Clinical characteristics of splenic marginal zone lymphoma with abnormal complete blood count / 中国实验血液学杂志

The aim of this study was to investigate the clinical and laboratorial characteristics of splenic marginal zone lymphoma (SMZL) with an abnormal complete blood count (CBC). Data of 19 newly diagnosed SMZL patients with abnormal CBC were analyzed retrospectively. Seven patients were diagnosed by using splenic histology, 12 patients who did not undergo splenectomy were diagnosed on the basis of typical clinical presentation and cytologic, immunophenotypic and histologic characteristics of peripheral blood and bone marrow, according to SBLG guidelines. The results showed that leukocytosis (≥ 10.0×10(9)/L) was seen in 5 cases (26.3%); leukocytopenia (< 4.0×10(9)/L) was found in 6 cases (31.6%), hemoglobin concentration less than 120 g/L was found in 14 cases (73.7%) and thrombocytopenia was found in 11 (57.9%) patients. Fourteen (73.7%) patients had cytopenia in one or more lineage. As a specific morphologic character, villous lymphocytes were found in 10 (52.6%) patients. Similar immunophenotype was determined by histology in both bone marrow and spleen. Various histological infiltration patterns including intrasinusoidal pattern were found in bone marrow. Nine out of 16 (56.3%) patients displayed an increase of serum monoclonal immunoglobin. Autoimmune phenomena was found in 12 out of 15 (80.0%) patients. Splenectomy, as the only treatment could not achieve a ≥ 50% improvement of CBC in 4 patients, and then was judged as no response. Splenectomy followed by chemotherapy achieved partial response (PR) in 1 patient. Overall response rate of the therapeutic strategies with Rituximab was 100.0% (11/11). Furthermore, complete response was achieved in 9 out of 11 (81.8%) patients. It is concluded that SMZL with abnormal CBC has a higher incidence of cytopenia, bone marrow involvement and autoimmune phenomena. Therapeutic strategies consisting of Rituximab show a better efficacy.

Expression profiling of microRNA and their target genes in peripheral T cell lymphoma, not otherwise specified / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the expression profile of microRNAs (miRNAs) in peripheral T cell lymphoma, not otherwise specified (PTCL-NOS) and to explore the underlying molecular characteristics.</p><p><b>METHODS</b>Twenty-one cases of PTCL-NOS were enrolled into the study. The tumor presented either as nodal (15/21) or extranodal (6/21) disease. TaqMan low density array was used to assess the expression level of 754 miRNAs in six cases of PTCL-NOS and three control cases of reactive lymphoid hyperplasia. Prediction of target genes for significant and differential expression of miRNAs was carried out using Targetscan and miRanda software. Bioinformatics tools were employed for GO-Analysis and Pathway-Analysis of target genes. The expression patterns of the three miRNAs were further analyzed in the remaining 15 cases of PTCL-NOS and 10 cases of reactive lymphoid hyperplasia, using single tube Taqman miRNA assays.</p><p><b>RESULTS</b>Eight miRNAs showed statistically significant difference in expression between PTCL-NOS and reactive lymphoid hyperplasia. miR-886-3p, miR-511, miR-1291, miR-572, miR-27a-3p, miR-25-3p and miR-886-5p were significantly overexpressed in PTCL-NOS while miR-182-5p was significantly underexpressed (P < 0.05). Target gene prediction showed that 1646 candidate genes involved in the pathogenesis and progression of PTCL-NOS. Further GO and Pathway-Analyses found that these genes significantly focused on 63 GO terms and 61 pathways. The results of three miRNA qRT-PCR confirmed that miR-572 and miR-1291 expression in PTCL-NOS had statistical significance.</p><p><b>CONCLUSIONS</b>Eight miRNAs are aberrantly expressed in PTCL-NOS, which may be involved in molecular regulation during the development of PTCL-NOS. The underlying mechanism is also related to a number of target genes and signaling pathways.</p>

Clinicopathologic study of 40 cases of mediastinal tumours of haematopoietic and lymphoid tissues / 中华病理学杂志

<p><b>OBJECTIVE</b>To study clinical and histopathological features, and diagnosis of mediastinal tumours of haematopoietic and lymphoid tissues (MTHL).</p><p><b>METHODS</b>Forty cases of MTHL were analyzed for clinicopathology by microscopy and immunohistochemical staining and in situ hybridization, according to the updated 2008 WHO classification of tumours of haematopoietic and lymphoid tissues.</p><p><b>RESULTS</b>In 40 cases of MTHL, there were 20 males and 20 females. The ratio of male/female was 1:1. The mean age was 31.8 years and median age was 29 years (range, 12 - 70 years).Superior vena cava syndrome was observed in 28 cases. The specimens of 4 cases were obtained by lumpectomy, whereas 36 cases by biopsy (25 cases by thoracoscopy, 1 by core needle aspiration). Twenty cases lay in anterior mediastinum, and 2 in posterior, 1 in superior, 8 in anterior and superior, 2 in posterior and superior, 2 in anterior and middle, 1 in middle and anterior mediastinum.Frozen section were performed in 28 cases, and 17 cases were diagnosed as tumours of haematopoietic and lymphoid tissues (consistency ratio was 60.7%). Twelve cases were classical Hodgkin lymphomas (cHL) (8 were nodular sclerosis subtype, and 3 were mixed cellarity, 1 was lymphocyte-rich subtype), and 10 were primary mediastinal (thymic) large B cell lymphoma (PMBCL), 10 were precursor lymphocyte neoplasm [8 were T lymphoblastic leukemia/lymphomas (T-LBL), 2 were B-LBL], 1 was MALT lymphoma, 1 was composite lymphoma (PMBCL and cHL), 2 were myeloid sarcomas, 4 were gray zone lymphomas (GZL) (3 had morphology reminiscent of cHL, and 1 of DLBCL, all cases were positive for CD20, PAX5, CD30 and CD15).EBER were detected in 11 cases by in situ hybridization, 2 of which were positive (18.2%), and the 2 positive cases were cHL.</p><p><b>CONCLUSIONS</b>MTHLs occur predominantly in adolescents and young adults, mainly present as superior vena cava syndrome and anterior mediasinal masses. cHL, PMBCL, T-LBL were the most common MTHLs.GZLs mainly occur in young adults, those whose morphology reminiscent of cHL, immunohistochemistry reminiscent of PMBCL, and vice versa. Thoracoscopy, frozen section and a suitable panel of antibodies were practical approaches to MTHL.</p>

Post-transplant lymphoproliferative disorder: a clinicopathologic study of 15 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinical and histopathologic features, diagnosis, pathogenesis and therapy of post-transplant lymphoproliferative disorders (PTLD).</p><p><b>METHODS</b>The clinical and pathologic features of 15 cases of PTLD were retrospectively analyzed by light microscopy, immunohistochemistry and in-situ hybridization, according to the updated 2008 WHO classification of tumors of hematopoietic and lymphoid tissues.</p><p><b>RESULTS</b>Amongst the 15 cases studied, 14 cases had received allogenic hematopoietic stem cell transplantation (AHSCT) and 1 case had received renal transplantation. There were altogether 12 males and 3 females. The male-to-female ratio was 4:1. The mean age was 30.4 years and the median age was 31 years (range from 9 to 60 years). PTLD developed 1.5 to 132 months after transplantation (median 13.0 months). The mean age of the 14 patients with AHSCT was 28.3 years (range from 9 to 45 years) and PTLD developed 1.5 to 19 months after transplantation (mean 4.5 months). Major clinical presentation included fever and lymphadenopathy. Twelve cases involved mainly lymph nodes and the remaining 3 cases involved tonsils, stomach and small intestine, respectively. The histologic types in 4 cases represented early lesions, including plasmacytic hyperplasia (n = 1) and infectious mononucleosis-like PTLD (n = 3). Seven cases were polymorphic PTLD, with 4 cases containing a predominance of large cells. Graft-versus-host disease was also seen in the case of small intestinal involvement. Four cases were monomorphic PTLD, 3 of which were diffuse large B-cell lymphoma, 1 was plasmablastic lymphoma and 1 was a mixture of monomorphic and polymorphic PTLD. Foci of necrosis were seen in 5 cases. The proliferating index of Ki-67 was high. The positive rate of EBV-encoded RNA in AHSCT was 92.9%. The duration of PTLD onset was shorter in EBV-positive cases (range from 1.5 to 7 months) than EBV-negative cases (range from 19 and 132 months). Some cases were treated by reduction of immunosuppression, antiviral agents or anti-CD20 monoclonal antibody Rituximab. The duration of follow-up in 14 patients ranged from 0 to 8 months. Five of the patients died of the disease.</p><p><b>CONCLUSIONS</b>The diagnosis of PTLD relies on morphologic examination and immunohistochemistry. Most of them are of B-cell origin. EBV plays an important role in the pathogenesis of PTLD. The duration of disease onset is shorter in EBV-positive cases. PTLD in AHSCT cases occurs in younger age group, with shorter duration of onset, as compared to solid organ transplantation. The prognosis of PTLD is poor. The modalities of treatment include reduction of immunosuppression, antiviral agents or anti-CD20 monoclonal antibody Rituximab.</p>