katarzyna M. Pawlak 

    1   Department of Gastroenterology, Hospital of the Ministry of Internal Affairs, Szczecin, Poland

  • Alvaro Martínez-Alcalá 

    2   Department of Gastroenterology, Helios Frankenwaldklinik, Kronach, Germany

  • Paul Thomas Kröner 

    3   Department of Gastroenterology, Ameos Teaching University Hospital (Otto-von-Guericke University – Magdeburg), Halberstadt, Germany

  • Lucía C. Fry

    2   Department of Gastroenterology, Helios Frankenwaldklinik, Kronach, Germany

  • Klaus Mönkemüller

    2   Department of Gastroenterology, Helios Frankenwaldklinik, Kronach, Germany
    3   Department of Gastroenterology, Ameos Teaching University Hospital (Otto-von-Guericke University – Magdeburg), Halberstadt, Germany
    4   University of Belgrade, Belgrade, Serbia
    5   Department of Gastroenterology “Prof. Carolina Olano”, Hospital de Clínicas, Universidad de la República, Montevideo, Uruguay
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A 75-year-old man was admitted to our hospital because of worsening chronic hemolytic anemia and hematochezia. His medical history was significant for diabetes mellitus, coronary artery disease, and autoimmune hemolytic anemia (AIHA). Idiopathic AIHA was diagnosed 3 years prior to the current admission and was warm antibody-mediated, and direct antiglobulin test (Coombs test) positive. Initially he was placed on steroids, but due to recurrent hemolytic episodes, he was subsequently placed on different immunosuppressive agents, including azathioprine, cyclophosphamide, and mycophenolate mofetil.

Esophagogastroduodenoscopy and colonoscopy were unremarkable. Capsule endoscopy showed an ulcerated tumor located in the ileum ([Fig. 1 a]). A retrograde deep enteroscopy confirmed a large tumor in the ileum ([Fig. 1 b]). The patient underwent surgical resection of the mass ([Fig. 1 c]). Histopathology revealed diffuse large B-cell lymphoma (DLBCL), an aggressive subtype of non-Hodgkin lymphoma (NHL) ([Fig. 1 d–f], [Video 1]).

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Fig. 1 Patient investigations. a Capsule endoscopy showing an ulcerated mass. b On enteroscopy, a stenotic, ulcerated, and bleeding mass was evident. c Operative photograph showing a large mass involving the terminal ileum. d Hematoxylin and eosin stain revealed neoplastic lymphoid cells. e The CD-20 marker confirmed the B-cell origin of the lymphoma. f Positive staining for Ki-67 in more than 90 % of the cells.

Video 1 Small-bowel B-cell lymphoma presenting as autoimmune hemolytic anemia and severe obscure gastrointestinal bleeding.

The Coombs test was negative 2 months later, suggesting that the mass was the source of the autoantibodies. Combination R-CHOP therapy (rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and prednisone) resulted in remission of the lymphoma.

This case highlights the occurrence of two conditions leading to severe anemia. Whenever an AIHA is present, the clinician must be aware of lymphomas. Interestingly, AIHA may occur prior to NHL (from 3 months to 13 years before lymphoma diagnosis), concurrent with (6 months before until 6 months after NHL diagnosis), at relapse of NHL, or in complete remission after successful treatment of the NHL; however, the majority of cases occur concurrently [1] [2] [3]. The most common primary small intestine lymphoma is DLBCL (58 % of cases) and perforation is a frequent complication. Endoscopically, AIHA can be of polypoid type in 25 % of cases, ulcerative type in 54 %, multiple polyposis in 5 %, diffuse-infiltrating type in 6 %, and mixed type in 10 % of patients [2] [3].

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Competing interests

The authors declare that they have no conflict of interest.


Corresponding author

Klaus Mönkemüller, FJGES (Japan)
Division of Gastroenterology
University Teaching Hospital – Ameos Klinikum Halberstadt
Gleimstraße 5
38820 Halberstadt
Germany   

Publication History

Article published online:
11 July 2023

© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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Zoom
Fig. 1 Patient investigations. a Capsule endoscopy showing an ulcerated mass. b On enteroscopy, a stenotic, ulcerated, and bleeding mass was evident. c Operative photograph showing a large mass involving the terminal ileum. d Hematoxylin and eosin stain revealed neoplastic lymphoid cells. e The CD-20 marker confirmed the B-cell origin of the lymphoma. f Positive staining for Ki-67 in more than 90 % of the cells.