Subscribe to RSS
DOI: 10.1055/s-2008-1027554
© Georg Thieme Verlag KG Stuttgart · New York
A Rare Cause of Ulcerative Colitis – Diarrhoea and Perianal Bleeding Due to Posttransplant Lymphoproliferative Disorder (PTLD)
Zur Differenzialdiagnose der ulzerierenden Kolitis – Diarrhö und Hämatochezie als Folge extranodalen Befalls einer posttransplantat-lymphoproliferativen ErkrankungPublication History
manuscript received: 12.3.2008
manuscript accepted: 24.5.2008
Publication Date:
11 March 2009 (online)
![](https://www.thieme-connect.de/media/zfg/200903/lookinside/thumbnails/10.1055-s-2008-1027554-1.jpg)
Zusammenfassung
Posttransplantat-lymphoproliferative Erkrankungen (PTLD) weisen charakteristischerweise in einem hohen Prozentsatz einen extranodalen Befall auf, in 20 – 25 % den Gastrointestinaltrakt betreffend. Die Erkrankung tritt als Rarität auch nach Knochenmarktransplantation (KMT) auf, häufiger nach Transplantation solider Organe. Wir berichten über einen 43 Jahre alten Patienten, der mit seit Kurzem bestehenden Diarrhöen, peranalem Blutabgang und Gewichtsverlust in unserer Klinik aufgenommen wurde. Bei ihm war 2 Jahre zuvor eine KMT wegen einer akuten lymphatischen Leukämie (B-Zell-ALL) erfolgt. Sigmoidoskopisch fanden sich tiefe, im Rektum beginnende Schleimhautulzerationen. Weitere Untersuchungen ergaben eine diffuse Rundherd-Infiltration von Leber, Milz, Nieren und Lungen. Histologisch zeigte sich eine monomorphe posttransplantat-lymphoproliferative Erkrankung in Form eines hochaggressiven diffus-großzelligen Non-Hodgkin-Lymphoms der B-Zell-Reihe. Die Zellen exprimierten Proteine des Epstein-Barr-Virus (EBV), und zwar sowohl Marker der latenten als auch der lytischen EBV-Infektion. Die EBV-Assoziation ist für die PTLD typisch und spielt vermutlich eine entscheidende Rolle in der Pathogenese der Erkrankung. Der derzeitige therapeutische Ansatz der hier vorliegenden Form der PTLD besteht in einer Chemotherapie nach dem CHOP-Schema, ergänzt durch den Anti-CD-20-Antikörper Rituximab bei deutlich überwiegenden CD 20-positiven Zellen. Der vorgestellte Patient verstarb wenige Tage nach dem ersten Chemotherapiezyklus an sich rasch entwickelndem Multiorganversagen.
Abstract
Post-transplant lymphoproliferative disorder (PTLD) is characterised by frequent extranodal manifestation, in 20 – 25 % including the gastrointestinal tract. This entity, which is more frequent after solid organ transplantation, rarely occurs after bone marrow transplantation (BMT). We report the case of a 43-year-old male presenting with a short history of rectal bleeding, diarrhoea and weight loss. He had received a bone marrow transplant two years previously for an acute lymphocytic leukaemia of B-cell origin. On sigmoidoscopy, deep ulcerations of the rectal and sigmoideal mucosa were found. Further investigations revealed a diffuse infiltration of the liver, spleen, both kidneys and lungs. Histologically, a monomorphic post-transplant lymphoproliferative disorder was diagnosed, the subtype was a high grade diffuse-large cell Non-Hodgkin’s lymphoma of B-cell origin. The biopsies showed that a major proportion of cells expressed Epstein-Barr virus encoded proteins typical for latent as well as lytic EBV infection. This is a common feature of PTLD and possibly plays a critical role in its pathogenesis. The current therapeutic approach to the subtype of PTLD we saw in this patient is CHOP chemotherapy, comprising the anti-CD 20 antibody rituximab if CD 20-positivity is present. This patient had a fatal course of the disease and died a few days after the first chemotherapy cycle due to severe multiple organ failure.
Schlüsselwörter
ulzerierende Kolitis - peranaler Blutabgang - diffus-großzelliger B-Zell-Lymphom - posttransplantat-lymphoproliferative Erkrankungen - Epstein-Barr-Virus
Key words
ulcerating colitis - lower gastrointestinal bleeding - diffuse large B-cell lymphoma - posttransplant lymphoproliferative disorder - Epstein-Barr virus
References
- 1 Hanauer S B. Update on the etiology, pathogenesis and diagnosis of ulcerative colitis. Nat Clin Pract Gastroenterol Hepatol. 2004; 1 (1) 26-31
- 2 Rubin R H. Cytomegalovirus infections: Epidemiology, diagnosis and treatment strategies. Rev Infect Dis. 1990; 12 691-849
- 3 Shigeno T, Akamatsu T, Fujimori K. et al . The clinical significance of colonoscopy in hemorrhagic colitis due to enterohemorrhagic Escherichia coli O 157:H 7 infection. Endoscopy. 2002; 34 (4) 311-314
- 4 Hartmann F. Colitis ulcerosa. Hahn GE, Riemann JF Klinische Gastroenterologie Stuttgart, Germany; Georg Thieme Verlag 2000: 742-748
- 5 Kakar S, Pardi D S, Burgart L J. Colonic ulcers accompanying collagenous colitis: implication of nonsteroidal anti-inflammatory drugs. Am J Gastroenterol. 2003; 98 (8) 1834-1837
- 6 Miyahara S, Ito S, Soeda A. et al . Two cases of systemic lupus erythematosus complicated with colonic ulcers. Intern Med. 2005; 44 (12) 1298-1306
- 7 Memain N, De B M, Guillevin L. et al . Delayed relapse of Churg-Strauss syndrome manifesting as colon ulcers with mucosal granulomas: 3 cases. J Rheumatol. 2002; 29 (2) 388-391
- 8 Naganuma M, Iwao Y, Kashiwagi K. et al . A case of Behcet’s disease accompanied by colitis with longitudinal ulcers and granuloma. J Gastroenterol Hepatol. 2002; 17 (1) 105-108
- 9 Hsiao C H, Kao H L, Lin M C. et al . Ulcerative colon T-cell lymphoma: an unusual entity mimicking Crohn’s disease and may be associated with fulminant hemophagocytosis. Hepatogastroenterology. 2002; 49 (46) 950-954
- 10 Okada M, Maeda K, Suzumiya J. et al . Primary colorectal T-cell lymphoma. J Gastroenterol. 2003; 38 (4) 376-384
- 11 Daniel F, Damotte D, Moindrot H. et al . A steroid-refractory ulcerative colitis revealing Epstein-Barr virus/cytomegalovirus-positive colonic lymphoma. Int J Colorectal Dis. 2006; 21 (3) 288-290
- 12 Nakamura T, Nakamura R, Maruyama K. et al . Refractory ulcerative colitis complicated by a cytomegaloviral infection requiring surgery: report of a case. Surg Today. 2004; 34 (1) 68-71
- 13 Lewthwaite P, Gill G V, Hart C A. et al . Gastrointestinal parasites in the immnuocompromised. Curr Opin Infect Dis. 2005; 18 (5) 427-435
- 14 Schneider T. Infektionen des Gastrointestinaltraktes. Alexander K, Daniel WG, Diener HC et al Thiemes Innere Medizin Stuttgart, Germany; Georg Thieme Verlag 1999 : 491-514
- 15 Hasegawa W, Pond G R, Rifkind J T. et al . Long-term follow up of secondary malignancies in adults after allogeneic bone marrow transplantation. Bone Marrow Transpl. 2005; 35 51-55
- 16 Leblond V, Dhedin N, Mamzer Bruneel M F. et al . Identification of prognostic factors in 61 patients with posttransplantation lymphoproliferative disorders. J Clin Oncol. 2001; 3 772-778
- 17 Poirel H A, Bernheim A, Schneider A. et al . Characteristic pattern of chromosomal imbalances in posttranplantation lymphoproliferative disorders: Correlation with histopathological subcategories and EBV status. Transplantation. 2005; 80 (2) 176-184
- 18 Choquet S, Leblond V, Herbrecht R. et al . Efficacy and safety of rituximab in B-cell post-transplant lymphoproliferative disorders: results of a prospective multicentre phase II study. Blood. 2006; 107 3053-3057
- 19 Caillard S, Lachat V, Moulin B. Posttransplant lymphoproliferative disorders in renal allograft recipients: report of 53 cases of a French multicenter study. PTLD French Working Group. Transpl Int. 2000; 13 (Suppl 1) 388-393
- 20 Pickhardt P J, Siegel M J. Posttransplant lymphoproliferative disorder of the abdomen: CT evaluation in 51 patients. Radiology. 1999; 1 73-78
- 21 Gassmann W. Hämatologische Diagnostik. Alexander K, Daniel WG, Diener HC et al Thiemes Innere Medizin Stuttgart, Germany; Georg Thieme Verlag 1999: 817-826
- 22 Hummel M, Anagnostopoulos I, Korbjuhn P. et al . Epstein-Barr Virus in B-cell Non-Hodgkin lymphomas: unexpected infection patterns and different infection incidence in low- and high grade types. J Pathol. 1995; 175 263-271
- 23 Trappe R, Oertel S, Riess H. HIV-assoziierte Lymphome und Posttransplantations-Lymphome. Der Onkologe. 2006; 12 641-650
- 24 Trappe R, Oertel S, Riess H. Pathogenese, Klinik, Diagnostik und Therapie transplantationsassoziierter lymphoproliferativer Erkrankungen. Dtsch Arztebl. 2006; 103 (48) A 3259-A 3267
- 25 Oertel S H, Verschuuren E, Reinke P. et al . Effect of anti-Cd 20 antibody rituximab in patients with post-transplant lymphoproliferative disorder (PTLD). Am J Transplant. 2005; 12 2901-2906
- 26 Trappe R, Oertel S H, Choquet S. et al . Sequential treatment with the anti-CD 20 antibody rituximab and CHOP+GSCF chemotherapy in patients with post-transplant lymphoproliferative disorder (PTLD): First results of a multicenter phase II study. Blood. 2005; 11 274a
- 27 Peterson M R, Emery S C, Yung G L. et al . Epstein-Barr virus-asociated posttransplantation lymphoproliferative disorder following lung transplantation is more commonly of host origin. Arch Pathol Lab Med. 2006; 130 (2) 176-180
- 28 Heslop H E, Savoldo B, Rooney C M. Cellular therapy of Epstein-Barr-virus-associated post-transplant lymphoproliferative disease. Best Pract Res Clin Haematol. 2004; 17 (3) 401-413
- 29 Loren A W, Porter D L, Stadtmauer E A. et al . Post-transplant lymphoproliferative disorder: a review. Bone Marrow Transplant. 2003; 31 (3) 145-155
Dr. Christian Godt
Medizinische Klinik, Klinikum Bremen-Ost
Züricher Str. 40
28325 Bremen
Phone: ++ 49/4 21/40 80
Email: cgodt@gmx.de