Antigenically defined subgroups of lymphoblastic lymphoma. Relationship to clinical presentation and biologic behavior

Sampaio

et al. 2015

Rev. Assoc. Med. Bras.

Section: Discussionsupporting

confidence: 54%

Outcome of children and adolescents with lymphoblastic lymphoma

Sampaio

et al. 2015

Rev. Assoc. Med. Bras.

“…predominant in T-LBL. 25 In the present study, mediastinal enlargement was present in more than half of the cases. Two patients had BM invasion, but CNS disease was not observed.…”

Section: 1521supporting

confidence: 52%

Outcome of children and adolescents with lymphoblastic lymphoma

Sampaio

et al. 2016

Rev. Assoc. Med. Bras.

SUMMARY Introduction: lymphoblastic lymphoma (LBL) is the second most common subtype of non-Hodgkin lymphoma in children. The aim of this study was to characterize the clinical course of children and adolescents with LBL treated at a tertiary center. Methods: this is a retrospective cohort study of 27 patients aged 16 years or less with LBL admitted between January 1981 and December 2013. Patients received intensive chemotherapy regimen derived from acute lymphoblastic leukemia (ALL) therapy. Diagnosis was based on biopsy of tumor and/or cytological examination of pleural effusions. The overall survival was analyzed using the Kaplan-Meier method. Results: the median age at diagnosis was 11.6 years (interquartile range, 4.6-13.8). LBL had T cell origin in 16 patients (59%). The most common primary manifestation in T-cell LBL was mediastinum involvement in 9 patients (56%). Intra-abdominal tumor was the major site of involvement in patients with pB-LBL. Most patients had advanced disease (18 patients - 67%) at diagnosis. Twenty-four patients (89%) achieved complete clinical remission. After a median follow-up of 43 months (interquartile range, 6.4-95), 22 patients (81%) were alive in first complete remission. Five children (18.5%) died, three of them soon after admission and two after relapsing. The probability of survival at five years for 20 patients with de novo LBL was 78% (SD 9.4). Conclusion: our findings confirm the favorable prognosis of children with LBL with an intensive chemotherapy regimen derived from ALL therapy.

“…Both of our cases ran a rapid downhill course and died in a short period of time. In other reports, the outcome of the patients was mentioned in ten cases, and seven patients died [8, 10,13,15]. Most patients died within 1 year, and the mean survival time was 8.5 months in the six patients reported by Sheibani , three were Asian.…”

Section: Discussionmentioning

confidence: 91%

A study of lymphoma of large granular lymphocytes with modern modalities: Report of two cases and review of the literature

et al. 1992

Two cases of lymphoma of large granular lymphocytes are reported. The first case expressed natural killer (NK) cell, some T-cell (CD 2, CD 5, CD 8), and HLA-DR antigens, but was negative for other T-cell (CD 3, CD 4, CD 7), T-cell receptor (TCR), B-cell, and myeloid antigens. Germline configuration was demonstrated for TCR, and immunoglobulin heavy and light chain genes. The second case expressed NK cell, some T-cell (CD 3, CD 7, CD 8), and TCR antigens, but was negative for other T-cell (CD 4, CD 5), B-cell, myeloid, and HLA-DR antigens. Rearrangement of TCR alpha and beta chains were detected. Thus, the findings of case 1 were consistent with true NK cell lineage and case 2 with NK-like T-cell lineage. Our report underscores the heterogeneity of this newly recognized lymphoma, which nevertheless carries a consistently poor prognosis and is probably more prevalent in the Asian population. This study also provides information concerning immunophenotypes of cellular infiltrates in internal organs and cytogenetic abnormalities in this lymphoma; neither has been reported frequently in the literature. The importance of detecting cytoplasmic granules in tissue imprints or electron micrographs for differentiating other T-cell lymphomas is emphasized, and the classification of large granular lymphoproliferative disorders is discussed.