Eculizumab-Responsive Adult Onset Protein Losing Enteropathy, Caused by Germline CD55-Deficiency and Complicated by Aggressive Angiosarcoma

David Hagin^#¹, Dror Lahav^#², Tal Freund³, Sivan Shamai⁴, Eli Brazowski⁵, Sigal Fishman⁶, Alina Kurolap⁷, Hagit Baris Feldman⁸, Mordechai Shohat⁹, Ophira Salomon¹⁰

Affiliations

¹ Allergy and Clinical Immunology Unit, Department of Medicine, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine,, University of Tel Aviv, 6 Weizmann St, 64239, Tel Aviv, Israel. Davidha@tlvmc.gov.il.
² Internal Medicine Consultations, Internal Medicine Division, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel.
³ Allergy and Clinical Immunology Unit, Department of Medicine, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine,, University of Tel Aviv, 6 Weizmann St, 64239, Tel Aviv, Israel.
⁴ Department of Oncology, Department of Medicine, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel.
⁵ The Institute of Pathology, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel.
⁶ The Gastroenterology Institute, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel.
⁷ The Genetics Institute, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
⁸ The Genetics Institute, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
⁹ The Genomic Unit, Sheba Cancer Research Center, Sheba Medical Center, and Sackler Faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel.
¹⁰ Institute of Thrombosis and Hemostasis, Sheba Medical Center, and Sackler Faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel.

^# Contributed equally.

PMID: 33215321
DOI: 10.1007/s10875-020-00910-7

Case Reports

Eculizumab-Responsive Adult Onset Protein Losing Enteropathy, Caused by Germline CD55-Deficiency and Complicated by Aggressive Angiosarcoma

David Hagin et al. J Clin Immunol. 2021 Feb.

. 2021 Feb;41(2):477-481.

doi: 10.1007/s10875-020-00910-7. Epub 2020 Nov 19.

Authors

David Hagin^#¹, Dror Lahav^#², Tal Freund³, Sivan Shamai⁴, Eli Brazowski⁵, Sigal Fishman⁶, Alina Kurolap⁷, Hagit Baris Feldman⁸, Mordechai Shohat⁹, Ophira Salomon¹⁰

Affiliations

¹ Allergy and Clinical Immunology Unit, Department of Medicine, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine,, University of Tel Aviv, 6 Weizmann St, 64239, Tel Aviv, Israel. Davidha@tlvmc.gov.il.
² Internal Medicine Consultations, Internal Medicine Division, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel.
³ Allergy and Clinical Immunology Unit, Department of Medicine, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine,, University of Tel Aviv, 6 Weizmann St, 64239, Tel Aviv, Israel.
⁴ Department of Oncology, Department of Medicine, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel.
⁵ The Institute of Pathology, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel.
⁶ The Gastroenterology Institute, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel.
⁷ The Genetics Institute, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
⁸ The Genetics Institute, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
⁹ The Genomic Unit, Sheba Cancer Research Center, Sheba Medical Center, and Sackler Faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel.
¹⁰ Institute of Thrombosis and Hemostasis, Sheba Medical Center, and Sackler Faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel.

^# Contributed equally.

PMID: 33215321
DOI: 10.1007/s10875-020-00910-7

No abstract available

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References

1. Ozen A, Comrie WA, Ardy RC, Dominguez Conde C, Dalgic B, Beser OF, et al. CD55 deficiency, early-onset protein-losing enteropathy, and thrombosis. N Engl J Med. 2017;377(1):52–61. - DOI
1. Kurolap A, Eshach-Adiv O, Hershkovitz T, Paperna T, Mory A, Oz-Levi D, et al. Loss of CD55 in eculizumab-responsive protein-losing enteropathy. N Engl J Med. 2017;377(1):87–9. - DOI
1. Kurolap A, Eshach Adiv O, Hershkovitz T, Tabib A, Karbian N, Paperna T, et al. Eculizumab is safe and effective as a long-term treatment for protein-losing enteropathy due to CD55 deficiency. J Pediatr Gastroenterol Nutr. 2019;68(3):325–33. - DOI
1. Wilson GC, Lluis N, Nalesnik MA, Nassar A, Serrano T, Ramos E, et al. Hepatic angiosarcoma: a multi-institutional, international experience with 44 cases. Ann Surg Oncol. 2019;26(2):576–82. - DOI
1. Vikkula M, Boon LM, Carraway KL, Calvert JT, Diamonti AJ, Goumnerov B, et al. Vascular dysmorphogenesis caused by an activating mutation in the receptor tyrosine kinase TIE2. Cell. 1996;87(7):1181–90. - DOI

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Eculizumab-Responsive Adult Onset Protein Losing Enteropathy, Caused by Germline CD55-Deficiency and Complicated by Aggressive Angiosarcoma

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Eculizumab-Responsive Adult Onset Protein Losing Enteropathy, Caused by Germline CD55-Deficiency and Complicated by Aggressive Angiosarcoma

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