2011 64-year-old man Rectal Bleeding and diarrhea Past history: IHD, S/P PCI Many years hemorrhoids with occasional bleedings. Normal CBC, except of Creat- 1.8, normal blood chemistry. Full colonoscopy • Hyperemic mucosa without vascular pattern and exudates up to 40 cm- Left sided colitis. • In sigma 2 cm pedunculated polypresected. Lt sided colitis Lt colon- biopsies Pathology • Ulcerated moderate chronic, active colitis associated with cryptitis and crypt abscesses. Negative for dysplasia. • Tubulovillous adenoma with focal high grade dysplasia. Therapy • Topical and systemic 5-ASA with little response. • T.Prednison 40 mg started. • No response. Ongoing rectal bleeding and diarrhea. Patient hospitalized for IV steroids. Revision of pathology CD 5 CD 20 Cyclin D1 positive too CD 20 Case report- Soroka • 80-year-old patient had a colonoscopy 6 months after sigmoidectomy for Ca. • Three polypectomies– tubular adenomas. • Later on bone marrow biopsy was done for pancytopenia and Mantle cell lymphoma diagnosed. • Reassessment of the pathology with immunohistochemistry showed Mantle cell lymphoma in the lamina propria in one of tubular adenomas. • Sztarkier I, Levy I, Walfisch S, Delgado J, Benharroch D Ann Diagn Pathol 2009 MCL mimicking UC • Robert ME, Kuo et al. Diffuse colonic mantle cell lymphoma in a patient with presumed ulcerative colitis. Am J Surg Pathol 1996 • Koksal AS Taskiran I et al. A rare endoscopic appearance of gastrointestinal mantle cell lymphoma resembling ulcerative colitis. Bratisl Lek Listy 2014 PET-CT צוואר-בתי שחי-מדיאסטינום-ראות -ללא קליטות פתולוגיות. בטן -קליטה פתולוגית בעוצמה גבוהה בעיבוי דופן פוליפואידי באנטרום בקוטר 2.4ס"מ הכבד תקין. קליטה פתולוגית בעוצמה גבוה ודיפוזית בטחול שנראה מוגדל .אין קליטות פתולוגיות במעי דק או גס. בדיקת מח עצםMantle Cell Lymphoma - -PET.מאמר (קליטה איפה שהסננה כבדה) Follow-up 2015 • טופל ב 2011-בכמוטרפיה עם תגובה טובה. החולה עדיין ברמיסיה. • – CT-PETתקין • קולונוסוקפיה ביקורת פוליפים -הכל תקין. Mantle Cell Lymphoma (MCL) • Mantle cell lymphoma is an aggressive B-cell lymphoma accounting for 3 % of all Non-Hodgkin lymphomas. • Chromosomal translocation t(11,14) involving cyclin D1 gene causing dysregulation of the cell cycle. • Typically in older patient with generalized lymphadenopathy. Median age -68. MCL- symptoms • Asymptomatic or B-symptoms, splenomegaly in 40% • Symptomatic GI-involvement in 25 %. • Abd.pain, diarrhea,rectal bleeding, protein loosing enteropathy,chylous ascites,abdominal mass, perforation. • In extreme cases polyposis coli. • Asymptomatic GI-involvement in 90% Mantle cell lymphoma- GI-tract • Spectrum of manifestations: • From masses in upper or lower GI-tract to polyposis or only mucosal changes that can be found only by biopsies. • PET-CT can be false negative for mucosal changes only. Mantle Cell Lymphoma of entire GI-tract Homing to GI-tract • Mantle lymphoma cells in enlarged peripheral lymph nodes expressed the α4β7 receptor integrin in 5/7 patients with GI-involvement while none (6/6) of the patients without GI-involvement had it (1). Mantle cells also express high levels of chemokine receptors (2). 1. Geissmann F et al Am J Pathol 1998 2 .Kurtova et al Blood 2009 Gut specific integrins α4β7 MadCam1 Vedolizumab ? Lesson • האם צריכים לדרוש צביעה אימונו-היסטוכימית בכל חולה לא צעיר עם אבחנה חדשה אנדוסקופית והיסטולוגית ( )H&Eשל ? IBD נראה כמו סוס ונשמע כמו סוס אבל זה זברה
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