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Purpose To investigate the clinicopathological features,molecular genetics and prognosis of high grade B cell lymphoma with concurrent MYC rearrangement and 11q aberra-tions(HGBCL-MYC-11q).MethodsThree cases of HGBCL-MYC-11q were reviewed and analyzed using hematoxylin-eosin staining,immunohistochemistry,EBER in situ hybridization and fluorescence in situ hybridization.Clinical data were collected with follow-up.Results All three patients were male,age was 10,61,and 74 years,respectively.All patients had Ann Arbor stage Ⅳ disease.All three cases were biopsies occurring in the nasopharynx,upper pharynx and ileocecus,respectively.Three cases were morphologically similar to diffuse infiltrative growth of tumor cells,moderate or moderately large cells,round to slightly irregular nuclei and easily visible mitotic figures.Focal necrosis was noted in one case.One case exhibited the distinct"starry sky"pattern.All cases expressed CD20,BCL6 and MUM1 and high Ki67 index,two cases expressed CD10 and two cases ex-pressed BCL2.CD3,CD30 and TDT were all negative.EBER in situ hybridization was all negative.FISH analyses using C-MYC break-apart probes were all positive and all cases had 11q aberrations.One case only had the 11q23.3 amplification;and one case only had the 11q24.3 loss.After a follow-up for 1-18 months,one patient died and two patients survived with disease.ConclusionHGBCL-MYC-11q is rare,morphologically similar to BL/HGBCL,with MYC rearrangement and 11q abnormali-ties.We should enhance awareness of the disease and improve more accurate diagnosis and differential diagnosis of the disease.
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@#High-Grade B-Cell Lymphoma (HGBCL) with gene rearrangements in MYC and BCL2 and/or BCL6 is an aggressive malignancy usually presenting in advanced stages. Current recommendations suggest the use of regimens more intensive than R-CHOP (rituximab, cyclophosphamide, vincristine, doxorubicin, prednisone), which are based on retrospective studies and single-arm prospective trials that included patients who are mostly in the advanced stage, and did not receive consolidation radiotherapy. The optimal approach and treatment of HGBCL, whether limited-stage (LS) or advanced-stage, remains to be determined. Here we describe the promising outcomes of three patients with LS and low IPI HGBCL with the use of R-CHOP as induction chemotherapy regimen, which was followed by consolidation radiotherapy. Three women, 54-, 60-, and 64-years of age diagnosed to have HGBCL with MYC, and BCL2 and/or BCL6 rearrangements, with Ann Arbor stages I-IIE were included in this case series. All three patients had complete metabolic response to 6 cycles of R-CHOP and was subsequently treated with consolidation involved site radiotherapy (ISRT; total dose 30-36 Gy). Chemotherapy and radiotherapy were tolerated very well. All patients remain to be in remission, with the longest being at 23 months. Outcomes of patients with HGBCL generally remain to be poor, but this may not be the case for patients with limited-stage disease and favorable clinicopathologic risk profile. Nevertheless, the treatment of HGBCL is currently evolving and more studies are needed to determine the ideal approach and preferred chemotherapy regimen. Also, more studies are needed to elucidate the potential role of consolidation radiotherapy in patients with limited-stage HGBCL to improve survival outcomes. Findings of this case series suggest that patients with LS HGBCL may still derive benefit from R-CHOP followed by consolidation ISRT, but prospective trials are needed to confirm this.
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Objective To analyze the cases that are easily misdiagnosed as diffuse large B-cell lymphoma (DLBCL),and to explore the diagnosis,differential diagnosis and clinicopathological features of DLBCL.Methods The clinicopathological data of 431 cases preliminarily diagnosed as DLBCL in the Department of Pathology,School of Basic Medical Science,Peking University from October 2016 to December 2017 were retrospectively analyzed.Further immunohistochemical staining and molecular biological tests were performed if necessary.Results The diagnostic accuracy rate of DLBCL was 88.86％ (383/431).Misdiagnosis mainly occurred in follicular lymphoma (36 cases,8.35％),high-grade B-cell lymphoma (HGBL) with gene rearrangement of c-myc and bcl-2 or bcl-6 (4 cases,0.93％),atypical lymphoid tissue hyperplasia (3 cases,0.70％),mantle cell lymphoma (3 cases,0.70％),classic Hodgkin lymphoma (CHL) (1 case,0.23％) and composite lymphoma (DLBCL and CHL) (1 case,0.23％).Except the poor quality of sections,the lack of immunohistochemical markers,the poor knowledge of the diagnosis of DLBCL and high-grade FL as well as HGBL were the main reasons of misdiagnosis.Conclusions The diagnosis and differential diagnosis of DLBCL is based on morphology,and it needs to combine with the corresponding immunohistological markers and molecular biological detection when necessary.The recognition of clinical and pathological features of various types of misdiagnosed lymphomas should be strengthened.
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Paciente de 35 años de edad, quien debuta con síndrome de pulmón riñón secundario a vasculitispulmonar y depósitos renales granulosos de IgA, en quien se diagnostica vasculitis sistémica porIgA, posteriormente, presenta compromiso pancreático y cutáneo (Púrpura de Henoch Schõnlein),falleciendo ocho meses más tarde por linfoma difuso de células B de alto grado. Este es elreporte de caso número catorce en el que se establece asociación entre neoplasia hematológicay Púrpura de Henoch Schõnlein