31/123. Inflammatory bowel disease associated with immune thrombocytopenic purpura in children.OBJECTIVE: Previous reports suggest an association between inflammatory bowel disease (IBD) and immune thrombocytopenic purpura (ITP) in adults. To date, only five children with both diseases have been described. The aim of the study was to describe the characteristics of children with IBD and ITP. methods: Cases were obtained from the pediatric gastroenterology community by means of the pediatric gastroenterology internet bulletin board in June 1999. Eight cases were submitted from seven medical centers. medical records were reviewed by two pediatric gastroenterologists and a pediatric hematologist. RESULTS: The age range of the patients was 2.1 to 16.5 years, with a mean age of 9.6 /- 5.2 years. Four children had ulcerative colitis, three had crohn disease, and one had indeterminate colitis. All had colonic involvement of IBD. Of eight patients, three had IBD first, three had ITP first, and two had both simultaneously. At ITP diagnosis, platelet count was less than 10,000/mL in five children, 17,000/mL in one child, and 50,000 to 60,000/mL in two children. Of the three children diagnosed with ITP first, two initially had rectal bleeding at the time of ITP diagnosis. bone marrow evaluations, performed in six of eight children, were consistent with ITP. Six of the eight children had chronic ITP, including three children who were 5 years of age or younger. Therapy for ITP included steroids (n = 6), intravenous immunoglobulin (n = 6), Rh o (D) intravenous immunoglobulin (n = 2), and splenectomy (n = 1). CONCLUSIONS: The authors describe the largest pediatric case series of children with IBD and ITP. More than 50% of the children had the chronic form of ITP. Most patients responded to conventional therapy for ITP and IBD.- - - - - - - - - - ranking = 1keywords = disease (Clic here for more details about this article) |
32/123. Resolution of chronic idiopathic thrombocytopenia purpura following syngeneic peripheral blood progenitor transplant.Idiopathic thrombocytopenia purpura (ITP) is an acquired disease of children and adults characterized by a low platelet count, an essentially normal bone marrow, and absence of evidence for other disease. We report the use of syngeneic peripheral blood progenitor transplantation (PBPT) in a 19-year-old male with chronic refractory ITP since the age of 5. Engraftment was successful and has resulted in resolution of his disease. We conclude that syngeneic PBPT is a potentially curative option for refractory ITP.- - - - - - - - - - ranking = 0.42857142857143keywords = disease (Clic here for more details about this article) |
33/123. Spontaneous renal hemorrhage associated with idiopathic thrombocytopenic purpura--a case report.Spontaneous renal hemorrhage is a rare and complicated clinical condition. We report a case of spontaneous renal hemorrhage associated with idiopathic thrombocytopenic purpura (ITP). To our knowledge, this is a second case associated with ITP in the literature. The main treatment of our case included absolute bed rest, platelet transfusion, steroid therapy and prevention of infection. The platelet count increased gradually and returned to the safe level greater than 50,000/microliter from the 10th day. The patient was discharged after 2 weeks due to stable condition and received further follow-up in the outpatient department. We suggest that conservative treatment for spontaneous renal hemorrhage caused by blood dyscrasias like ITP is appropriate because immediate surgical intervention may pose a higher risk due to the tendency for severe bleeding. However, long term follow-up by serial CT should be performed to rule out the possibility of concomitant diseases especially small renal cell carcinoma, which is the most common cause of spontaneous renal hemorrhage.- - - - - - - - - - ranking = 0.14285714285714keywords = disease (Clic here for more details about this article) |
34/123. Laparoscopic accessory splenectomy with intraoperative gamma probe localization for recurrent idiopathic thrombocytopenic purpura.Laparoscopic excision of retained splenic tissue has been described as a treatment of recurrent hematologic disease after formal splenectomy. It is associated with a shorter hospital stay, more rapid recovery, and lower or equivalent morbidity compared with open surgery. However, intraoperative identification of residual splenic tissue remains difficult, particularly when preoperative computed tomography or magnetic resonance imaging results are unremarkable. It has been suggested that the laparoscopic approach has a lower success rate due to the loss of tactile feedback. We report a case of successful laparoscopic excision of retained splenic tissue using technetium sulfur colloid injection and intraoperative gamma probe localization in a patient with recurrent idiopathic thrombocytopenic purpura, 12 years after open splenectomy. This represents the first report of this intraoperative adjunctive measure for the laparoscopic identification and excision of functional accessory splenic tissue.- - - - - - - - - - ranking = 0.14285714285714keywords = disease (Clic here for more details about this article) |
35/123. melatonin for refractory idiopathic thrombocytopenic purpura: a report of 3 cases.In addition to its well-known influence on circadian rhythms, melatonin has been described as being able to increase the number of platelets in circulating blood. This provided the rationale to evaluate melatonin for toxicity and efficacy in three patients with idiopathic thrombocytopenic purpura (ITP) refractory to initial treatment with corticosteroids or splenectomy (refractory ITP). patients received melatonin for 1 month. The therapy was continued for 2 additional months in patients with stable or responding disease. After 3 months, the stable or responding patients continued the therapy for 3 months and more. All patients had a partial response after 1 month. Continuing with the treatment, none of the three patients had disease progression (average follow-up time of 31 months; range: 23-46 months). Toxicity was lacking, with the only side effect being drowsiness. Our experience suggests that melatonin may be safe and effective in patients with refractory ITP.- - - - - - - - - - ranking = 0.28571428571429keywords = disease (Clic here for more details about this article) |
36/123. Rituximab: an anti-CD20 antibody for the treatment of chronic refractory immune thrombocytopenic purpura.Immune thrombocytopenic purpura (ITP) is an autoimmune disease characterized by antibody-mediated platelet destruction. Despite initial response to corticosteroids, most adults relapse during steroid taper, and splenectomy is the treatment of choice for these patients. Those whom splenectomy fails to cure present a therapeutic challenge. Subsequent management usually involves some form of chronic immune suppression, which has serious side effects and long-term morbidity. Rituximab, a recently-approved anti-CD20 chimeric monoclonal antibody, has shown efficacy in preliminary studies. We report the cases of 3 patients with refractory ITP who achieved acceptable platelet counts after treatment with rituximab.- - - - - - - - - - ranking = 0.14285714285714keywords = disease (Clic here for more details about this article) |
37/123. Anti-Ku antibody-positive scleroderma-dermatomyositis overlap syndrome developing Graves' disease and immune thrombocytopenic purpura.Graves' disease (GD) has been reported to be frequently complicated with other autoimmune diseases. However, it is rarely complicated with scleroderma-polymyositis overlap syndrome. Recently, we encountered a 35-year-old woman who developed GD and immune thrombocytopenic purpura during follow-up observation of scleroderma-dermatomyositis overlap syndrome. Platelet counts recovered after high-dose gamma-globulin therapy and bolus methylprednisolone therapy. The present case is the first report of a combination of scleroderma, dermatomyositis, GD, and immune thrombocytopenic purpura. The patient was anti-Ku antibody-positive and had relatively low natural killer T cell counts, both of which might contribute to the complication of multiple autoimmune diseases.- - - - - - - - - - ranking = 1keywords = disease (Clic here for more details about this article) |
38/123. association of breast cancer and immune thrombocytopenic purpura.Immune thrombocytopenic purpura (ITP) and breast cancer are common disorders. Only six cases in which patients have had both diseases have been reported. We describe a 40-year-old woman who had ITP while responding to therapy for metastatic breast cancer. Given the few reported cases, the diverse presentations of thrombocytopenia during the course of each patient's breast cancer and the variable therapeutic responses of ITP, the association of breast cancer and ITP is probably coincidental.- - - - - - - - - - ranking = 0.14285714285714keywords = disease (Clic here for more details about this article) |
39/123. Effective B cell depletion with rituximab in the treatment of autoimmune diseases.In a pilot study four patients with systemic lupus erythematosus (SLE) and autoimmune thrombocytopenia (ITP) were treated with rituximab, a B cell depicting chimeric human/mouse anti-CD20 antibody. Treatment could be performed without serious side effects and resulted in a depletion of B cells from the peripheral blood for at least 4 months. Examination of one patient three months after treatment revealed a complete depletion of B cells in the bone marrow and in the spleen as well. The time point when peripheral B cells returned into the normal range varied between 8 months and over one year and could be observed also in the spleen. The follow up over more than 12 months revealed no significant treatment-associated side effects. Total immunoglobulin and specific antibody levels did not change except for one SLE-patient receiving additional immunosuppressive treatment including cyclophosphamide because of progressive disease. Clinical effectiveness cannot be judged by the small number of patients. However, one SLE patient with refractory severe thrombocytopenia had a very Favourable response with stable platelet numbers over 100.000/microl now for more than 6 months and disappearance of anti-dna antibodies. The treatment failure in another SLE patient could be due to the persistence of CD20-negative plasmablasts in peripheral blood which are not targeted by anti-CD20 treatment. Further studies are needed to assess the clinical benefit of B cell depletion in the treatment of autoimmune diseases.- - - - - - - - - - ranking = 0.85714285714286keywords = disease (Clic here for more details about this article) |
40/123. Severe bleeding symptoms in refractory idiopathic thrombocytopenic purpura: a case successfully treated with melatonin.The prognosis of patients with idiopathic thrombocytopenic purpura refractory to corticosteroids and splenectomy (refractory ITP) is poor; these patients present high morbidity from disease and its treatment and have a mortality rate of approximately 16%. A patient is reported with severe bleeding symptoms related to refractory ITP successfully treated with melatonin.- - - - - - - - - - ranking = 0.14285714285714keywords = disease (Clic here for more details about this article) |
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