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Introduction Large granular lymphocytes ( LGLs) are medium- to large-sized lymphocytes with azurophilic cytoplasmic granules. Reactive vs. neoplastic LGLs are usually morphologically indistinguishable. Methods We investigated 25 consecutive cases of LGL lymphoproliferation using flow cytometric T cell receptor Vβ ( FC-Vβ) repertoire and T cell receptor gene rearrangement ( TCR- GR) in detecting clonality. Results Seventeen patients (68%) were T- LGL leukemia (T- LGLL) with a male predominance, a median age of 67, and a median absolute LGL count of 2.592 × 109/L. All cases were clonal using the FC-Vβ analysis, and all but one (94%) was clonal by TCR- GR. Eight patients (32%) had reactive LGL lymphoproliferation. Two had EBV-associated infectious mononucleosis; one was clonal by both FC-Vβ and TCR- GR; and the other was clonal only by TCR- GR. The remaining six cases were polyclonal by both assays. Patients with reactive LGL lymphoproliferation were more frequently associated with an underlying/concurrent malignancy than those with T- LGLL (4/8 cases vs. 1/17; P = 0.023, Fisher's exact test). We compared the demographic, hemogram, and clonality data between these two groups and found that the only significant difference was the lower median platelet count in the LGL lymphocytosis group (201 × 109/L vs. 223 × 109/L; P = 0.031; Student's t-test). A literature review including the current study showed a high sensitivity of FC-Vβ analysis for T- LGLL (97.2%; 107/110 cases). Conclusions FC-Vβ analysis was slightly more sensitive than TCR- GR for the detection of clonal T cell lymphoproliferation. However, we must interpret the laboratory findings with clinical context as clonal T cell lymphoproliferation may occur in patients with viral infection. [ABSTRACT FROM AUTHOR]

Aims:  To elucidate the clinicopathological features and prognostic factors of primary intestinal diffuse large B-cell lymphoma (PI-DLBL). Methods and results:  Archival tissues from 30 tumours were used for tissue microarray construction, immunohistochemistry and interphase fluorescence in situ hybridization for chromosomal translocation. The M:F ratio was 1.7:1, with a median age of 60 years. The ileum and ileocaecum were most frequently involved (40% each). Fourteen (47%) were at stage IE disease, 15 (50%) at stage IIE. Five (17%) tumours were perforated at presentation. The tumours expressed Bcl-6 (73%), MUM1 (70%), Bcl-2 (67%) and CD10 (23%). Nine (30%) were classified as germinal centre B-cell (GCB) phenotype and 21 non-GCB. Eight of 30 (27%), 7/30 (23%) and 2/29 (7%) cases were positive for rearrangements involving IGH, BCL6, and C-MYC loci, respectively, whereas all cases were negative for BCL2 and CCND1 translocation. Perforation was a poor prognostic indicator, with a hazard ratio of tumour-related death at 8.75 ( P = 0.001). The differentiation antigens, GCB versus non-GCB phenotype, or lymphoma-associated translocations were of no prognostic significance. Conclusions:  We found a higher rate of perforation and lower frequency of GCB phenotype in PI-DLBL in Taiwan compared with other geographical areas; perforation is a poor prognostic indicator. [ABSTRACT FROM AUTHOR]

Aims: To characterize the clinicopathological features of sporadic Burkitt lymphoma (BL). Methods and results: A retrospective study of 17 paediatric and 14 adult BLs with history and histopathology review, immunohistochemistry, Epstein–Barr virus (EBV) in situ hybridization (EBER) and fluorescence in situ hybridization. There was no statistically significant difference in gender, frequency of central nervous system (CNS) involvement and leukaemic change at presentation, or frequency of CD10+/Bcl-2−/Bcl-6+ (88% versus 86%), Ki67 labelling index, EBER (24% versus 21%), or C-MYC translocation (100% versus 92%) between paediatric and adult tumours. Correct pretreatment diagnoses were made in 13/17 (76%) paediatric and in 9/14 (64%) adult tumours. Twenty-eight patients received chemotherapy including 13/16 (81%) paediatric and 3/12 (25%) adult patients with appropriate regimens; 16 (57%) received CNS prophylaxis. The 1- and 5-year overall survival (OS) rates for paediatric patients were 80% and 50%, respectively, whereas 1-year OS for adults was 15%. Conclusions: Sporadic paediatric and adult BLs were phenotypically and genotypically similar. The significant prognosticators were age ( P = 0.001), with or without CNS prophylaxis ( P = 0.004), and CNS involvement ( P = 0.008) and leukaemic change ( P = 0.019) in disease course. The poor outcome in adult patients might be related to incorrect diagnosis and inappropriate treatment. [ABSTRACT FROM AUTHOR]