The incidence of follicular lymphoma differs significantly between white and Japanese individuals. Translocation between the BCL-2 and immunoglobulin heavy chain genes is detected in 85% to 90% of all follicular lymphomas in whites. Recently, BCL-2/J H translocation was detected in peripheral blood lymphocytes from more than 50% of healthy white individuals. To clarify the reason for the difference in incidence of follicular lymphoma between whites and Japanese, the frequency of BCL-2/J H translocation in peripheral blood lymphocytes of healthy Japanese individuals was compared with that of German individuals. The prevalence of BCL-2/J H translocation in Japanese adults appeared to be significantly lower than that in German adults. The present data suggest that the low frequency of BCL-2/J H translocation in the Japanese general population may be one of the major reasons for the difference in incidence of follicular lymphoma between whites and Japanese. IntroductionThe incidence of follicular lymphoma differs between white and Japanese individuals, accounting for about 40% of all non-Hodgkin lymphomas in whites, compared with less than 10% in Japanese. [1][2][3][4][5] The age-adjusted incidence of follicular lymphoma (per 100 000) is reported to be 3.8 in the United States and 0.5 in Japan. 2 The reason for this difference between the 2 populations is unknown.The translocation t(14;18)(q32;q21) between the BCL-2 protooncogene and the J H immunoglobulin gene region is detected in 85% to 90% and approximately 50% of all follicular lymphomas in whites and Japanese, respectively. [5][6][7][8][9][10] This leads to overexpression of BCL-2, conferring a growth advantage on the neoplastic cells. 11 Recently, it has been reported that BCL-2/J H translocation is frequently detected in peripheral blood lymphocytes of healthy white individuals. [12][13][14][15][16][17] In the multihit theory of tumorigenesis, translocation of the BCL-2 gene is considered to be the first somatic mutation, and additional mutations are needed for development of follicular lymphoma. From this viewpoint, the low incidence of follicular lymphoma in Japanese individuals may be due to the low frequency of BCL-2/J H translocation in the general population or other factors affecting lymphomagenesis. Accordingly, we compared the frequency of BCL-2/J H translocation in peripheral blood lymphocytes of Japanese individuals with that of German individuals. The results showed that the incidence of BCL-2/J H translocation in healthy Japanese individuals is significantly lower than that in German individuals. Study designPeripheral blood mononuclear cells were obtained with informed consent from Japanese and German (white) individuals who had no serious diseases, and the DNA was extracted using standard procedures. The DNA samples from German individuals were sent to Japan and used for experiments. The presence of the BCL-2/J H translocation in the major breakpoint region (MBR) of the BCL-2 gene was examined using a nested polymerase chain reaction (PCR), a...

Summary:A 61-year-old man with angioimmunoblastic lymphoma in first complete remission underwent autologous peripheral blood stem cell transplantation. At 1 month post transplant, asymptomatic large granular lymphocytosis developed. The surface marker profile of the cells was CD3 þ CD8 þ CD56ÀCD57 þ . The disease course was chronic and indolent. The patient remains in complete remission from angioimmunoblastic lymphoma more than 6 months post transplant with persistent large granular lymphocytosis (lymphocyte count, 5-15 Â 10 9 /l). Although post transplantation T-cell lymphoproliferative disorders have mostly occurred in allogeneic transplantation recipients and presented as aggressive lymphomas/leukemias, we suggest that chronic indolent T-cell large granular lymphocytic leukemia can occur after autologous stem cell transplantation. Bone Marrow Transplantation (2004) 33, 99-101. doi:10.1038/sj.bmt.1704298 Keywords: T-cell large granular lymphocytic leukemia; post transplantation lymphoproliferative disorders; autologous peripheral blood stem cell transplantation T-cell large granular lymphocytic (T-LGL) leukemia is a lymphoproliferative disease derived from post-thymic immunocompetent T lymphocytes. 1 Large granular cell morphology, CD3 þ CD56ÀCD57 þ immunophenotype and the clonal rearrangement of T-cell receptor genes characterize T-LGL leukemia, which presents clinically with a chronic indolent disease course, complicated by frequent infections secondary to neutropenia.Post transplantation lymphoproliferative disorders (PTLD) are a well-recognized complication of solid organ and allogeneic bone marrow transplantation, and accumulating data have suggested that aggressive immunosuppression is closely associated with an increased risk of PTLD. 2,3 The majority of PTLD are of B-cell origin and are associated with active infection with Epstein-Barr virus (EBV). T-cell PTLD are much less common but, similar to B-cell PTLD, they mostly present as aggressive lymphomas following a rapidly fatal course. 4,5 EBV is infrequently involved in the pathogenesis. Autologous stem cell transplantation (ASCT) is another transplantation procedure. In spite of the profound myelosuppression associated with autografting, this procedure is inherently less immunosuppressive than allogeneic bone marrow transplantation and we were able to find less than 20 cases in the English literature describing PTLD following ASCT. 6-8 Here, we report the first case of chronic T-LGL leukemia following ASCT for angioimmunoblastic lymphoma. Case reportA 61-year-old man was admitted to our hospital in April 1997 because of fever, skin rash, edema, generalized lymphadenopathy and hemolysis. On physical examination, there was a maculo-papular rash on the trunk and extremities, and superficial lymphadenopathy was found in various regions including the neck, axilla and groin. A left pleural effusion and moderate splenomegaly (20 mm on the left midclavicular line) were also noted. The hemoglobin (Hb) was 8.1 g/dl, white blood cell count (WBC) 9.1 Â 10 9 /l...

Several national and local external quality assurance schemes have been developed to improve the plasma fibrinogen assay in Japan over the past 30 years. Now most commercial calibrant plasma may be calibrated against an International Standard preparation, in order to achieve agreement of results obtained by different laboratories. However, we have never achieved satisfactory results, according to an external quality control survey regarding the fibrinogen assay. Therefore, we distributed two kinds of fibrinogen standards to be used as common calibrators, along with three plasma samples, among 183 general laboratories in Japan. The results of this collaborative study showed that the assigned value for the commercially available calibrators remained problematic. Furthermore, it was concluded that the between-laboratory variability could not be improved beyond a certain degree of standardization, even if a common calibrator was used for the Clauss-derived assay carried out by an automatic coagulometer.

Summary. Coagulation factor XIII consists of two A-and two B-subunits, and either gene mutation can cause a complete de®ciency. In a newborn patient with persistent bleeding from the umbilical cord stump, the plasma A-subunit protein was not detectable. Direct PCR sequencing revealed an nt 389 (ins G) frameshift mutation in exon 4 resulting in a new stop codon and a Ser 413 Leu missense mutation in exon 10 in either allele. His mother and father were heterozygous for the nt 389 (ins G) and the Ser 413 Leu, respectively, with about 50% reduction of the plasma A-subunit proteins. In all family members examined only those with either mutation showed the reduced subunit A protein levels. Thus, this complete de®ciency of factor XIII was due to a novel compound heterozygous mutation in the A-subunit gene.