ABSTRACT
A 61-year-old woman experienced a high fever with anemia and APTT prolongation after suffering a herpes zoster virus infection. Physical examination revealed a large splenomegaly without lymphadenopathy. Laboratory evaluations were positive for lupus anticoagulant (LA) and monoclonal IgM-kappa protein. LA was associated with the presence of anti-beta2GPI antibody, anti-cardiolipin antibody, and anti-prothrombin antibody. Moreover, the results of factors IX, XI, and XII assays and CRP and FDP-E were disturbed. A splenectomy was performed, and a splenic marginal zone lymphoma (SMZL) was diagnosed. All hematological findings rapidly recovered after the splenectomy. No thrombotic events occurred after the splenectomy even though thrombosis prophylaxis was not performed. The clinical course suggested that the SMZL-producing antibody induced immunological abnormalities in the labolatory tests. Since the patient suffered disease progression soon after the splenectomy, an autologous peripheral stem cell transplantation with rituximab administration was performed.
Subject(s)
Antibodies, Antiphospholipid/blood , Lymphoma, B-Cell/immunology , Splenic Neoplasms/immunology , Antibodies, Anticardiolipin/blood , Antibodies, Monoclonal/administration & dosage , Antibodies, Monoclonal, Murine-Derived , Antineoplastic Agents/administration & dosage , Disease Progression , Female , Humans , Lupus Coagulation Inhibitor/blood , Lymphoma, B-Cell/diagnosis , Lymphoma, B-Cell/therapy , Middle Aged , Peripheral Blood Stem Cell Transplantation , Remission Induction , Rituximab , Splenectomy , Splenic Neoplasms/diagnosis , Splenic Neoplasms/therapy , Transplantation, Autologous , Treatment OutcomeABSTRACT
Nephrotic syndrome after hematopoietic stem cell transplantation (HSCT) followed by donor lymphocyte infusion (DLI) has never been described. We report the case of a myelodysplastic syndrome (MDS) patient who developed nephrotic syndrome with membranous nephropathy 18 months after allogeneic HSCT and 4 months after DLI. A 50-year-old woman with MDS underwent allogeneic bone marrow transplantation from her HLA-matched brother. MDS relapsed 55 days after transplantation, donor lymphocytes were infused as adoptive immunotherapy, and complete remission was achieved. Four months after the third DLI, the patient developed nephrotic syndrome with proteinuria up to 9 g/day. Renal biopsy revealed granular deposits of immunoglobulin G along the glomerular basement membrane, and subepithelial electron-dense deposits. A diagnosis of membranous nephropathy was made. For maintenance of the immunotherapeutic effect of DLI, minimum doses of immunosuppressive therapy for decreasing proteinuria were administered, and improvement of nephrotic syndrome and persistent complete remission of MDS were achieved.
Subject(s)
Bone Marrow Transplantation/adverse effects , Glomerulonephritis, Membranous/etiology , Lymphocyte Transfusion/adverse effects , Female , Glomerulonephritis, Membranous/drug therapy , Humans , Middle Aged , Myelodysplastic Syndromes/complications , Myelodysplastic Syndromes/therapy , Nephrotic Syndrome/drug therapy , Nephrotic Syndrome/etiology , Proteinuria/drug therapy , Proteinuria/etiology , Remission Induction , Transplantation, HomologousABSTRACT
An adult patient suffering from G-CSF-resistant very severe aplastic anemia received a cord blood transplantation from a three-loci HLA-mismatched unrelated donor after nonmyeloablative conditioning. Cord blood was infused after conditioning with fludarabine (180 mg/m2) and cyclophosphamide (100 mg/kg). Cyclosporin A and short-term methotrexate were used for prophylaxis against acute GVHD. Engraftment was achieved on day 23, and there was no serious GVHD. A full-donor type T-cell chimerism was obtained by day 30. Normal hematopoiesis and complete chimerism have been maintained 14 months after transplantation.
