A 40-year-old man with recurrent, life-threatening gastrointestinal bleeding was found to have an intestinal lymphangioma with extensive secondary lymphangiectasia in what researchers described as the first reported case requiring small bowel transplantation.
The case, published in Case Reports in Pathology, illustrates how intestinal lymphatic disease can present with severe hemorrhage despite the absence of laboratory features typically associated with protein-losing enteropathy.
The patient presented with gastrointestinal bleeding, iron-deficiency anemia, and abdominal pain. CT showed hypodense mesenteric lymphadenopathy, raising concern for a lymphoproliferative disorder. Double-balloon enteroscopy demonstrated diffuse jejunal infiltration, and technetium-99m–labeled red blood cell scintigraphy localized active bleeding to the territory of the superior mesenteric artery.
Endoscopic biopsy showed a vascular-rich lesion, with immunohistochemical staining positive for CD31, CD34, and D2-40. Subsequent pathology review identified cystic spaces lined by endothelial cells and dilated lymphatic channels, supporting a diagnosis of lymphangioma with diffuse secondary lymphangiectasia.
The presentation differed from previously reported cases of extensive intestinal lymphangiectasia. In the researchers’ review of 16 adult cases, 81.3% had hypoalbuminemia, 56.3% had lymphopenia, and 37.5% had hypogammaglobulinemia. In contrast, this patient had relatively preserved albumin without lymphopenia or significant immunoglobulin deficiency, despite refractory hemorrhage requiring more than 20 units of packed red blood cells.
Persistent bleeding, diffuse disease precluding segmental resection, and failure of conservative management led to small bowel transplantation with subtotal colectomy. Pathology demonstrated lymphangiectasia involving the small and large intestines, appendix, and gallbladder. Mesenteric lymph nodes showed reactive follicular hyperplasia without lymphoma, supporting the authors’ emphasis on tissue diagnosis when imaging suggests lymphoproliferative disease.
The patient subsequently developed empyema, extensive venous thrombosis, and multiorgan failure and died 20 days after transplantation. The researchers advised considering intestinal lymphangiectasia in adults with unexplained gastrointestinal bleeding, even in the absence of classic protein-losing enteropathy features.
The authors reported no funding for the manuscript and no conflicts of interest. All data relevant to the case were included in the published article.
Source: Case Reports in Pathology
