Hisashi Tsutsumi

Abnormal N-glycosylation of the immunoglobulin G κ chain in a multiple myeloma patient with crystalglobulinemia : Case report

Spontaneous crystallization of monoclonal immunoglobulins (crystalglobulin) is a rare complication of multiple myeloma. We describe a 64-year-old Japanese man with skin ulcers and renal failure associated with immunoglobulin G κ multiple myeloma. Crystallized immunoglobulin was detected in his serum at room temperature. Analysis of the patient’s crystalglobulin by sodium dodecyl sulfate-polyacrylamide gel electrophoresis and mass spectrometry suggested that the crystallization was due to abnormal glycosylation of the immunoglobulin light chain. Treatment with thalidomide and dexamethasone improved the severe skin ulcers on the patient’s extremities and partially reversed his renal failure. This report is the first of abnormal glycosylation of immunoglobulin possibly caused by modification ofN-glycans in the light chain. We concluded that abnormal glycosylation of the immunoglobulin light chain might be the cause of the patient’s skin ulcers and renal dysfunction. cr 2007 The Japanese Society of Hematology

Prognosis of elderly patients with acute myelogenous leukemia: analysis of 126 AML cases.

We retrospectively analyzed 126 acute myelogenous leukemia (AML) patients aged ≥60 years who had all been referred to the same hematological department between 1989 and 1999. In 76 de novo AML cases, 53 patients (median age, 72 years) were treated with combination chemotherapy (CT) for remission induction. Complete remission (CR) rate was 57.1%. The median overall survival (OS) was 16 months, and the rate of 3-year OS was 28%. The favorable prognostic factors were performance status ≥2, cholinesterase ≥100 IU, and intermediate or favorable karyotype (P < .01). Seventeen patients (median age, 78 years) with hypocellular bone marrow or poor general condition were treated with low-dose cytosine arabinoside (LDAraC). In these patients, the CR rate was 50% and the median OS was 11 months, with an OS estimate at 3 years of 14%. All patients with hypocellular bone marrow who received LDAraC for 21 days achieved CR. In 50 patients who developed AML following a myelodysplastic syndrome (MDS/AML), 22 patients (median age, 74 years) were treated with CT, and 14 (median age, 74 years) patients were treated with LDAraC. The CR rates were 22.7% and 21.4%, respectively, and the median OS durations were 8 months and 11 months, respectively. There were no significant factors that would indicate a good prognosis in MDS/AML patients.

A new glucose-6-phosphate dehydrogenase variant (G6PD Tsukui) associated with congenital hemolytic anemia

SummaryA new glucose-6-phosphate dehydrogenase (G6PD) variant associated with chronic nonspherocytic hemolytic anemia was found in a 20-year-old Japanese male who showed mild hemolysis after an upper respiratory tract infection. The patient had been noted to have jaundice and reticulocytosis several times before this episode. The enzyme activity of the variant was 1.5% of normal. The enzymatic characteristics were slow anodal electrophoretic mobility, high Km G6P, normal Km NADP, decreased heat stability, and a normal pH optimum. From these results, the enzyme was considered to be a new class 1 variant and was designated G6PD Tsukui.

Bone lesions in elderly patients with multiple myeloma: a multicenter study by the Society for Geriatric Hematology

Background:  Bone lesions in multiple myeloma (MM) have a significant impact on the quality of life of elderly patients, but they have not been extensively investigated in the elderly.

Etoposide が著効を示した Heavy chain disease の1例

A 79-year-old man with marked hepatosplenomegaly and lymphadenopathy was admitted to the hospital. Analysis of serum protein resulted in the diagnosis of gamma-chain disease (total protein 6.2 g/dl, M-protein positive, IgG 4150 mg/dl, IgG-Fc fragment positive). Specimens obtained by lymph node biopsies showed infiltration of plasmacytoid cells, which were stained with anti-IgG but not with anti-kappa or anti-lambda antibodies. The patient was given combination chemotherapy, but without effect. Then a regimen of long-term administration of low-dose etoposide was begun, and resulted in remission of the lymphadenopathy, hepatosplenomegaly, and abnormal IgG.