University of Puerto Rico School of Medicine San Juan, Puerto Rico
Karelys Burgos Irizarry, MD1, Jorge J. Cruz, MD, MSc2, Pablo Costas Caceres, MD2, Roman Velez Rosario, MD1, Pablo Acosta Garayua, MD1; 1University of Puerto Rico School of Medicine, San Juan, Puerto Rico; 2University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico
Introduction: Mantle cell lymphoma (MCL) is a rare and aggressive form of B cell non-Hodgkin lymphoma that typically presents with lymphadenopathy, hepatosplenomegaly and bone marrow infiltration. However, 25% may present as extranodal disease, including invasion of the gastrointestinal tract, pleura and orbit. Involvement of the gastrointestinal tract is usually characterized endoscopically by the presence of multiple lymphomatous polyposis (MLP), but other non-specific features such as pale folds, nodules and inflammatory changes may be the only endoscopic finding. Chronic diarrhea as the single presenting symptom of MCL is very unusual. We report the case of an older male who presented with isolated chronic non-bloody diarrhea. Colonoscopy revealed features indistinguishable from ulcerative colitis, and ultimately proved to be MCL.
Methods: An 84 years old man with cardiac and pulmonary comorbidities presented to the clinic with an 8 year history of isolated chronic non-bloody diarrhea and a 9 kg weight loss. The diarrhea was not associated with meals, fever, chills or any other gastrointestinal symptom and was controlled until recently with loperamide. Workup for infectious etiologies was negative. A colonoscopy revealed mucosal erythema, patchy loss of vascular pattern and multiple submucosal hemorrhages at the sigmoid colon and rectum, suggestive of idiopathic proctosigmoiditis. Unexpectedly, biopsies revealed infiltration by MCL confirmed with immunochemical stains (Figure 1 and 2). Bone marrow aspiration demonstrated 30% involvement by MCL. A PET scan demonstrated prominent lymphadenopathy with increased metabolic activity at the lymph nodes of the left cervical, axillary, pre-tracheal, periaortic and inguinal regions, as well as spleen and lung parenchyma, compatible with extensive lymphomatous infiltration. The patient completed 6 cycles of lenalidomide and rituximab. Follow up PET scan showed complete resolution of previously identified lymphadenopathy and pulmonary lesions. There was complete resolution of the diarrhea. Discussion: This report contributes to a better understanding of an atypical presentation of MCL, and how it may lead to a delay in diagnosis. In this case, isolated chronic non bloody diarrhea for 8 years was the initial manifestation of the disease. Furthermore, endoscopic recognition is challenging, as gastrointestinal involvement may present with non-specific mild inflammatory changes that mimic other conditions and require biopsy for definite diagnosis.
Figure 1. Sigmoid colon with diffuse monomorphic proliferation of small sized lymphoid cells (200x). Insert showing small cells with irregular nuclear contours, dispersed chromatin and inconspicuous nucleoli(1000x, oil immersion)
Figure 2. A. Diffuse positivity for CD5 (400x). B. Negative CD3 (positive in background T lymphocytes) (400x) C. Diffusely positive CD20 (400x). D. Nuclear expression of cyclin D1 (400x)
Disclosures: Karelys Burgos Irizarry indicated no relevant financial relationships. Jorge Cruz indicated no relevant financial relationships. Pablo Costas Caceres indicated no relevant financial relationships. Roman Velez Rosario indicated no relevant financial relationships. Pablo Acosta Garayua indicated no relevant financial relationships.