MALToma Discovered on a Hemorrhagic Shock Associated with Gastric Dieulafoy’s Lesion: A Case Report Open Access

Introduction: Dieulafoy’s lesion is a rare but severe cause of digestive bleeding and can rarely present as a hemorrhagic shock which is associated with a severe prognosis. MALToma is a B-cell lymphoma of the mucosa-associated lymphoid tissue. It was reported that MALToma can be revealed by a Dieulafoy’s lesion. Due to the lack of specific endoscopic or clinical characteristics, several endoscopies may be needed to establish the diagnosis of MALToma. Case Presentation: A 61-year-old male patient presented to the emergency department with melena and hematemesis. The patient was transferred to the intensive care unit due to hemorrhagic shock. The endoscopic work-up showed large gastric folds which were later confirmed as MALToma in anatomo-pathologic analysis. The echoendoscopy showed a vessel going through the entire gastric wall which endoscopically corresponded to a red non-bleeding lesion, confirming Dieulafoy’s lesion as the origin of the bleeding. The patient received an endoscopic treatment and was rapidly discharged from the intensive care unit. The evolution was spontaneously favorable, and there was no repeat active bleeding. Conclusion: A gastric MALToma was discovered thanks to a hemorrhagic shock on a Dieulafoy’s lesion which has not yet been described in the literature.

Dieulafoy’s lesion is a rare but severe cause of digestive bleeding. It is described as an abnormal large artery localized in the submucosa, eroding the muscularis mucosae, which can cause bleeding due to its superficial location. Dieulafoy’s lesion can rarely present as a hemorrhagic shock which is associated with a severe prognosis [1, 2].

MALToma is a B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT). This extra-nodal non-Hodgkin lymphoma occurs in patients in the fifth and sixth decade. The diagnosis can be difficult and is often missed in the early stages. Indeed, in case of MALToma, the mucosa may have an aspecific aspect such as irregular superficial erosions, enlarged gastric folds, gastric nodules, or thickened gastric walls. Moreover, the signs and symptoms are aspecific; the patients mostly present with dyspepsia and abdominal pain. Several endoscopies may be needed to establish the diagnosis. MALToma is strongly associated with Helicobacter pylori infection. H. pylori must actively be searched and eradicated when present as it is known to contribute to tumorigenesis [3].

Due to the nonspecific symptoms and the nonspecific endoscopic aspect, some patients with MALToma can be missed. Several endoscopies may be needed to establish the diagnosis. In this case, Dieulafoy’s lesion uncovered the large fold gastropathy with MALToma that could have been missed as the patient was asymptomatic.

This article aimed to draw attention to the diagnostic challenges of large fold gastropathies and to present a rare presentation of MALToma. The CARE Checklist has been completed by the authors for this case report, attached as online supplementary material (for all online suppl. material, see https://doi.org/10.1159/000545012).

A 61-year-old male patient presented to the emergency department with melena and hematemesis. The patient was immediately transferred to the intensive care unit due to hemorrhagic shock with hyperlactatemia (5.9 mmol/L) and tachycardia. He was treated with massive fluid perfusion, blood transfusion, and high doses of intravenous PPI. No amines were required.

The first esophagogastroduodenoscopy showed a large blood clot in the gastric body that was not removable. No active bleeding was visualized, suggesting a Dieulafoy’s lesion even though no vessel was visualized. As the large blood clot was not removable and was obturing the visualization of the mucosa underneath, and as the patient was in hemorrhagic shock with evident gastric bleeding, and without a clear diagnosis after the initial endoscopy, a second-look was required for an optimal examination.

The day after, a second esophagogastroduodenoscopy was performed and showed large gastric folds without any active bleeding (shown in Fig. 1, 2). Biopsies were performed, and pathological results showed marginal zone B-cell lymphoma of the MALT.

The echoendoscopy showed a hypoechogenic thickening of the mucosa involving the muscular layer and the perigastric fat and a vessel going through the entire gastric wall which endoscopically corresponded to a red non-bleeding lesion, confirming Dieulafoy’s lesion as the origin of the bleeding (shown in Fig. 3). No secondary lesion was identified on the thoraco-abdominal scanner (shown in online suppl. Fig. 1) and TEP scanner (shown in online suppl. Fig. 2).

The definitive diagnosis of marginal zone B-cell lymphoma of the MALT was confirmed on gastric biopsies, with small lymphoid cells positive for BCL2 and CD20 and negative for CD5 (shown in online suppl. Fig. 3). No H. pylori infection was seen in the context of concomitant PPI use. The patient was then diagnosed as a T3N0M0 MALToma according to the TNM classification and IE according to Ann Arbor classification.

Two endoscopic clips were put in place, and the patient was rapidly discharged from the intensive care unit.

The evolution was spontaneously favorable, and there was no repeated bleeding. At the 6-month follow-up, the patient was asymptomatic, with no repeated bleeding. He has received antibiotherapy for H. pylori eradication without any H. pylori infection evidence as recommended in the latest ESMO Guidelines (2020) [2] and is currently under active surveillance until the spontaneous recovery of the mucosa. The follow-up breath test is negative. The patient’s perspective was positive, as the disease was closely monitored during the follow-up.

MALToma represents 8% of the non-Hodgkin lymphoma. It is localized in 35% in the stomach and associated with H. pylori infection in 90% of cases [4]. The diagnosis is made on anatomo-pathological samples by morphological, immune-histological, and genetic analyses. Histologically, MALToma is characterized by the presence of lymphocytes “centrocytes-like” diffuse or organized in nodules, lymphoepithelial lesions, and reactional lymphoid follicles. The typical immunohistochemical phenotype is positive for B-cell marker CD20 and negative for CD5, CD10, and cyclin D1. The genetic analyses have a prognosis value as the chromosomal translocation t(11;18) (q21;q21) is associated with poor response to H. pylori eradication and with advanced stage of the disease [5, 6].

Endoscopically, MALToma can present with aspecific gastric lesions such as a large fold gastropathy. Large fold gastropathies can be caused by a wide range of diagnoses. We differentiate the real hyperplastic gastropathies from the hypertrophic gastropathies. The term hyperplastic refers to an augmentation of the number of cells that create more volume, while the term hypertrophic designs only an increase of volume without an etiologic explanation [7]. Within the “real” hyperplastic gastropathies, we have to consider mainly Menetrier disease, lymphocytic gastropathy, and Zollinger-Ellison syndrome-associated gastropathy.

• Menetrier disease usually presents with abdominal pain, vomiting, and generalized oedema with hypoalbuminemia due to gastric plasmatic exudation. Menetrier disease is a very rare disease characterized by the increase of the crypt/gland ratio. There is no consensus for Menetrier disease’s treatment, but it is usually recommended to administrate PPI therapy associated with high protein intake to substitute the protein loss. A treatment by somatostatin analogue can be proposed. If H. pylori is present, it is recommended to eradicate it. Finally, if all fail, cetuximab can be proposed [7, 8].

• Lymphocytic gastropathy is a microscopically described disease associated with H. pylori infection or celiac disease which represent each 30% of cases.

• Zollinger-Ellison syndrome-associated gastropathy is characterized by a low crypt/gland ratio with an increase of the fundus glands and parietal cells. This gastropathy is secondary induced by the hypergastrinemia due to pancreatic or duodenal neuroendocrine tumor.

The hypertrophic gastropathy is a very heterogeneous group that is characterized by large folds secondary to chorion invasion by inflammatory cells, tumoral cells, or extracellular depots. Within the hypertrophic group, we can find cancerous disease (adenocarcinoma and lymphoma), infectious gastropathies (H. pylori, tuberculosis, CMV, etc.), inflammatory disease (Crohn, IgG4 disease), extracellular depots (amyloidosis), and vascular disease (GAVE, portal hypertension gastropathy).

Whether the gastropathy is hyperplastic or hypertrophic, the diagnosis is essentially determined by anatomo-pathological analysis and can be helped with endoscopic and radiologic devices. Menetrier disease, although rare, must be considered when large fold gastropathy is encountered, and as it is a complicated diagnostic that requires a deeper biopsy, it is recommended to perform a mucosectomy with snare biopsies to obtain larger samples [7].

Dieulafoy’s lesion is a rare but serious cause of digestive bleeding that is described as an abnormally large artery localized in the submucosa which can cause recurrent bleeding due to its superficial location. It can be sometimes present as a hemorrhagic shock which is associated with a severe prognosis [9]. Dieulafoy’s lesion may be caused by the use of NSAIDs and is often associated with conditions that affect blood coagulation, such as chronic renal failure and cardiovascular disease [3].

An esophagogastroduodenoscopy can make the diagnosis when active arterial bleeding is visualized and propose a treatment by mechanical and thermal techniques. Indeed, it is recommended to apply two hemostasis modalities with actively bleeding ulcers (FIa, FIb) according to ESGE Guidelines 2021 [10]. In case of endoscopic treatment failure, an angiographic embolization can be proposed. A surgical removal of the lesion is recommended by wedge resection in refractory cases in case of endoscopic and radiologic therapy failures [11].

While the physiopathology between MALToma in particular and Dieulafoy’s lesion is not clear in the literature, previous reports have presented an association between gastric carcinogenesis and Dieulafoy’s lesion such as gastric adenocarcinoma [12]. We suggest that the chronic inflammation (usually caused by H. pylori) leading to MALToma may fragilitate the surrounding gastric tissue and can favorize the apparition of vascularization abnormalities such as Dieulafoy’s lesions. Another hypothesis proposed by Taketsuka et al. [13] is that the presence of Dieulafoy’s lesion will cause hypoxic environment in gastric tissue and induce repeated mucosal regeneration that will promote the apparition of abnormal gastric cells with dysplasia and thus lead to carcinogenesis.

Due to the nonspecific symptoms and the nonspecific endoscopic aspects, some patients with MALToma can be missed. Several endoscopies may be needed to establish the diagnosis.

It was reported that MALToma can be revealed by a Dieulafoy’s lesion [14]. In our case, the patient presented hemorrhagic shock due to a Dieulafoy’s lesion on a gastric MALToma which has, to the best of our knowledge, not yet been described in the literature.

Ethical approval is not required for this case report in accordance with local guidelines. Written informed consent was obtained from the patient for publication of the details of their medical case and any accompanying images.

We declare no conflict of interest.

This case report was not supported by any sponsor or funder.

E.L. wrote and edited the script. R.M. selected the PET scan images. N.N. selected the pathology images. E.T., M.A., and A.C. reviewed and supervised the article.

All data analyzed during this case report are included in this article. Further inquiries can be directed to the corresponding author.