mycophenolic-acid and Remission--Spontaneous

Henoch-Schönlein purpura, now called immunoglobulin A (IgA) vasculitis, is a systemic, immune complex-mediated, small-vessel leukocytoclastic vasculitis characterized by nonthrombocytopenic palpable purpura, arthritis, and abdominal pain. It is the most common vasculitis in children but can also occur in adults. Diagnostic testing is required only to exclude other etiologies of purpura, to identify renal involvement, and, if indicated, to determine its extent with biopsy. Imaging or endoscopy may be needed to assess organ complications. IgA vasculitis spontaneously resolves in 94% of children and 89% of adults, making supportive treatment the primary management strategy. However, a subset of patients experience renal involvement that can persist and relapse years later. Additional complications can include gastrointestinal bleeding, orchitis, and central nervous system involvement. Systematic reviews have shown that steroids do not prevent complications and should not be used prophylactically. However, randomized trials have demonstrated success with high-dose steroids, cyclosporine, and mycophenolate in treating glomerulonephritis and other complications. Long-term prognosis depends on the extent of renal involvement. Six months of follow-up is prudent to assess for disease relapse or remission.

Topics: Acetaminophen; Adrenal Cortex Hormones; Adult; Analgesics, Non-Narcotic; Anti-Inflammatory Agents, Non-Steroidal; Arthralgia; Child; Cyclosporine; Disease Progression; Glomerulonephritis; Humans; IgA Vasculitis; Immunosuppressive Agents; Mycophenolic Acid; Nephrology; Recurrence; Referral and Consultation; Remission, Spontaneous; Urinalysis

Genetic polymorphisms adjacent to IL28B have been previously associated with spontaneous clearance of hepatitis C virus (HCV) and a higher rate of sustained virological response to interferon-based treatment in HCV genotype 1-infected patients. A recent study has shown that patients with the CC genotype of the rs12979860 single nucleotide polymorphism upstream from the IL28B gene are more likely to clear HCV spontaneously relative to the CT or TT genotype. In the liver transplant cohort, HCV recurs almost universally in patients with detectable HCV RNA at the time of transplantation. The spontaneous clearance of HCV infection after transplant is very rare. We report two cases of spontaneous clearance of HCV genotype 1 infection after liver transplantation from homozygous IL28B CC donors. This finding may be explained by alterations in the host immune responses to HCV after transplantation with a CC donor liver, which has potential implications for donor selection in HCV-positive recipients.

Topics: Adult; Alcoholism; Drug Therapy, Combination; Hepatitis C, Chronic; Humans; Immunosuppressive Agents; Interferons; Interleukins; Liver Transplantation; Living Donors; Male; Methylprednisolone; Mycophenolic Acid; Polymorphism, Genetic; Remission, Spontaneous; Tacrolimus; Treatment Outcome

Topics: Anti-Inflammatory Agents, Non-Steroidal; Cytomegalovirus Infections; Female; Humans; Middle Aged; Mycophenolic Acid; Remission, Spontaneous; Scleroderma, Diffuse

The objective of this paper was to study the incidence, risk factors, clinical outcome, management and prevention of pure red cell aplasia (PRCA) following major ABO-incompatible allogeneic hematopoietic stem cell transplantation (allo-HSCT). We retrospectively analyzed 11 cases of PRCA from a series of 42 patients undergoing major ABO-incompatible allo-HSCT from April 1997 to December 2005. Eleven out of the 42 patients developed PRCA (26.1%). All the 11 cases of PRCA were in blood group O recipients of grafts from blood group A donor (n = 9) or blood group B donor (n = 2). The following factors were associated with an increased risk of PRCA: (1) blood group O recipient; (2) blood group A donor; and (3) blood group O/A in recipient/donor pair. Only blood group O/A in recipient/donor pair was identified as being significantly associated with the occurrence of PRCA by multivariate analysis. Six patients who received donor-type plasma exchange did not develop PRCA and among them 5 cases were the blood group O recipients. Eight patients obtained spontaneous remission and in the remaining 3 patients 2 with long-lasting PRCA were successfully treated with plasma exchange with donor-type plasma replacement and the other one who was also complicated by EBV-associated lymphoproliferative disorder (EBV-PTLD) responded rapidly to anti-CD20 monoclonal antibody and achieved complete resolution of clinical finding and symptom of both EBV-PTLD and PRCA. We conclude that blood group A/O in donor/recipient pair is identified as being significantly associated with the occurrence of PRCA by multivariate analysis. Donor-type plasma exchange and anti-CD20 monoclonal antibody is an effective approach for the treatment of PRCA. PRCA could be prevented by plasma exchange prior to transplantation.

Topics: ABO Blood-Group System; Adult; Antibodies, Monoclonal; Antibodies, Monoclonal, Murine-Derived; Antigens, CD20; beta-Thalassemia; Blood Group Incompatibility; Busulfan; Child, Preschool; Cyclosporine; Epstein-Barr Virus Infections; Female; Graft Survival; Graft vs Host Disease; Hematopoietic Stem Cell Transplantation; Humans; Immunosuppressive Agents; Leukemia; Lymphoproliferative Disorders; Male; Methotrexate; Middle Aged; Mycophenolic Acid; Plasma Exchange; Red-Cell Aplasia, Pure; Remission, Spontaneous; Retrospective Studies; Risk Factors; Rituximab; Transplantation Conditioning; Transplantation, Homologous; Vidarabine; Whole-Body Irradiation

Topics: Cyclosporine; Female; Humans; Immunosuppressive Agents; Kidney Transplantation; Middle Aged; Mycophenolic Acid; Postoperative Complications; Remission, Spontaneous; Retreatment; Sarcoma, Kaposi; Skin Neoplasms