eISSN: 2084-9869
ISSN: 1233-9687
Polish Journal of Pathology
Current issue Archive Manuscripts accepted About the journal Supplements Editorial board Abstracting and indexing Subscription Contact Instructions for authors Ethical standards and procedures
SCImago Journal & Country Rank
1/2012
vol. 63
 
Share:
Share:
more
 
 

Enterocolic lymphocytic phlebitis: an unusual cause of abdominal complaints

Krystyna Gałązka
,
Tomasz Tokarek
,
Tomasz Gach
,
Joanna Szpor

Pol J Pathol 2012; 1: 75-79
Online publish date: 2012/04/24
Article file
Get citation
ENW
EndNote
BIB
JabRef, Mendeley
RIS
Papers, Reference Manager, RefWorks, Zotero
AMA
APA
Chicago
Harvard
MLA
Vancouver
 
 

Introduction



Enterocolic lymphocytic phlebitis (ELP), also called mesenteric veno-occlusive disease (MVOD) is a rare disorder, which affects typically small and medium-sized intramural and mesenteric veins of the intestines, most commonly – of the large bowel [1]. The disease is characterized by sparing of arterial and lymph vessels. Inflammatory changes of the veins occur most frequently in the right colon, although rare cases of other locations of the disease were reported [2, 3]. Inflammation of the vessel wall is usually complicated by thrombosis, thus blood flow disturbances manifesting as ischemic complications frequently result in clinical symptoms of the acute abdomen. The clinical picture with abdominal acute pain, peritoneal signs at physical examination, nausea and vomiting, diarrhea, sometimes bloody, is most common in the course of ELP [2, 4-9]. Due to the frequent location in the right colon, it simulates acute appendicitis. Rarely, the disease manifests as a chronic dysfunction of the organ occupied by inflammatory changes or abdominal mass only [3, 10-12].

The ELP has been known for about 35 years [4, 13] as a disease occurring very rarely (to our knowledge, about 50 cases reported worldwide; no case has been described in Poland yet) of unexplained etiopathogenesis. The case reported by us supports the adequacy of the other name used for designation of the disease, idiopathic colonic phlebitis as ELP has developed in a healthy man, without any previous diseases, risk factors for thrombosis or any drug/treatment history.

Case report



Clinical history



A 35-year-old Caucasian male was admitted to the Chair and Clinic of General and Gastroenterological Surgery of the Jagiellonian University, Collegium Medicum with symptoms pointing to acute appendicitis: acute pain localized in the right lower abdomen lasting for two days accompanied by vomiting and diarrhea. Physical examination revealed the acute abdomen symptoms only: tenderness and pain of the abdomen, positive Blumberg’s and Rowsing’s signs. The results of basic laboratory analyses indicated inflammation and resulted from dehydration: white blood cell (WBC) 14.400 (elevated neutrophilic leukocytosis), hemoglobin (Hb) 18.9 g/dl (thereafter, Hb level normalized during hospitalization). Minimally prolonged activated partial thromboplastin time (APTT) (37.7 s) and decrease in prothrombin time (PT) (INR 0.86) excluded mesenteric venous thrombosis as primary etiology of the symptoms. The results of other laboratory tests (Na and K levels, HCT, PLT, amylase, lipase, urea, creatinine, bilirubin, ethyl alcohol) were unremarkable. The ultrasound examination of the abdominal cavity revealed a small amount of peritoneal effusion and fusion of intestines above the right iliac crest. On laparoscopy, the appendix was unchanged, whereas the cecum and ascending colon appeared abnormal, with thickening of the walls and tumorous mass. Due to a suspicion of Crohn’s disease, the right hemicolectomy was performed. The surgical hemicolectomy specimen was fixed in formalin solution and sent to the Department of Pathomorphology.

On the 3rd day after surgery, because of symptoms pointing to intraabdominal bleeding, re-laparotomy was performed in the patient with evacuation of clots and the hemolyzed blood from the peritoneal cavity. During that procedure, no signs of the recent hemorrhage were found. The course thereafter was uneventful and the patient was discharged from the clinic in good general condition on the 11th day after the first surgery. No recurrence or other complications were noted for the 18-month follow-up of the patient.



Pathological examination



In the surgical right hemicolectomy specimen, in the cecum and ascending colon (25 mm from the Bauhin’s valve) transmural tumorous thickening was visible, 5 cm in maximal dimension, covered by dirty-red, velvety mucosa with flattened rugs. On the cross section, the mass was homogenous, grayish-purple, with a blurred structure of the colonic wall. The wall of the terminal ileum (6 cm-long segment) was unremarkable; the mucosal folds of the colon in the vicinity of the above-described lesion and at some distance from it were slightly thicker with some stiffness.

Histologically, in sections from the tumorous lesion, recent ischemic and partially hemorrhagic necrosis was found concomitant with marked edema and superficial ulceration (Fig. 1 A). The necrosis was transmural focally with mild fibrinous serosal exudate. Many small veins of submucosa, muscularis propria and subserosa presented with circumferential or crescentic inflammatory infiltrate of the walls and in their surroundings, varying in intensity, abundant or scarce (Fig. 1 B). In some affected vessels thromboses, focal mild necrosis of the walls and myointimal proliferation were found (Fig. 1 C). Inflammatory infiltrates were composed almost exclusively of small

lymphocytes, with only few histiocytes, in a single

vein with the presence of giant multinucleated cells (Fig. 1 D). The arteries and lymph vessels were normal, not affected by inflammation (Figs. 1 C, 2 A). The cuff lymphocytic infiltrates of intramural veins sometimes with thrombi and myointimal proliferation were visible in numerous sections obtained from the remaining colonic wall, not affected by necrosis, also in the surgical colonic margin (Figs. 2 B, 2 C), Bauhin’s valve (Fig. 2 D) and in the appendiceal wall, whereas no such lesions were found in the surgical margin of the terminal ileum segment. Numerous lymph nodes of the ileo-cecal region and at the wall of the ascending colon appeared strongly congested and with non-significant reactive changes.

The histological diagnosis was enterocolic lymphocytic phlebitis (mesenteric veno-occlusive disease) (No. 1701640).

Discussion



The ELP cases, reported also as necrotizing and giant cell granulomatous phlebitis, idiopathic myointimal hyperplasia of mesenteric veins, mesenteric inflammatory veno-occlusive disease (MIVOD), necrotizing phlebitis, intramural mesenteric venulitis and idiopathic colonic phlebitis are rare, however, they may be underdiagnosed as endoscopy specimens are usually non-diagnostic [3] due to deep intramural location of the changed veins.

The disease affects both women and men, usually middle-aged and older adults [1-10, 12], it rarely occurs in young adults (the youngest patient reported was 25 years old [11]). The patients present typically with either acute abdomen symptoms or chronic abdominal complaints, with frequent gastrointestinal hemorrhage or tumor-like mass appearance due to ischemic damage to the intestinal wall [1-12].

The ischemic complications result from damage to the intestinal intramural veins mediated by lymphocytes, the T cells mainly of cytotoxic lineage [1, 3, 5, 6, 12]. A trigger of the injurious reaction of these cells to vein walls is unknown. An association with some drugs (e.g. Venoruton) was suggested but no proof was found for such etiology as in many patients with ELP reported in the literature, no history of any drug was found [1, 7, 11]. On the other hand, in some patients, the abnormal immune reaction with dysregulated activation of T lymphocytes may be triggered by any drug as single cases of ELP [12] were reported to co-occur with lymphocytic colitis, for which infectious agents and drugs were proposed as possible etiopathological factors.

The ELP has to be distinguished from other disorders being known to manifest or be complicated by inflammatory involvement of the vessels of the gastrointestinal tract [14]. Histological differential diagnosis includes Behcet’s disease, vasculitis in the course of systemic immune diseases, e.g. SLE, secondary vasculitis, spontaneous thrombosis of the mesenteric veins and hypersensitivity reactions. The diagnosis is usually easy, as in diseases other than ELP, the arterial vessels are frequently involved, the inflammation of vessels is more systemic rather than localized, and/or the cellular composition of inflammatory infiltrate differs, with the presence of granulocytes, whereas in ELP the infiltrate is composed of mononuclear cells with small lymphocytes prevailing.

Lymphocytic perivenular circumferential or crescentic infiltrates, occurring both in ischemic and viable bowel segments are characteristic of ELP [1, 4, 5]. The presence of variable histological changes in ELP, lymphocytic infiltrates only or with myointimal hyperplasia of the veins and thrombosis, granulomatous vasculitis seems to depend on the stage of the pathological changes [1].

In endoscopic biopsies, the pathological hallmarks of ELP are usually undetectable because of their deep intramural location. Thus, the treatment mode is surgical resection, the diagnosis of the disease is established after the surgery, and data on conservative treatment do not exist. The disease seems to be self-limiting as the post-surgery follow-up of the patients is usually with no complications, even in cases with positive surgical margins [1, 4, 6, 11]. The uncomplicated course was also observed in our case, in which the surgical margin of the colon was affected by lymphocytic phlebitis.

References



 1. Saraga E, Bouzourenne H. Enterocolic (lymphocytic) phlebitis: a rare cause of intestinal ischemic necrosis. A series of six patients and review of the literature. Am J Surg Pathol 2000; 24: 824-829.

 2. Nakamura Y, Ohta G, Doishita K, et al. Granulomatous liver disease in the small hepatic and portal veins. Arch Pathol Lab Med 1980; 104: 456-458.

 3. Abraham SC, Solem CA, Hauser SC, Smyrk TC. Chronic antral ulcer associated with gastroduodenal lymphocytic phlebitis. Am J Surg Pathol 2004; 28: 1659-1663.

 4. Saraga E, Costa J. Idiopathic entero-colic lymphocytic phlebitis: a cause of ischemic necrosis. Am J Surg Pathol 1989; 13: 303-308.

 5. Medlicott SAC, Guggisberg KA, DesCoteaux JG, Beck P. Enterocolic lymphocytic phlebitis: statistical analysis of histology features in viable and ischemic bowel. Int J Surg Pathol 2006; 14: 200-205.

 6. Tuppy H, Haidenthaler A, Schandalik R, Oberhuber G. Idiopathic enterocolic lymphocytic phlebitis: a rare cause of ischemic colitis. Mod Pathol 2000; 13: 897-899.

 7. Hu JCC, Forshaw MJ, Thebe P, Stewart M. Mesenteric inflammatory veno-occlusive disease as a cause of acute abdomen: report of five cases. Surg Today 2005; 35: 961-964.

 8. Mesina C, Vasile I, Pasalega M, et al. Mesenteric inflammatory veno-occlusive disease as a rare cause of acute abdomen. Rom J Morphol Embryol 2007; 48: 83-86.

 9. Pares D, Biondo S, Marti-Rague J, et al. Enterocolic lymphocytic phlebitis of the right colon as a cause of massive gastrointestinal bleeding. Colorectal Dis 2003; 5: 376-379.

10. Arain FA, Willey J, Richter J, et al. An usual presentation of enterocolic lymphocytic phlebitis. J Clin Gastroenterol 2002; 34: 252-254.

11. Bao P, Welch DC, Washington MK, Herline AJ. Resection of mesenteric inflammatory veno-occlusive disease causing ischemic colitis. J Gastrointest Surg 2005; 9: 812-817.

12. Jain R, Chetty R. Enterocolic lymphocytic phlebitis and lymphocytic colitis: drug-related coexistent pathology. Int J Colorectal Dis 2009; 24: 473-464.

13. Stevens SM, Gue S, Finckh ES. Necrotizing and giant cell granulomatous phlebitis of caecum and ascending colon. Pathology 1976; 8: 259-264.

14. Ahn E, Luk A, Chetty R, Butany J. Vasculitides of the gastrointestinal tract. Semin Diagn Pathol 2009; 26: 77-88.



Address for correspondence

Krystyna Gałązka MD, PhD

Department of Pathomorphology

Jagiellonian University, Collegium Medicum

ul. Grzegórzecka 16

31-531 Kraków, Poland

e-mail: bengot@wp.pl
Copyright: © 2012 Polish Association of Pathologists and the Polish Branch of the International Academy of Pathology This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (http://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
Quick links
© 2022 Termedia Sp. z o.o. All rights reserved.
Developed by Bentus.