1/441. Low-dose cyclosporin A therapy in children with refractory immune thrombocytopenic purpura. Although splenectomy is the most effective treatment for chronic idiopathic thrombocytopenic purpura (ITP), many post-splenectomy patients have recurrent thrombocytopenia refractory to multiple medical therapies. Three consecutive patients with relapsed ITP after splenectomy and who were refractory to multiple medical therapies were treated with low dose cyclosporin A (CsA). In all 3 patients, the platelet count increased dramatically within 1 month from the onset of CsA therapy. The only detectable toxicity was hypomagnesemia and mild hypertension in 1 patient. CsA may be efficacious in treating patients with chronic ITP, which is refractory to all medical and surgical therapies currently being used. ( info) |
2/441. danazol therapy in cyclic acquired amegakaryocytic thrombocytopenic purpura: a case report. Cyclic acquired amegakaryocytic thrombocytopenic purpura (AATP) is a rare disorder characterized by periodic fluctuations in the platelet counts due to a defect in the platelet production. We describe a 42-year-old female with cyclic AATP, in whom the cyclic fluctuations in the platelet counts ceased with danazol therapy. The pathogenesis of the disease and the possible mechanisms of danazol action have been reviewed. ( info) |
3/441. Relationship between megakaryocyte mass and serum thrombopoietin levels as revealed by a case of cyclic amegakaryocytic thrombocytopenic purpura. Cyclic amegakaryocytic thrombocytopenic purpura is a rare syndrome characterized by periodic failure of megakaryocytopoiesis. In this report we describe a patient with cyclic amegakaryocytic thrombocytopenic purpura associated with a megakaryocyte specific serum IgG antibody, who responded to cyclosporin A therapy. Serial serum thrombopoietin assays during an episode of platelet cycling demonstrated a reciprocal relationship between serum thrombopoietin level and megakaryocyte mass, suggesting that megakaryocytes have an important role in the regulation of thrombopoietin metabolism. ( info) |
4/441. Autoimmune thrombocytopenic purpura and dermatomyositis associated with chronic hepatitis c. A therapeutic dilemma. We recently observed a patient with chronic C hepatitis who developed first autoimmune thrombocytopenic purpura and subsequently dermatomyositis. While the association could be coincidental, it is possible that hepatitis c virus could have induced autoantibodies or circulating immune complexes which contributed to both. Although concerns are sometimes raised about the use of corticosteroids and immunosuppressive therapy to control symptoms in hepatitis c virus infected patients with rheumatic or autoimmune diseases, corticosteroid and immunosuppressive therapies are considered appropriate in cases of chronic C hepatitis with rapidly progressive autoimmune diseases. Our case illustrates the paradox that, despite the continuing presence of a viremic form of chronic C hepatitis, clinical symptoms improved with combined immunosuppressive therapy, without deterioration of the hepatitis. Our results could be the consequence of the association of immunosuppressive therapy intensive enough to control the autoimmune thrombocytopenic purpura and dermatomyositis with alpha-interferon whose antiviral capacity may have been able to prevent re-exacerbation of the hepatitis. ( info) |
| We report a child with wiskott-aldrich syndrome with severe, refractory, symptomatic thrombocytopenia who achieved an excellent response to combination therapy with vincristine 1.5 mg/m(2) x 1 day, intravenous immunoglobulin 1 g/kg x 3 days, and methylprednisolone 25 mg/kg x 3 days (VIM) for 7 years after failing multiple treatments. He did not have a histocompatible donor for bone marrow transplantation. When the patient ceased to respond to this regimen, he was rescued with pulse dexamethasone. vincristine, immunoglobulin, and methylprednisolone might serve as a novel treatment option for the patient with refractory thrombocytopenia. Our patient had a sustained remission of symptomatic thrombocytopenia without toxicity. Furthermore, pulse dexamethasone might be an alternative treatment option to which patients with wiskott-aldrich syndrome may respond. ( info) |
6/441. Unwanted corticosteroid effects in childhood bone marrow failure, renal failure and brain damage: case report. The case report of the corticosteroid complication in an eight-year-old girl with immune thrombocytopenic purpura is presented. She was treated with high dosage corticosteroids and incurred severe side effects, including bone marrow depression, renal magnesium stones, osteoporosis, depression of affect, convulsions with cerebral damage and adrenal suppression. ( info) |
7/441. Human granulocytic ehrlichiosis presenting with acute renal failure and mimicking thrombotic thrombocytopenic purpura. A case report and review. We present the case of an elderly female patient presenting with recurrent acute renal failure, fever, altered mental status, abdominal pain, thrombocytopenia and a small number of fragmented red cells on peripheral smear mimicking recurrent thrombotic thrombocytopenic purpura (TTP). Eventually, however, she was diagnosed to have human granulocytic ehrlichiosis (HGE), and after treatment for HGE her clinical and laboratory abnormalities resolved. ehrlichiosis mimicking TTP, diagnosed at postmortem examination, has been described in a single prior case. As illustrated in this case, there are potential difficulties in diagnosing HGE after plasma exchange, blood transfusion and immunosuppressive therapy. ehrlichiosis, a potentially curable disease, should be considered in the differential diagnosis of thrombotic microangiopathic disorders. copyright copyright 1999 S. Karger AG, Basel ( info) |
8/441. Spontaneous gingival bleeding in an otherwise asymptomatic patient. This case is presented to challenge the reader to formulate a differential diagnosis for a patient who visits the dentist with spontaneous, continuous gingival bleeding. When this situation occurs, it is serious and requires immediate attention and a specific treatment plan to arrive at the underlying diagnosis and control the bleeding. The signs and symptoms of a patient with gingival bleeding are presented for diagnosis; the history and management are detailed, and may be useful in diagnosing and treating similar patients. ( info) |
9/441. vitrectomy for vitreous bleeding and tractional retinal detachment in a case of Evans syndrome. PURPOSE: To evaluate the effect of vitrectomy to treat Evans syndrome with vitreous bleeding followed by tractional retinal detachment. CASE: vitrectomy was performed on a 21-year-old man with Evans syndrome who developed tractional retinal detachment in the left eye after experiencing vitreous bleeding and tissue proliferation related to the bleeding. OBSERVATIONS: After the proliferative tissue was removed and tractional retinal detachment corrected, the patient's visual acuity improved from 6/20 to 10/20. A small amount of residual proliferative tissue remained after the vitrectomy. CONCLUSIONS: vitrectomy may be beneficial in patients with serious vitreous complications induced by hematological diseases such as Evans syndrome. ( info) |
| A very unusual clinical presentation of hodgkin disease with immune thrombocytopenia and autoimmune hemolytic anemia is reported. A 6.5-year-old boy presented with thrombocytopenia, Coombs' positive hemolytic anemia, and multiple small posterior cervical lymph nodes. After a course of high-dose methylprednisolone therapy with a diagnosis of Evans syndrome, complete response for thrombocytopenia and partial response for anemia was achieved. Six weeks later there was a sudden increase in the size of left posterior cervical lymph nodes and a biopsy was compatible with hodgkin disease, mixed cellularity type. The child was successfully treated with chemotherapy and radiation therapy. He has been off therapy for 28 months and has no clinical or laboratory evidence of autoimmune cytopenia. A combination of immune thrombocytopenia and autoimmune hemolytic anemia may be associated with hodgkin disease. The recognition of this clinical picture as a complication of hodgkin disease has important implications. This complication appeares to be managed best by the definitive treatment of hodgkin disease. ( info) |