64-year-old man Rectal Bleeding and diarrhea Past history: IHD, S/P

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