Severe gastrointestinal disease development coinciding with the progression of myelodysplastic syndrome after a long‐term mild course of Behcet's disease
Y. Takizawa, K. Setoguchi, Takeshi Kobayashi
Nov 1, 2017
Citations
4
Citations
Quality indicators
Journal
International Journal of Rheumatic Diseases
Full text
Semantic Scholar
Key Takeaway Key Takeaway: A long-term mild course of Behcet's disease can lead to severe gastrointestinal complications, with gastrointestinal BD being a potential cause of gastric ulcer development.
Abstract
Dear Editor, A 70-year-old man began to suffer from recurrent manifestations of aphthous stomatitis. Erythema nodosum and pseudofolliculitis began to appear on the legs and face, with repeated spontaneous remission and recurrence. They were occasionally accompanied by fever. With a positive pathergy test, he was diagnosed with Behcet’s disease (BD) based on the revised international criteria for BD. His human leukocyte antigen (HLA) typing was A24, B35, B75. Because his symptoms were mild, nonsteroidal anti-inflammatory drugs (NSAIDs), topical glucocorticoids and topical analgesics were given in compliance with his wishes. As for laboratory results, mild normocytic anemia with a hemoglobin (Hb) level of 9–10 g/dL was continuously observed, which was attributed to recurrent inflammatory attacks. After the mild clinical course had lasted for 6 years, the patient came to the emergency room because of a sudden episode of hematemesis. Hb had dropped from 10.1 g/dL at the last visit to 5.8 g/dL and his blood pressure was 94/50 mmHg, accompanied by tachycardia. Gastric endoscopy identified pulsating bleeding from a sharply demarcated gastric ulcer (Fig. 1a), for which hemostasis was achieved by ethanol injection and clipping. The second-look endoscopy performed on the next day confirmed the hemostasis; in addition, two small ulcers with a round, sharply demarcated border were seen in the lower esophagus (Fig. 1b), which were morphologically consistent with BD. Their relevant differential diagnoses, gastric acid-induced ulcer, microorganism infection and retention of certain drugs, were unlikely. Thus, although gastric ulcer development can be attributed to the occasional use of NSAIDs, gastrointestinal (GI) BD was suspected from the overall clinical picture of the ulcers. All the ulcers healed with bowel rest, a special diet and the use of proton pump inhibitors. After anemia had been treated, the patient was discharged. Three months later, he visited the emergency room again with a complaint of severe dyspnea. Close examination showed that he had left-sided heart failure, which had developed in association with severe anemia. The complete blood count was: white blood cell (WBC) 3800/lL (neutrophils 2500, lymphocytes 1000); hemoglobin 5.5 g/dL; and platelets 21.5 9 10/lL. The ulcers had almost healed on gastric endoscopy; furthermore, no abnormality on colon endoscopy or iron deficiency on a blood test was observed. To investigate the cause of severe anemia, bone marrow examination was performed, which revealed morphological abnormalities in all three lineages with a mild increase of blasts (7%). He was diagnosed with myelodysplastic syndrome (MDS)refractory anemia with an excess of blasts (RAEB)-1. Chromosome banding revealed a complicated karyotype: 45XY, add(2)(q11.2), der (2)add(2)(p11.2)add (2)(q33), -5, add(6)(q21), add(7)(p11.2). Considering his advanced age and expected poor prognosis, supportive therapy such as blood transfusions was selected. Four months later, the patient came to the emergency room because of bloody stools. His vital signs were near a shock state. Emergent colon endoscopy revealed multiple small ulcers in the terminal ileum (Fig. 1c) and an ileocecal, sharply-demarcated, bleeding ulcer with an exposed vessel (Fig. 1d), which were typical lesions of GI BD. Intestinal tuberculosis, nonspecific chronic ulcers of the small intestine and lymphoma were considered as differential diagnoses, but they were unlikely, judging from the clinical course and other laboratory and radiological examinations. Clipping of the vessel achieved hemostasis. Because severe bleedings due to GI BD successively occurred within 6 months, specific treatment was thought to be required. After prednisolone (PSL) at 60 mg/day was given for 3 days, PSL at 20 mg/day was