CASE REPORT Hematological malignancies in chronic inflammatory bowel diseases: report of five cases and review of the literature Pietro Crispino & Roberta Pica & Erika Angelucci & Adriana Consolazio & Margherita Rivera & Claudio Cassieri & Paolo Paoluzi Accepted: 28 July 2006 / Published online: 7 October 2006 # Springer-Verlag 2006 Abstract Several forms of primary and secondary hemato- logical malignancies were rarely observed during the clinical course of inflammatory bowel diseases (IBD). Patients needing a prolonged treatment with immunosuppressants, such as azathioprine or methotrexate, with familiarity and genetic predisposition seem to be at a higher risk of leukemia. On the other hand, asthenia, thickness, and fever may be the symptoms of the onset of each kind of hematological malignancy. The finding of anemia, alteration of leukocyte count and large undetermined cells may suggest increased probability of abnormal proliferation of a single white blood cell line. In this report, the occurrence of hematological malignancies is described in five patients affected by IBD (three with ulcerative colitis and two with Crohn’ s disease) attending our Gastroenterology Unit. Keywords Hematological malignancies . Inflammatory bowel diseases . Immunological disorders Introduction Crohn’ s disease (CD) and ulcerative colitis (UC) are chronic inflammatory bowel diseases (IBD) of unknown etiology, even if a large amount of data would appear to suggest a basic autoimmune dysfunction. Hematological changes, including anemia, hypercoagulable state, throm- bocytosis, and leucocytosis, were observed in association with IBD. Some Authors [1, 2] reported the occurrence of acute or chronic lymphoid forms or myeloid leukemia in IBD patients and this association seems not to be occasional, the relative risk of developing hematological malignancies being 5.3 in IBD patients. The association seems to be related to several factors, including genetic susceptibility, exposure to environmental toxic agents (pesticides and organic solvents), autoimmune diseases or exposure to diagnostic or therapeutic radiation, and as- sumption of drugs as immunosuppressants [3]. In this paper, we report the occurrence of blood malignancies in five IBD patients, two affected by CD and three by UC. The possible relationship with underlying inflammatory disease was evaluated, taking into consider- ation the available literature data on this topic (Table 1). Case 1 R. D., a 78-year-old man with CD of the ileum and rectum was referred to our Gastroenterology Unit for the occur- rence of severe asthenia and dyspnea. CD was diagnosed 11 years before according to standard endoscopic and histological criteria. The patient was also affected by diabetes, which was treated by glibenclamide. After diagnosis, the disease showed a chronic active course and in the last 2 months, required the frequent administration of systemic steroids in addition to a maintenance treatment with 5-aminosalicylic acid (5-ASA), administered per os (2.4 g/die) and topically (4 g/die). At admission, the patient showed poor general condi- tions, mild fever (37–38°C), and complained of diarrhea (three to four bowel motions of liquid stools per day), abdominal pain, and rectal bleeding. The Crohn’ s disease activity index (CDAI) was 364. Int J Colorectal Dis (2007) 22:553–558 DOI 10.1007/s00384-006-0202-x P. Crispino : R. Pica : E. Angelucci : A. Consolazio : M. Rivera : C. Cassieri : P. Paoluzi (*) Gastroenterology Unit, Department of Clinical Sciences, University “La Sapienza”, Policlinico Umberto I, Viale del Policlinico 157, Rome 00161, Italy e-mail: paolo.paoluzi@uniroma1.it