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Evans syndrome is a rare disorder characterized by combined autoimmune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Standard treatments consist of transfusions, corticosteroids, splenectomy, Elite Kids Eugene Monroe jersey IVIG, anabolic steroids, vincristine, alkylating agents, or cyclosporine. In a patient with refractory disease, an allogeneic hematopoietic stem cell transplant (HSCT) resulted in complete clinical and serologic remission for more than 30 months. Allogeneic HSCT may be the only current curative therapy for Evans syndrome but may also be complicated by Authenitc Black Crockett Gillmore jersey significant toxicities. Symptoms first occurred in Authenitc Blue Dennis Pitta jersey February 1996 when epistaxis and ecchymosis developed following flu like symptoms of headache and diarrhea. Thrombocytopenia and anemia were present with platelets of 2 109 hemoglobin of 8.8 g and WBC of 6.3 109 Evidence of hemolysis included hyperbilirubinemia (3.8 mg an increased LDH (424 U decreased haptoglobin (7 mg reticulocytosis (corrected reticulocyte Elite Blue Kids Dennis Pitta jersey Dennis Pitta jersey Womens Crockett Gillmore jersey count 5.5%), bone marrow erythroid hyperplasia, and a direct Coombs test (DAT) positive for warm reactive anti IgG and anti C3D. Evidence for ITP included anti Crockett Gillmore jersey platelet antibodies and increased megakaryocytes within the marrow. Cytomegalovirus (CMV) antibody was positive for IgM but negative for IgG indicating recent infection. There was no other evidence of coincidental or precipitating infections. Serology was negative for HIV, hepatitis A, B, and C, and indicated past Epstein virus infection. Anti nuclear and anti double stranded DNA antibodies were negative.The patient was transplanted under IRB and FDA approved protocol IDE 6991. The transplant conditioning regimen was cyclophosphamide (200 mg and antithymocyte globulin (90 mg The allograft was unmanipulated bone marrow enriched with peripheral blood CD34+ cells from the patient’s HLA matched sister. CD34+ cell purification was performed utilizing Isolex (Nexell, Irvine, CA, USA) positive selection system. A total of 5.65 106 CD34+ cells were infused. Cyclosporine and corticosteroids were continued as GVHD Elite Blue Eugene Monroe jersey prophylaxis, while liposomal amphotericin B (three times aerosolized pentamidine, and azithromycin were continued as infection prophylaxis. WBC and platelet engraftment occurred on Authenitc Womens Brandon Williams jersey days 10 and 14, respectively. The initial post transplantation course was uncomplicated. RBC and platelet antibodies gradually disappeared over 3 months. Chimerism studies demonstrated 100% donor engraftment.Thrombotic thrombocytopenic purpura (TTP) developed 5 weeks after HSCT and resolved with plasmapheresis and discontinuation of cyclosporine. GVHD prophylaxis was changed to FK506 and low dose corticosteroids. TTP recurred and resolved Authenitc Blue Dennis Pitta jersey upon discontinuation of FK506. With no evidence of GVHD, TTP, immune mediated cytopenias, or infection, the patient was discharged home on mycophenolate mofetil and prednisone. Since the patient was unable to tolerate cyclosporine or FK505 due to TTP, while on only corticosteroid immune suppression, acute gastrointestinal grade IV GVHD occurred. Treatment consisted of high dose corticosteroid, oral beclomethasone, mycophenolate mofetil, and daclizumab.Extensive chronic cutaneous and gastrointestinal GVHD developed. Post transplant opportunistic infections have included Pseudomonas and Nocardia bacteremia, CMV retinitis, and persistent CMV reactivation. Platelet counts have been intermittently suppressed by medications and infections but recover to normal. Platelet antibodies and direct and indirect Coombs test remain negative without evidence of hemolysis. Currently, the skin has improved to normal and there are no GI symptoms on mycophenolate mofetil, prednisone, and infliximab. The last platelet transfusion was in August 2000 and RBC transfusion was 4 months ago. In severe Evans syndrome, treatment options are limited and the disease can be fatal.1 Although the literature is limited, Evans syndrome appears to be a more aggressive and fatal disease than either ITP or AIHA alone.2,3 Treatment for Evans syndrome is often ineffective. Of three patients treated with high dose cyclophosphamide (200 mg without stem cell support, only partial responses occurred and two died of relapsed disease and of new onset of ITP.9 In the published literature, 13 patients have received high dose cyclophosphamide and autologous HSCT. One died during mobilization; four patients had no response; two had a partial response; two had a complete response but clinically relapsed; one had a complete clinical remission but remains DAT (direct antiglobulin test) positive; and three remain in complete remission with short follow up of 6, 20, and 23 months.10,11,12,13,14,15,16,17,18 Elite Kids Eugene Monroe jersey These data suggest that the possibility of a durable remission or cure from high dose Authenitc Womens Brandon Williams jersey immune suppression with or without autologous HSCT for ITP, AIHA, or Evans syndrome is unlikely for the majority of patients.To our knowledge, this is the first adult case of primary Evans syndrome cured by allogeneic hematopoietic transplantation. Allogeneic HSCT using sibling matched umbilical cord blood,19 matched unrelated BM20 or matched sibling BM21 has been used to treat Evans syndrome, AIHA or pure Crockett Gillmore jersey red cell aplasia (PRCA), respectively. In none of the three cases did the disease relapse after allogeneic HSCT although follow up was short: 289 days, 18 months and Authenitc Chris Canty jersey 3 years, respectively. In our patient, there has been no evidence of immune mediated thrombocytopenia or hemolysis for 2 years and 6 months since the allogeneic transplant. The patient is 100% donor chimeric and antibodies to RBCs and platelets are negative. However, the post transplant course has been complicated by drug related TTP, chronic extensive GVHD, and immune suppression related opportunistic infections.1 Savasan S, Warrier I, Ravindranath Y. The spectrum of Evans’ syndrome. Arch Dis Child 1997; 77: 245 2 Portielje JEA, Westendorp RGJ, Kluin Nelemans HC, Brand A. Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood 2001; 97: 2549 3 Stasi R, Stipa E, Masi M et al. Long term observation of 208 adults with chronic idiopathic thrombocytopenic purpura. Am J Med 1995; 89: Elite Black Elite Kids Brandon Williams jersey Crockett Gillmore jersey 4364 Ng SC. Evans syndrome: a report on 12 patients. Clin Lab Haematol 1992; 14: 189 5 Mathew P, Chen G, Wang W. Evans syndrome: results of Authenitc Jeromy Miles jersey a national survey. J Pediatr Hematol 1997; 19: 4336 Wang WC. Evans syndrome in childhood: pathophysiology, clinical course, and treatment. Am J Ped Hematol 1998; 10: 3307 Shvidel L, Shtarlid M, Klepfish A et al. Evans syndrome complicating fludarabine treatment for advanced B CLL. Br J Haematol 1997; 99: 706, 8 Wang W, Herrod H, Pui CH et al. Immunoregulatory abnormalities in Evans syndrome. Am J Hematol 1983; 15: 381 9 Brodsky RA, Petri M, Smith BD et al. Immunoablative high dose cyclophosphamide without stem cell rescue for refractory, severe autoimmune disease. Ann Intern Med 1998; 129: 1031 10 Martino R, Sureda A, Brunet S. Peripheral blood stem cell mobilization in refractory autoimmune Evans syndrome: a cautionary case report. Bone Marrow Transplant 1997; 20: Crockett Gillmore jersey 521, 11 Musso M, Porretto F, Crescimanno A et al. Autologous peripheral blood stem and progenitor (CD34+) cell transplantation for systemic lupus erythematosus complicated by Evans syndrome. Lupus 1998; 7: 492 12 Skoda RC, Tichelli A, Tyndall A et al. Autologous peripheral blood stem cell transplantation in a patient with chronic autoimmune thrombocytopenia. Br J Haematol 1997; 99: 56 13 Marmont AM, van Lint MT, Occhini D et al. Failure of autologous stem cell transplantation in refractory thrombocytopenic purpura. Bone Marrow Transplant 1998; 22: 827 14 Lim SH, Kell J, Al Sabah A et al. Peripheral blood stem cell Kids Dennis Pitta jersey transplantation for refractory autoimmune thrombocytopenic Mens Dennis Pitta jersey purpura. Lancet 1997; 349: 475, 15 Demirer T, Celebi H, Arat M et al. Autoimmune thrombocytopenia in a patient with small cell lung cancer developing after chemotherapy and resolving autologous peripheral blood stem cell transplantation. Bone Marrow Transplant 1999; 24: 335 16 Huhn RD, Read EJ, Rick M et al. Intensive immunosuppression with high dose cyclophosphamide and autologous CD34+ selected hematopoietic cell support for chronic refractory autoimmune thrombocytopenia (interim report). Blood 1998; 92 (Suppl.): 178 (Abstr. 719),17 Pillard C, Kanold J, Halle P et al. Two step immunoablative treatment with autologous peripheral blood CD34+ cell transplantation in an 8 year old boy with autoimmune haemolytic anaemia. Br J Haematol 2000; 110: 900 18 Jindra P, Koza V, Fiser J et al. Autologous CD34+ cell transplantation after FAMP treatment in patient with CLL and persisting AIHA: complete remission of lymphoma with control of autoimmune complications. Bone Marrow Transplant 1999; 24: 215 19 Raetz E, Beatty PG, Adams RH. Treatment of severe Evans syndrome with an allogeneic cord blood transplant. Bone Marrow Transplant 1997; 20: 427 20 De Stefano P, Zecca M, Giorgiani G et al. Resolution of immune hemolytic anaemia with allogeneic bone marrow transplantation after unsuccessful autograft. Br J Haematol 1999; 106: 1063 21 Muller BU, Tichelli A, Passweg JR et al. Successful treatment of refractory acquired pure red cell aplasia (PRCA) by allogenic bone marrow transplantation. Bone Marrow Transplant 1999; 23: 1205.Evans syndrome is a rare disorder characterized by combined autoimmune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Standard treatments consist of transfusions, corticosteroids, splenectomy, IVIG, anabolic steroids, vincristine, alkylating agents, or cyclosporine. In a patient with refractory disease, an allogeneic hematopoietic stem Elite Dennis Authenitc Kids Chris Canty jersey Pitta jersey cell transplant (HSCT) resulted in complete clinical and serologic remission for more than 30 months. Allogeneic HSCT may be the only current curative therapy for Evans syndrome but may also be complicated by significant toxicities. Symptoms first occurred in February 1996 when epistaxis and ecchymosis developed following flu like symptoms of headache and diarrhea. Thrombocytopenia and anemia were present with platelets of 2 109 hemoglobin of 8.8 g and WBC of 6.3 109 Evidence of hemolysis included hyperbilirubinemia (3.8 mg an increased LDH (424 U decreased haptoglobin (7 mg reticulocytosis (corrected reticulocyte count 5.5%), bone marrow erythroid hyperplasia, and a direct Coombs test (DAT) positive for warm reactive anti IgG and anti C3D. Evidence for ITP included anti platelet antibodies and increased megakaryocytes within the marrow. Cytomegalovirus (CMV) antibody was positive for IgM but negative for IgG indicating recent infection. There was no other evidence of coincidental or precipitating infections. Serology was negative for HIV, hepatitis A, B, and C, and indicated past Epstein virus infection. Anti nuclear and anti double stranded DNA antibodies were negative.The patient was transplanted under IRB and FDA approved protocol Elite Mens Dennis Pitta jersey IDE 6991. The transplant conditioning regimen was cyclophosphamide (200 mg and antithymocyte globulin (90 mg The allograft was unmanipulated bone marrow enriched with peripheral blood CD34+ cells from the patient’s HLA matched sister. CD34+ cell purification was performed utilizing Isolex (Nexell, Irvine, CA, USA) positive selection system. A total of 5.65 106 CD34+ cells were infused. Cyclosporine and corticosteroids were continued as GVHD prophylaxis, while liposomal amphotericin B (three times aerosolized pentamidine, and azithromycin were continued Elite Blue Brandon Williams jersey as infection prophylaxis. WBC and platelet engraftment occurred on days 10 and 14, respectively. The initial post transplantation course was uncomplicated. RBC and platelet antibodies gradually disappeared over 3 months. Chimerism studies demonstrated 100% donor engraftment.Thrombotic thrombocytopenic purpura (TTP) developed 5 weeks after HSCT and resolved with plasmapheresis and discontinuation of cyclosporine. Mens Eugene Monroe jersey GVHD prophylaxis was changed to FK506 and low dose corticosteroids. TTP recurred and resolved upon discontinuation of FK506. With no evidence of GVHD, TTP, immune mediated cytopenias, or infection, the patient was discharged home on mycophenolate mofetil and prednisone. Since the patient was unable to tolerate cyclosporine or FK505 due to TTP, while on only corticosteroid immune suppression, acute gastrointestinal grade IV GVHD occurred. Treatment consisted Authenitc Womens Chris Canty jersey of high dose corticosteroid, oral beclomethasone, mycophenolate mofetil, Authenitc Blue Brandon Williams jersey and daclizumab.Extensive chronic cutaneous and gastrointestinal GVHD developed. Post transplant opportunistic infections have included Pseudomonas and Nocardia bacteremia, CMV retinitis, and persistent CMV reactivation. Platelet counts have been intermittently suppressed by medications and infections but recover to normal. Platelet antibodies and direct and indirect Coombs test remain negative without evidence of hemolysis. Currently, the skin has improved to normal and there are no GI symptoms on mycophenolate mofetil, prednisone, and infliximab. The last platelet transfusion was in August 2000 and RBC transfusion was 4 months ago. In severe Evans syndrome, treatment options are limited and the disease can be fatal.1 Although the literature is limited, Evans syndrome appears to be a more aggressive and fatal disease than either ITP or AIHA alone.2,3 Treatment for Evans syndrome is often ineffective. Of three patients treated with high dose cyclophosphamide (200 mg without stem cell support, only partial responses occurred and two died Jeromy Miles jersey of relapsed disease and of new onset of ITP.9 In the published literature, 13 patients have Kids Jeromy Miles jersey received high dose cyclophosphamide and autologous HSCT. One died during mobilization; four patients had no response; two had a partial response; two had a complete response but clinically relapsed; one had a complete clinical remission but remains DAT (direct antiglobulin test) positive; and three remain in complete remission with Authenitc Mens Dennis Pitta jersey short follow up of 6, 20, and 23 months.10,11,12,13,14,15,16,17,18 These data suggest that Authenitc Mens Crockett Gillmore jersey the possibility of a durable remission or cure from high dose immune suppression with or without autologous HSCT for ITP, AIHA, or Evans syndrome is unlikely for Authenitc Brandon Williams jersey the majority of patients.To our knowledge, this is Youth Jeromy Miles jersey the first adult case of primary Evans syndrome cured by allogeneic hematopoietic transplantation. Allogeneic HSCT using sibling matched umbilical cord blood,19 matched unrelated BM20 or matched sibling BM21 has been used to treat Evans syndrome, AIHA or pure red cell aplasia (PRCA), respectively. In none of the three cases did the disease relapse after allogeneic HSCT although follow up was short: 289 days, 18 months and 3 years, respectively. In our patient, there has been no evidence of immune mediated thrombocytopenia or hemolysis for 2 years and 6 months since the allogeneic transplant. The patient is 100% donor chimeric and antibodies to RBCs and platelets are negative. However, the post transplant course has been complicated by drug related TTP, chronic extensive GVHD, and immune suppression related opportunistic infections.1 Savasan S, Warrier I, Ravindranath Y. The spectrum of Evans’ syndrome. Arch Dis Child 1997; 77: Authenitc Youth Chris Canty jersey 245 2 Portielje JEA, Westendorp RGJ, Kluin Nelemans HC, Brand A. Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood 2001; 97: 2549 3 Stasi R, Stipa E, Masi M et al. Long term observation of 208 adults with chronic idiopathic thrombocytopenic purpura. Am J Med 1995; 89: 4364 Ng SC. Evans syndrome: a report on 12 patients. Clin Kids Crockett Gillmore jersey Lab Haematol 1992; 14: 189 5 Mathew P, Chen G, Wang W. Evans syndrome: results of a national survey. J Pediatr Hematol 1997; 19: 4336 Wang WC. Evans syndrome in childhood: pathophysiology, clinical course, and treatment. Am J Ped Hematol 1998; 10: 3307 Shvidel L, Shtarlid M, Klepfish A et al. Evans syndrome complicating fludarabine treatment for advanced B CLL. Br J Haematol 1997; 99: 706, 8 Wang W, Herrod H, Pui CH et al. Immunoregulatory abnormalities in Evans syndrome. Am J Hematol 1983; 15: 381 9 Brodsky RA, Petri M, Smith BD et al. Immunoablative high dose cyclophosphamide without stem cell rescue for refractory, severe autoimmune disease. Ann Intern Med 1998; 129: 1031 10 Martino R, Sureda A, Brunet S. Peripheral blood stem cell mobilization in Elite Youth Brandon Williams jersey refractory Kids Eugene Monroe jersey autoimmune Evans syndrome: a cautionary case report. Bone Marrow Transplant 1997; 20: 521, 11 Musso M, Porretto F, Crescimanno A et al. Autologous Elite Black Brandon Williams jersey peripheral blood stem and progenitor (CD34+) cell transplantation for systemic lupus erythematosus complicated by Evans syndrome. Lupus 1998; 7: 492 12 Skoda RC, Tichelli A, Tyndall A et al. Autologous peripheral blood stem cell transplantation in a patient with chronic autoimmune thrombocytopenia. Br J Haematol 1997; 99: 56 13 Marmont AM, van Lint MT, Occhini D et al. Failure of autologous stem cell transplantation in refractory thrombocytopenic purpura. Bone Marrow Transplant 1998; 22: 827 14 Lim SH, Kell J, Al Sabah A et al. Peripheral blood stem cell transplantation for refractory Mens Chris Canty jersey autoimmune thrombocytopenic purpura. Lancet 1997; 349: 475, 15 Demirer T, Celebi H, Arat M et al. Autoimmune thrombocytopenia in a patient with small cell lung cancer developing after chemotherapy and resolving autologous peripheral blood stem cell transplantation. Bone Marrow Transplant 1999; 24: 335 16 Huhn RD, Read EJ, Rick M et al. Intensive immunosuppression with high dose cyclophosphamide and autologous CD34+ selected hematopoietic cell support for chronic refractory autoimmune thrombocytopenia (interim report). Blood 1998; 92 (Suppl.): 178 (Abstr. 719),17 Pillard C, Kanold J, Halle P et al. Two step immunoablative treatment with autologous peripheral blood CD34+ cell transplantation in an 8 year old boy with autoimmune haemolytic anaemia. Br J Haematol 2000; 110: 900 18 Jindra P, Koza V, Fiser J et al. Autologous CD34+ cell transplantation after FAMP treatment in patient with CLL and persisting AIHA: complete remission of lymphoma with control of autoimmune complications. Bone Marrow Transplant Authenitc Womens Brandon Williams jersey 1999; 24: 215 19 Raetz E, Beatty PG, Adams RH. Treatment of severe Evans syndrome with an allogeneic cord blood transplant. Bone Marrow Transplant 1997; 20: 427 20 De Stefano P, Zecca M, Giorgiani G et al. Resolution of immune hemolytic anaemia with allogeneic bone marrow transplantation after unsuccessful autograft. Br J Haematol 1999; 106: 1063 21 Muller BU, Tichelli A, Passweg JR et al. Successful treatment of refractory acquired pure red cell aplasia (PRCA) by allogenic bone marrow transplantation. Bone Marrow Transplant 1999; 23: 1205.
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Whether you a fan of the game or just of game day food, there no denying the appeal of football cuisine. For this month recommendations, I set out to assemble an all star lineup of the best of Food Network Library tailgating cookbooks. I fast found myself slipping down a rabbit hole into a vast and unfamiliar world of community cookbooks devoted to collegiate tailgating a world where the NCAA begins to look like one massive Junior League that as devoted to recipes as to pass receptions. It was amazing how numerous these cookbooks turned out to be Youth Tuukka Rask Authenitc Jeremy Lauzon Jersey Jersey (ripe terrain for a collector, for sure). To name just a few: Tar Heel Tailgating (University of North Carolina), Purdue Alumnus Tailgate Recipe Cookbook, University of Texas Longhorns Cookbook, Teatime to Tailgates (Kansas State University), Rocky Top Saturdays (University of Tennessee) and Men Reilly Smith Jersey my favorite (in title, at least) Let the Big Dawg Eat (University of Georgia). Now, I would love to recommend three or four of these books from the library
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