High-dose Immunoglobulin Infusion for Thrombotic Thrombocytopenic Purpura Refractory to Plasma Exchange and Steroid Therapy

The outcomes of the treatment of thrombotic thrombocytopenic purpura (TTP) have been shown to be improved by the administration of plasma exchange. However, treatment options are currently limited for cases refractory to plasma exchange. The autoantibodies that block the activity of ADAMTS13 have been demonstrated to play a role in the pathogenesis of TTP; therefore, high-dose immunoglobulin, which can neutralize these autoantibodies, may be useful for refractory TTP. However, successful treatment with high-dose immunoglobulin for TTP refractory to plasma exchange and corticosteroids has yet to be reported in Korea. Herein, we describe a refractory case which was treated successfully with high-dose immunoglobulin. A 29-year-old male diagnosed with TTP failed to improve after plasma exchange coupled with additional high-dose corticosteroid therapy. As a salvage treatment, we initiated a 7-day regimen of high-dose immunoglobulin (400 mg/kg) infusions, which resulted in a complete remission, lasting up to the last follow-up at 18 months. High-dose immunoglobulin may prove to be a useful treatment for patients refractory to plasma exchange; it may also facilitate recovery and reduce the need for plasma exchange

Acute lymphoblastic leukemia with a variant Philadelphia translocation, der(9), and der(19) chromosomes

We report here one of 15 cases of acute lymphoblastic leukemia (ALL) cytogenetically studied, with hypodiploidy, a variant Ph translocation, and der (9) and der (19) chromosomes. The patient, a 14-year-old girl, underwent combination chemotherapy and bone marrow transplantation and is still in remission 22 months after transplantation. © 1995

The nature of small intestinal mucositis: A video-capsule endoscopy study

Background and aims: Alimentary mucositis is a life-threatening side effect in patients receiving conditioning therapy for stem cell transplantation. While oral mucosa can be easily inspected, there are no endoscopic data on small intestinal mucositis. The aim of our study was to investigate small intestinal lesions using video-capsule endoscopy in patients undergoing peripheral blood stem cells transplantation. Materials and methods: We prospectively studied five consecutive patients who underwent peripheral blood stem cells transplantation: three patients (four studies) without concomitant recombinant human keratinocyte growth factor (palifermin) treatment and two patients with palifermin treatment. We performed video-capsule endoscopy studies 7 days after cells infusion, when transplant patients usually have severe abdominal symptoms. Results: In three patients who did not receive palifermin, video-capsule endoscopy revealed extensive confluent ulcerations with bleeding spots in the small intestine. Two patients had lesions in the jejunum and in the ileum. One of them underwent repeat video-capsule endoscopy 6 days later that showed significant healing of the lesions, but still there were diffuse edema of small intestinal mucosa, erosions and scarce ulcerations. In the third patient, the capsule remained in the stomach for 7.5 h but showed duodenal erosions with bleeding spots. Both patients on palifermin treatment had normal examinations. All studies were uneventful. Conclusion: Our data reveal for the first time that small intestinal mucositis in patients undergoing conditioning therapy for peripheral blood stem cells transplantation presents as small intestinal ulcerations and that palifermin treatment prevents these lesions. © 2008 Springer-Verlag