Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Immune thrombocytopenic purpura (ITP), alone or in combination with autoimmune hemolytic anemia (Evans syndrome) and/or autoimmune neutropenia, is frequent in patients with common variable immunodeficiency (CVID). A 34-year-old man with CVID had long-standing unresponsive ITP. The patient had a 9-year history of CVID on substitutive therapy with intravenous immunoglobulin (IVIG). The clinical course of CVID was complicated with refractory fistulizing inflammatory bowel disease, nodular regenerative hyperplasia of the liver, splenomegaly, severe portal hypertension, and hypercatabolism of IgG. ITP was refractory to medical therapy, including different combinations of corticosteroids, high-dose IVIG, azathioprine, and vincristine. Splenectomy was not performed because of severe portal hypertension. He received a total five doses of rituximab, a monoclonal antibody directed against CD20 antigen, at a dose of 375 mg/m(2). After an initially slow response, his platelet count increased to more than 50,000/microL by the fourth week of infusion. Therapy was well tolerated, and B lymphocytes were effectively depleted from the peripheral blood. The patient was completely tapered off glucocorticoids and maintained platelets at above 40,000/microL. The patient has not taken immunosuppressive agents for 11 months. Early treatment with rituximab might be an option for patients with CVID and ITP that do not respond to other treatments or for patients for whom a splenectomy is contraindicated.
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PMID:Partial response to anti-CD20 monoclonal antibody treatment of severe immune thrombocytopenic purpura in a patient with common variable immunodeficiency. 1612 7

We report a case of successfully treated acute thrombocytopenia associated with preexisting ulcerative colitis (UC). The patient had typical symptoms of UC, and colonoscopy showed pancolitis. During treatment with sulfasalazopyridine (SASP) and steroids, thrombocytopenia was observed. Despite the cessation of drugs, severe thrombocytopenia was noted. Immune thrombocytopenic purpura (ITP) was suspected based on a normal bone marrow megakaryocyte count, positive autoantibody to platelet membrane antigen, and the absence of splenomegaly. Medical treatment, including increased dosage of steroids, failed to control UC and acute thrombocytopenia in this patient. Moreover, acute severe pancreatitis developed and abdominal computed tomography showed toxic megacolon. Platelet count recovered after urgent total colectomy without splenectomy. When patients with UC develop thrombocytopenia, particularly in the presence of extensive and significant colonic inflammation, a diagnosis of ITP should be considered. In such patients, preexisting UC might be involved in the immunological causal mechanism of ITP. In this situation, colectomy might cure both UC and resistant thrombocytopenia. Steroid-refractory and life-threatening UC complicated by thrombocytopenia presumably caused by ITP is therefore a possible indication for colectomy.
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PMID:Acute thrombocytopenia associated with preexisting ulcerative colitis successfully treated with colectomy. 1648 45