1/6. Ovarian non-Hodgkin's lymphoma: a clinicopathologic study of eight primary cases.Primary (localized) non-Hodgkin's lymphoma (NHL) of the ovary is rare. We studied eight cases of primary ovarian NHL to better understand the clinicopathologic and immunophenotypic features of these tumors. The patients ranged in age from 29 to 62 years (mean 47 years). Pelvic complaints were the most common symptoms; however, three of eight neoplasms were discovered incidentally. All tumors were unilateral and Ann Arbor stage I(E). The three incidental NHL were microscopic (largest 1.2 cm), whereas the grossly evident lesions ranged from 7.5 to 20 cm (mean 13.3). Each tumor was classified according to the world health organization classification as follows: diffuse large B-cell lymphoma (three cases), follicular lymphoma (two cases), burkitt lymphoma (one case), T-cell anaplastic large cell lymphoma (one case), and precursor T-lymphoblastic lymphoma (one case). Six tumors were of B-cell lineage, and two tumors were of T-cell lineage. All three diffuse large B-cell lymphomas were positive for BCL-6, two were positive for CD10, and two were positive for BCL-2. Estrogen and progesterone receptors were negative in all NHLs assessed. patients were treated by various combinations of surgery, chemotherapy, and radiotherapy. Clinical follow-up ranged from 1.3 to 11.7 years (mean 5.2) and all patients were alive without disease at last follow-up. We conclude that most patients with primary ovarian NHL present with symptoms attributable to an ovarian mass, but in a subset of patients ovarian NHL may be detected incidentally. With appropriate therapy, patients appear to have a favorable prognosis although follow-up is short for some patients in this study.- - - - - - - - - - ranking = 1keywords = precursor (Clic here for more details about this article) |
2/6. Is B-lineage acute lymphoblastic leukemia with a mature phenotype and l1 morphology a precursor B-lymphoblastic leukemia/lymphoma or Burkitt leukemia/lymphoma?CONTEXT: B-lineage acute lymphoblastic leukemia (ALL) with a mature phenotype and L1 morphology is a rare condition that may pose a diagnostic and management challenge. OBJECTIVE: To report our experience with 2 such unusual cases of pediatric B-lineage ALL. DESIGN: Morphologic, immunophenotypic, and cytogenetic features of the leukemic blast cells were reviewed in conjunction with clinical and other laboratory findings. RESULTS: The leukemic blast cells in both cases were small to medium with scant basophilic cytoplasm and several small inconspicuous nucleoli, characteristic of L1 lymphoblasts. Immunophenotypically, they were positive for CD19, CD22, and low-density CD20, with expression of surface immunoglobulin lambda light chain. They were negative for immature (CD34 and terminal deoxynucleotidyl transferase), myeloid, and T-cell-associated markers. Conventional cytogenetic and fluorescent in situ hybridization studies failed to demonstrate chromosomal translocations involving the c-myc gene. Both patients were treated with Children's Cancer Group ALL protocols and had good responses. CONCLUSIONS: B-lineage ALL with a mature phenotype, L1 morphology, and absent chromosomal translocations involving the c-myc gene is best classified and managed as precursor B-lymphoblastic leukemia/lymphoma instead of Burkitt leukemia/lymphoma.- - - - - - - - - - ranking = 5keywords = precursor (Clic here for more details about this article) |
3/6. A subtle t(3;8) results in plausible juxtaposition of MYC and BCL6 in a child with burkitt lymphoma/leukemia and ataxia-telangiectasia.Translocations involving 3q27 that affect the BCL6 gene are common and specific chromosomal abnormalities in B-cell precursor non-Hodgkin lymphoma (mainly diffuse large-cell and follicular lymphoma), but they have not been reported in burkitt lymphoma. Here, we describe a case in which a BCL6 rearrangement and additional complex cytogenetic abnormalities occurred in a child with burkitt lymphoma/leukemia and ataxia-telangiectasia. Although cytogenetic analysis of the bone marrow revealed clonal abnormalities of chromosome arms 8q and 14p and other subclonal abnormalities, the t(8;14) or its variants typically associated with burkitt lymphoma were not observed. fluorescence in situ hybridization with locus-specific probes and multicolor spectral karyotyping demonstrated a complex pattern of chromosomal rearrangements leading to a subtle t(3;8)(q27;q24.1) that rearranged BCL6 and placed it adjacent to MYC. We speculate that this genetic lesion occurred as a result of chromosomal instability due to the underlying disease.- - - - - - - - - - ranking = 1keywords = precursor (Clic here for more details about this article) |
4/6. Surface immunoglobulin positive lymphoblastic leukemia in adults; a genetic spectrum.Precursor B-lymphoblastic leukemia is typically surface immunoglobulin (sIg) negative. Although rare cases of sIg precursor lymphoblastic leukemia are recognized, sIg leukemia often represent leukemic phase of burkitt lymphoma or other non-Hodgkin lymphoma such as blastic mantle cell lymphoma. This study reports four adults, two women (56 and 58 years old) and two men (35 and 41 years old) with lymphoblastic leukemia that displayed lambda, surface immunoglobulin restriction (sIg ). The leukemic cells were all dim CD45 positive with side scatter light characteristic of blasts. Two cases were positive with the blasts associated with antigens TdT and CD34. Genetic abnormalities were detected in all cases and in three cases included abnormalities commonly present in precursor lymphoblastic leukemia. Translocation (1;19) (q23;p13) was present in the first case. Deletion of the 3' region of the mixed lineage leukemia (MLL) gene at chromosome 11q23 as well as t(14;18) were detected in the second case. In the 3rd case, a BCR-ABL fusion gene was detected as part of a complex abnormal karyotype. Translocation (1;19)(q23;p13) was present in one case. Deletion of the 3' region of the mixed lineage leukemia (MLL) gene at chromosome 11q23 as well as t(14;18) were detected in one case. BCR-ABL fusion gene was detected as part of a complex abnormal karyotype in one case. These cases illustrate that lymphoblastic leukemias occurring in adults exhibit a morphologic, immunophenotypic as well as a genetic spectrum and represent either non-Hodgkin lymphoma or precursor lymphoblastic leukemia. A multi-parameter approach including flow cytometric and genetic studies is crucial in separating these cases.- - - - - - - - - - ranking = 3keywords = precursor (Clic here for more details about this article) |
5/6. Burkitt cell acute lymphoblastic leukemia with partial expression of T-cell markers and subclonal chromosome abnormalities in a man with acquired immunodeficiency syndrome.A 45-year-old white male, bisexual, with a 2-year history of acquired immunodeficiency syndrome (AIDS) prodrome, developed a Burkitt cell-like acute lymphoblastic leukemia (ALL). Marker studies of marrow blasts show an unusual and possibly unique pattern, in that an unequivocal monoclonal B cell leukemia, having K-IgM with HLA-DR and B cell subset antigen (BA-1) expression, was superimposed with a mature suppressor T cell marker profile (pan-T, mature T, and suppressor/cytotoxic T antigens). The leukemic blasts were totally negative for terminal deoxynucleotidyl transferase (TdT), human T cell leukemia-lymphoma virus (HTLV) p19 antigen, and other immunoglobulin isotypes. Chromosome analysis of marrow cells disclosed that 70% of the cells had 47,XY, 12,t(8;14)(q24;q32) chromosome complement, and 30% of the cells had a 47,XY, 12,dup1q (q22-31),t(8;14)(q24;q32). The consistent finding of the specific chromosome rearrangement (8/14 translocation) in all abnormal cells suggests that the cells were derived from a common precursor. With regard to the partial T cell marker expression, the significance of these markers in B cell leukemia is unclear, as is their relation to the additional chromosome abnormalities that apparently developed in the process of clonal evolution.- - - - - - - - - - ranking = 1keywords = precursor (Clic here for more details about this article) |
6/6. Two cases of de novo precursor B-cell acute lymphoblastic leukemia with t(14;18), but without cytogenetic evidence of an associated Burkitt's or Burkitt's variant translocation.This study reports two cases of acute lymphoblastic leukemia of precursor B-cell type. The cases were associated with a t(14;18), but no evidence of a Burkitt's-type translocation was found. There was also no evidence of a preexisting follicular lymphoma. This cytogenetic variety of acute lymphoblastic leukemia has not been reported previously.- - - - - - - - - - ranking = 5keywords = precursor (Clic here for more details about this article) |