Takashi Sunagawa

Successful treatment of a non‐haemophilic patient with inhibitor to factor VIII by double‐filtration plasmapheresis

Factor VIII (FVIII) inhibitors appear in 3–20% of haemophilia A patients after injection of FVIII concentrates. However, autoantibodies to FVIII are also reported in non‐haemophiliacs. In these patients FVIII inhibitor disappears spontaneously or diminishes in response to immunosuppressive therapy. However, a few patients show resistance to immunosuppressive therapy. We describe a non‐haemophilic elderly patient with acquired FVIII inhibitor who failed to respond to prednisolone. He was treated with double‐ filtration plasmapheresis (DFPP) which resulted in a very rapid reduction in inhibitor levels and resolution of symptoms.

Nodular regenerative hyperplasia of the liver and Castleman's disease: potential role of interleukin-6.

A 45-year-old man presented in September 1999, with a 9-year history of weakness, fatigue, intermittent fever, and liver dysfunction. He had had muscle atrophy of the right arm due to cervical spondylosis. On examination, he was afebrile and had nontender cervical lymphadenopathy and splenomegaly. Laboratory evaluation revealed an increased erythrocyte sedimentation rate (91 mm/hr) and C-reactive protein (73.4 mg/L), with a normal white blood cell count (6.4 × 109/L). Other values were as follows—hemoglobin, 11.8 g/dL; platelet count, 17.9 × 109/L; alkaline phosphatase, 1020 IU/L (normal value [NV], 80–260); γ -glutamyl transpeptidase, 102 IU/L (NV, 10– 65)—and the rest of the liver function tests were normal. He had hyperglobulinemia: total protein was 82 g/L (NV, 67–83), of which 35 g/L was albumin, and the serum immunoglobulin showed an excess of polyclonal IgG at 29.9 g/L (NV, 8.7– 17), IgM at 2.1 g/L (NV, 0.3–1.9), and IgA at 7.36 g/L (NV, 1.1–4.1). Serum markers of viral, autoimmune, and metabolic causes of liver dysfunction were negative. A liver biopsy was performed, which showed the nodular formation displacing the normal liver parenchyma and fibrous expansion of the portal tract with chronic inflammatory infiltrate (Figure 1). These findings were consistent with NRH of the liver. Infectious diseases

HLA class-I-restricted and colon-specific cytotoxic T cells from lamina propria lymphocytes of patients with ulcerative colitis.

We established cytotoxic T-lymphocyte (CTL) lines against colonic epithelial cell line from LPLs of patients with ulcerative colitis by continuous stimulation with human lymphocyte antigen A (HLA-A) matched colonic epithelial cell lines and clones from LPLs using polyclonal stimulation with phytohemagglutinin. The established CTL lines and clones showed cytotoxicity against HLA-A-matched colonic epithelial cell line but not against HLA-mismatched colonic epithelial cell lines, and HLA-A-matched lung and esophagus cell lines. The CTL response was HLA class I-restricted and mediated by CD8-positive T-lymphocytes. Moreover, the CTL line showed cytotoxicity against autologous B-LCLs pulsed with peptides extracted from HLA-A-matched colonic epithelial cell line but not against other organ-derived peptides pulsed and unpulsed autologous B-LCLs. CTL lines and clones established from LPLs of patients with ulcerative colitis showed colon-specific and HLA class I-restricted killing of human colonic epithelial cell line, suggesting that these CTLs may play a role in colonic epithelial cell damage in some patients with ulcerative colitis.

Bamboo trees in the stomach

A 46 year old man presented with general fatigue and abdominal discomfort. He had a surgical history of ileal perforation at the age of 40 years. …

Gastrointestinal: Aphthous lesions to overt Crohn's disease

The word aphthous is derived from the Greek word aptha and can be translated as an inflamed spot. The terms aphthous ulcers and aphthous lesions were initially used to describe small superficial ulcers in the mouth but were subsequently used to describe small ulcers in any part of the gastrointestinal tract. Although aphthous lesions have been regarded as early features of Crohn’s disease, they may occur in a variety of other clinical settings including infectious enterocolitis. In Crohn’s disease of the small bowel, aphthous lesions typically occur over Peyer’s patches while in the large bowel, lesions frequently occur over lymphoid aggregates. As the disease progresses, aphthous lesions can enlarge and coalesce, initially forming stellate ulcers and subsequently forming deeper longitudinal and transverse linear ulcers. Islands of mucosa not affected by ulceration can create a cobblestone appearance. The typical endoscopic appearance of an aphthous lesion is a superficial ulcer, 1–5 mm in diameter, with surrounding erythema. Although progression of Crohn’s disease is widely believed to follow the above pattern, radiological documentation of progression is uncommon. The patient illustrated below was a 13-year-old boy who presented with anorectal fistulas. Blood tests revealed mild anemia with an elevated erythrocyte sedimentation rate and C-reactive protein. A double-contrast barium enema study (Fig. 1) showed (Æ) a small number of aphthous lesions in the ascending colon. He was treated with an elemental diet. After an interval of 21 months, he began to lose weight and developed an intermittent fever. A repeat barium enema X-ray (Fig. 2) showed abnormalities in the ascending colon and transverse colon with narrow areas, eccentric rigidity, nodularity and longitudinal ulcers. The appearance was typical of Crohn’s disease.

Gastrointestinal: Duodenal Crohn's disease

Contributed by Drs A Hokama, F Kinjo, R Sugama, K Kobashigawa, R Matayoshi, Y Yonamine, R Tomiyama, T Sunagawa, M Kawane and A Saito, First Department of Internal Medicine, Department of Endoscopy, University of the Ryukyus, 207 Uehara, Nishihara, Okinawa 903–0215, Japan. Contributions to the Images of Interest Section are welcomed and should be submitted to Professor IC RobertsThomson, Department of Gastroenterology, The Queen Elizabeth Hospital, Woodville South, South Australia 5011, Australia.