Unusual Presentation of Non-Hodgkin’s Lymphoma Presenting as Anasarca and Protein-Losing Enteropathy: A Case Report

Leila Shafiq^1, , Andrew J Shychuk² and Joanna M Chaffin³

¹University of Florida College of Medicine, Gainesville, Florida, USA

²Department of Internal Medicine, University of Florida Health, Gainesville, Florida, USA

³Department of Pathology, University of Florida Health, Gainesville, Florida, USA

Cite this paper:
Leila Shafiq, Andrew J Shychuk and Joanna M Chaffin. Unusual Presentation of Non-Hodgkin’s Lymphoma Presenting as Anasarca and Protein-Losing Enteropathy: A Case Report. American Journal of Medical Case Reports. 2021; 9(12):743-747. doi: 10.12691/ajmcr-9-12-21

Abstract

Non-Hodgkin’s lymphomas (NHL) manifest in a multitude of presentations dependent on the subtype, aggressiveness, and primary location of the lymphoma. Because of varied clinical presentations, NHL can oftentimes be difficult to diagnose. However, a high index of suspicion and timely diagnosis is critical, especially in the case of aggressive NHL, in order to initiate prompt treatment for the most optimal outcomes. Here we describe the case of a 62 year-old Caucasian male who presented to a new primary care provider for further evaluation of edema, at which time he was found to have evidence of progression to anasarca along with evidence of protein-losing enteropathy. This occurred after initial workup for symptoms had been terminated prematurely by the previous provider with a move toward symptomatic management without establishing a diagnosis due diagnostic challenges of his nonspecific symptoms. The disease course had an insidious onset. By the time adenopathy became apparent and was biopsied, a diagnosis of aggressive and advanced angioimmunoblastic T-cell lymphoma and secondary Epstein-Barr virus (+) B-cell lymphoma was eventually made. This case highlights an uncommon form of aggressive NHL (angioimmunoblastic T-cell lymphoma) presenting insidiously as edema most likely secondary to protein-losing enteropathy. Lymphatic invasion can obstruct drainage leading to intestinal lymphangiectasia, ulceration, and inflammatory exudation of protein causing anasarca. Additional mechanisms postulated in the presentation of edema in NHL include an increase in Tumor Necrosis Factor Alpha and vascular endothelial growth factor. Therefore, it is important to consider the diagnosis of NHL in patients with similar manifestations when workup is otherwise inconclusive. Additionally, this case brings to light the critical role that primary care providers can play in maintaining a high index of suspicion for cancer, continuing to work up patients not responsive to initial therapy in a prompt manner to mitigate any delays in diagnosis.

Keywords:
NHL anasarca lymphoma protein losing enteropathy edema primary care

This work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

Figures

References:

[1]	Bognich, J., Hampton, L., Kim, J., Evan, T., Wendy, W., Acharya, V., & Lonnemann, E. Non-Hodgkin's Lymphoma [Internet]. Physiopedia. 2021 [cited 17 July 2021].
[2]	Konar A, Brown C, Hancock B, Moss S. Protein losing enteropathy as a sole manifestation of non-Hodgkin's lymphoma. Postgraduate Medical Journal. 1986; 62(727): 399-400.
[3]	Jillella A, Day D, Severson K, Kallab A, Burgess R. Non-Hodgkin's Lymphoma Presenting as Anasarca: Probably Mediated by Tumor Necrosis Factor Alpha (TNF-α). Leukemia & Lymphoma. 2000; 38(3-4): 419-422.
[4]	Takada K, Kawamura T, Kono T, Sone T, Ogata H, Saito K, Okada Y, Tachi N, Teramoto M, Kato S, Maekawa T, Kobayashi S, Sato K, Sato K, Kimura F. VEGF secretion from Epstein-Barr virus-infected cells as a cause of severe anasarca in a patient with angioimmunoblastic T-cell lymphoma. Rinsho Ketsueki. 2019; 60(12): 1647-1651.
[5]	Swerdlow S, Campo E, Harris N, Jaffe E, Pileri S, Stein H et al. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. World Health Organization; 2017.
[6]	Jaffe E, Arber D, Campo E, Harris N, Quintanilla-Martinez L. Hematopathology. 2nd ed. Elsevier; 2016.
[7]	Swann R, McPhail S, Witt J, Shand B, Abel G, Hiom S et al. Diagnosing cancer in primary care: results from the National Cancer Diagnosis Audit. British Journal of General Practice. 2017; 68(666): e63-e72.
[8]	Surgey, A., Disbeschl, S., Lewis, R., Hiscock, J., Nafees, S., & Law, R. et al. Think Cancer! The multi-method development of a complex behaviour change intervention to improve the early diagnosis of cancer in primary care. 2020.