Recurrent Massive Gastrointestinal Bleeding as a first presentation of AL Amyloidosis in a 76-Year-Old Patient

Case Report

Ann Hematol Onco. 2023; 10(6): 1443.

Recurrent Massive Gastrointestinal Bleeding as a first presentation of AL Amyloidosis in a 76-Year-Old Patient

Eilam Rabina1; Sagee Tal2; Yaron Rudnicki3; Assaf Rahmani3; Timna Naftali4,6; Martin H Ellis5,6; Osnat Jarchowsky Dolberg1,5,6

1Internal medicine A ward, Meir Medical Center, Kfar Saba Israel

2Pathology Institute, Meir Medical Center, Kfar Saba Israel

3General surgery B ward, Meir Medical Center, Kfar Saba Israel

4Gastroenterology and Hepatology Institute, Meir Medical Center, Kfar Saba Israel

5Hematology Institute, Meir Medical Center, Kfar Saba Israel

6Tel Aviv University, Israel

*Corresponding author: Osnat Jarchowsky Dolberg Internal medicine A ward, Meir Medical Center, 59 Tchernichovsky St, Kfar Saba 44281, Israel Tel Aviv University, Israel. Email: Osnat.jarchowsky@clalit.org.il

Received: November 28, 2023 Accepted: December 21, 2023 Published: December 28, 2023

Abstract

Background: Light chain or AL amyloidosis is a systemic disease that occasionally involves the gastrointestinal tract, but very rarely presents as gastrointestinal bleeding without evidence of systemic involvement. In such cases recognition of amyloidosis as the cause of bleeding is essential in order to rapidly initiate effective treatment to stop the bleeding.

Case presentation: We describe a 76-year-old patient who presented with recurrent massive gastrointestinal bleeding as the initial manifestation of AL amyloidosis. One episode necessitated emergency right colectomy, and histopathological analysis of the resected bowel revealed diffuse amyloid infiltration. This finding prompted further investigations, which demonstrated elevated plasma lambda light chain levels, and 10% monoclonal plasma cells in the bone marrow. Treatment with bortezomib, dexamethasone, and daratumumab was initiated and no further gastrointestinal bleeding occurred.

Conclusion: The diagnosis of amyloidosis involving the gastrointestinal tract should be considered in adult patients with unexplained recurrent gastrointestinal bleeding.

Background

Gastrointestinal Bleeding (GIB) has various etiologies, the commonest being peptic ulcer disease and diverticulosis [1,2].

Amyloid Light chain (AL) amyloidosis, characterized by the deposition of proteins derived from Immunoglobulin (Ig) Light Chains (LCs) in a variety of solid organs, is a rare condition [3]. Involvement of the Gastrointestinal Tract (GIT) in this disorder is not uncommon [4,5]; yet few cases of GIB as the initial presentation of amyloidosis have been reported [6,7], and none in which recurrent massive GIB is the sole clinical manifestation.

In this report, we present a rare case of recurrent massive GIB that initially eluded explanation. Subsequently, the patient was diagnosed with AL amyloidosis, with diffuse GIT involvement which was the source of GIB. Appropriate treatment for amyloidosis was initiated with a salutary effect on the bleeding.

Case Presentation

A 76-year-old patient with a history of Atrial Fibrillation (AF) managed with rivaroxaban presented to the Emergency Department (ED) with massive hematochezia. This resulted in hemorrhagic shock with a hemoglobin (Hb) level of 4.5g/dL (normal range: 12–16g/dL). He was treated with systemic tranexamic acid, multiple blood transfusions were administered and rivaroxaban was discontinued. Emergency upper endoscopy was normal and colonoscopy revealed two, 0.8 cm long, polypoid protrusions, a round solid 4 cm-diameter submucosal mass with a bleeding marker in the ascending colon, and a 2 cm long clot attached to the mucosa near the hepatic flexure (Figure 1). Biopsies were taken from the polyps and from the submucosal mass showed mild inflammation only. One month later, the patient again presented to the ED with hemorrhagic shock due to GIB. Computer tomogram angiography identified active colonic bleeding near the hepatic flexure, thickening of terminal ileum and cecum wall, and enlarged abdominal lymph nodes (Figure 2). Emergency right hemicolectomy and diversion ileostomy were performed. Histology of the resected colon and ileum of diffuse submucosal infiltration of amorphous material that was positive for amyloid with Congo red staining under polarized light. Amyloid deposits were also found in many of the 42 peri-colonic lymph nodes that were resected.