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Published in: International Journal of Colorectal Disease 2/2020

01-02-2020 | Lymphoma | Case Report

Challenges in the management of life-threatening complications caused by a rare case of sarcoid-lymphoma syndrome

Authors: Stephanie S. Taha-Mehlitz, Irin I. Zschokke, Jürg J. Metzger, Lana L. Fourie

Published in: International Journal of Colorectal Disease | Issue 2/2020

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Abstract

Purpose

The sarcoid-lymphoma syndrome is a rare condition where both entities co-exist in the same patient. Overlapping clinical manifestations, imaging findings, and pathological characteristics pose both a diagnostic as well as therapeutic challenge, especially in the setting of life-threatening complications. The objective of this case report is to highlight rare complications caused by abdominal sarcoidosis as well as a subsequently diagnosed non-Hodgkin lymphoma.

Methods

A 35-year-old man presented with massive upper gastrointestinal bleeding caused by a large retroperitoneal mass. Following several endoscopic and radiologic interventions, successful hemostasis could only be achieved by an emergency Whipple procedure. Biopsies of the mass showed an abdominal manifestation of sarcoidosis and corticosteroid therapy was started. The patient required several additional emergency surgeries due to a complicated bronchoperitoneal fistula, splenic abscess, perforation of the sigmoid colon, small bowel leakage, and repetitive intra-abdominal bleeding.

Results

Histopathological findings finally revealed a concurrent lymphoma, as clinically suspected earlier. Ultimately, polychemotherapy was administered.

Conclusion

A concurrent lymphoma can lead to emergency surgery and should be considered when sarcoidosis treatment does not improve symptoms, lymphadenopathy occurs, or hematologic changes persist under therapy.
Literature
1.
go back to reference Brincker H (1986) The sarcoidosis-lymphoma syndrome. Br J Cancer 54(3):467–473CrossRef Brincker H (1986) The sarcoidosis-lymphoma syndrome. Br J Cancer 54(3):467–473CrossRef
2.
3.
go back to reference Karakantza M, Matutes E, MacLennan K, O’Connor NT, Srivastava PC, Catovsky D (1996) Association between sarcoidosis and lymphoma revisited. J Clin Pathol 49(3):208–212CrossRef Karakantza M, Matutes E, MacLennan K, O’Connor NT, Srivastava PC, Catovsky D (1996) Association between sarcoidosis and lymphoma revisited. J Clin Pathol 49(3):208–212CrossRef
4.
go back to reference Kornacker M, Kraemer A, Leo E, Ho AD (2002) Occurrence of sarcoidosis subsequent to chemotherapy for non-Hodgkin’s lymphoma: report of two cases. Ann Hematol 81(2):103–105CrossRef Kornacker M, Kraemer A, Leo E, Ho AD (2002) Occurrence of sarcoidosis subsequent to chemotherapy for non-Hodgkin’s lymphoma: report of two cases. Ann Hematol 81(2):103–105CrossRef
5.
go back to reference Iwai K, Takemura T, Kitaichi M, Kawabata Y, Matsui Y (1993) Pathological studies on sarcoidosis autopsy. II. Early change, mode of progression and death pattern. Acta Pathol Jpn 43(7–8):377–385PubMed Iwai K, Takemura T, Kitaichi M, Kawabata Y, Matsui Y (1993) Pathological studies on sarcoidosis autopsy. II. Early change, mode of progression and death pattern. Acta Pathol Jpn 43(7–8):377–385PubMed
6.
go back to reference Brincker H, Wilbek E (1974) The incidence of malignant tumours in patients with respiratory sarcoidosis. Br J Cancer 29(3):247–251CrossRef Brincker H, Wilbek E (1974) The incidence of malignant tumours in patients with respiratory sarcoidosis. Br J Cancer 29(3):247–251CrossRef
7.
go back to reference Bonifazi M, Bravi F, Gasparini S, La Vecchia C, Gabrielli A, Wells AU, Renzoni EA (2015) Sarcoidosis and cancer risk: systematic review and meta-analysis of observational studies. Chest 147(3):778–791CrossRef Bonifazi M, Bravi F, Gasparini S, La Vecchia C, Gabrielli A, Wells AU, Renzoni EA (2015) Sarcoidosis and cancer risk: systematic review and meta-analysis of observational studies. Chest 147(3):778–791CrossRef
8.
go back to reference Fujimura T, Kambayashi Y, Aiba S (2013) Expression of CD39/Entpd1 on granuloma-composing cells and induction of Foxp3-positive regulatory T cells in sarcoidosis. Clin Exp Dermatol 38(8):883–889CrossRef Fujimura T, Kambayashi Y, Aiba S (2013) Expression of CD39/Entpd1 on granuloma-composing cells and induction of Foxp3-positive regulatory T cells in sarcoidosis. Clin Exp Dermatol 38(8):883–889CrossRef
9.
go back to reference Fujimura T, Kakizaki A, Furudate S, Kambayashi Y, Aiba S (2016) Tumor-associated macrophages in skin: how to treat their heterogeneity and plasticity. J Dermatol Sci 83(3):167–173CrossRef Fujimura T, Kakizaki A, Furudate S, Kambayashi Y, Aiba S (2016) Tumor-associated macrophages in skin: how to treat their heterogeneity and plasticity. J Dermatol Sci 83(3):167–173CrossRef
10.
go back to reference Suen JS, Forse MS, Hyland RH, Chan CK (1990) The malignancy-sarcoidosis syndrome. Chest 98(5):1300–1302CrossRef Suen JS, Forse MS, Hyland RH, Chan CK (1990) The malignancy-sarcoidosis syndrome. Chest 98(5):1300–1302CrossRef
11.
go back to reference London J, Grados A, Ferme C, Charmillon A, Maurier F, Deau B, Crickx E, Brice P, Chapelon-Abric C, Haioun C, Burroni B, Alifano M, Le Jeunne C, Guillevin L, Costedoat-Chalumeau N, Schleinitz N, Mouthon L, Terrier B (2014) Sarcoidosis occurring after lymphoma: report of 14 patients and review of the literature. Medicine (Baltimore) 93(21):e121CrossRef London J, Grados A, Ferme C, Charmillon A, Maurier F, Deau B, Crickx E, Brice P, Chapelon-Abric C, Haioun C, Burroni B, Alifano M, Le Jeunne C, Guillevin L, Costedoat-Chalumeau N, Schleinitz N, Mouthon L, Terrier B (2014) Sarcoidosis occurring after lymphoma: report of 14 patients and review of the literature. Medicine (Baltimore) 93(21):e121CrossRef
12.
go back to reference Lieberman J (1976) The specificity and nature of serum-angiotensin-converting enzyme (serum ACE) elevations in sarcoidosis. Ann N Y Acad Sci 278:488–497CrossRef Lieberman J (1976) The specificity and nature of serum-angiotensin-converting enzyme (serum ACE) elevations in sarcoidosis. Ann N Y Acad Sci 278:488–497CrossRef
13.
go back to reference Kis A, Eszes N, Tamasi L, Losonczy G, Csekeo A, Csomor J, Muller V (2013) Sarcoidosis lymphoma syndrome - the value of PET-CT in the diagnosis. World J Surg Oncol 11:235CrossRef Kis A, Eszes N, Tamasi L, Losonczy G, Csekeo A, Csomor J, Muller V (2013) Sarcoidosis lymphoma syndrome - the value of PET-CT in the diagnosis. World J Surg Oncol 11:235CrossRef
Metadata
Title
Challenges in the management of life-threatening complications caused by a rare case of sarcoid-lymphoma syndrome
Authors
Stephanie S. Taha-Mehlitz
Irin I. Zschokke
Jürg J. Metzger
Lana L. Fourie
Publication date
01-02-2020
Publisher
Springer Berlin Heidelberg
Published in
International Journal of Colorectal Disease / Issue 2/2020
Print ISSN: 0179-1958
Electronic ISSN: 1432-1262
DOI
https://doi.org/10.1007/s00384-019-03484-y

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