Central Nervous System ; pathology ; Humans ; Lymphoproliferative Disorders ; pathology ; Transplantation ; adverse effects

Splenic lymphoma with villous lymphocytes (SLVL) or splenic marginal zone lymphoma with circulating villous lymphocytes is rare, and prolymphocytic transformation of SLVL is rarer. At present, only one case of SLVL with t(8;14)(q24;q32) translocation has been reported. In this study, we report a case of B-lymphoproliferative disorder with villous lymphocytes harboring t(8;14)(q24;q32) chromosome translocation that we inclined to SLVL with a prolymphocytic transformation. A 73-year-old female showed marked hepatosplenomegaly and high lymphocytosis (lymphocytes > 200 × 10/L). The abnormal lymphocytes had short coarse villi and round nuclei with prominent nucleoli. The immunophenotypes showed CD19, CD20, HLA-DR, CD22, CD5, Kappa, CD25, CD71, Lambda, CD7, CD10, CD23, CD34, CD33, CD13, CD14, CD117, CD64, CD103, and CD11c. The karyotype showed complex abnormality: 46XX,+ 3,-10, t(8;14)(q24; q32)[11]/46XX[9]. The cytoplasmic projection, immunological characteristics, and trisomy 3 chromosome abnormality supported the diagnosis of SLVL. However, the presence of prominent nucleoli and high lymphocytosis suggested prolymphocytic transformation, probably as a result of t(8,14) chromosome translocation. In this report, we described an unusual case of B-lymphoproliferative disorder with villous lymphocytes harboring t(8;14)(q24;q32) translocation, which could provide help in the diagnosis and differential diagnosis of B-lymphocytic proliferative diseases.
Aged ; B-Lymphocytes ; pathology ; Female ; Humans ; Immunophenotyping ; Lymphoproliferative Disorders ; genetics ; pathology ; Translocation, Genetic

Castleman's disease (CD) is a rare lymphoproliferative disorder that can involve single or multiple lymph nodes in the body. Especially, the localized form of CD is known to be well-controlled by using a surgical resection. On occasion, the surgeon may confront an abdominal and retroperitoneal mass of unknown origin. Thus, we present this case in which we treated a 16-year-old female patient for CD and investigated how to evaluate and manage the situation from the standpoint of CD. Also, we give a review of the pathology, clinical manifestation, diagnosis, and treatment of CD.
Abdomen* ; Adolescent ; Diagnosis ; Female ; Giant Lymph Node Hyperplasia* ; Humans ; Lymph Nodes ; Lymphoproliferative Disorders ; Pathology, Clinical

Epstein-Barr Virus Infections ; Herpesvirus 4, Human ; Humans ; Lymphoproliferative Disorders ; classification ; pathology ; virology

OBJECTIVETo evaluate clinical and pathological features of post-transplant lymphoproliferative disorders (PTLD) and its significance in diagnosis.

METHODSSix cases of PTLD were studied by light microscope, immunohistochemistry, in-situ hybridization, and gene rearrangement analysis. The clinical and follow-up information were also reviewed.

RESULTSAmong the 6 cases, 3 with monomorphic PTLD were renal transplant recipients, and died 4, 2, and 1 months after diagnosis. 2 were liver transplant recipients, 1 of whom with monomorphic PTLD died 5 months after diagnosis, the other one was diagnosed as early lesion of PTLD and the post-bone marrow transplant case was classified as polymorphic PTLD who survived for 12 months after diagnosis of PTLD. EBER 1/2 DNA was demonstrated in 4 cases.

CONCLUSIONSPTLD is a lymphoproliferative disease with distinctive morphologic and clinical characteristics after organ transplantation. The prognosis of PTLD correlates with the pathological subtypes and clinical stage.

Adult ; Female ; Follow-Up Studies ; Humans ; Lymphoproliferative Disorders ; etiology ; pathology ; Male ; Middle Aged ; Postoperative Complications ; pathology ; Prognosis ; Transplantation, Homologous ; pathology

Humans ; Lymphoma ; classification ; pathology ; Lymphoma, B-Cell ; classification ; pathology ; Lymphoma, T-Cell ; classification ; pathology ; Lymphoproliferative Disorders ; pathology ; Pseudolymphoma ; pathology ; Skin Neoplasms ; classification ; pathology ; World Health Organization

The aim of this study was to analyze the clinical features and laboratory findings of adult Epstein-Barr virus associated T/NK cell lymphoproliferative disease (EBV+T/NK-LPD) and to investigate the early diagnosis and prognosis of EBV+T/NK-LPD. The clinical data of 19 adult patients with EBV+T/NK-LPD were retrospectively analyzed. The results indicated that there were 11 males and 8 females. The median age was 32 years (range: 20-70 years). The average duration from onset of symptoms to diagnosis was 3.5 months. The median survival time was 2.5 months. Unkown fever, hepatosplenomegaly, liver dysfunction and interstitial pneumonia were the main clinical features. High levels of β2-MG, LDH, TNF, IL-6 and significantly increased EBV-DNA level (median level > 10(6) copies/ml) were occurred in all the patients. Cytopenia was seen in 18 cases. Morphologically, atypical large granular lymphocytes and hemophagocytosis were common in bone marrow smears. Deletion of CD5 or CD7 were frequently observed in T/NK lymphocytes in bone marrow cells by flow cytometry. Bone marrow biopsy showed atypical lymphocyte interstitial infiltrated in 10 cases, while a few large cells infiltrated in 6 cases. Immunohistochemistry showed the expression of CD3(+)CD56(+) were seen in 2 cases, CD3(+)CD8(+) in 11 cases and CD3(+)CD4(+) in 3 cases. TIA-1 and EBER were positive in all biopsy specimens. Three cases underwent biopsy of lymph nodes showed reactive proliferations of lymphocytes. All the patients died of multiorgan failure. It is concluded that the fever, hepatosplenomegaly are the most common clinical features in adult EBV+T/NK-LPD, the bone marrow infiltration of EBV-infected T/NK lymphocytes and significantly increased EBV-DNA level can be observed in all cases, the clinical outcome of this disease is poor, these clinical and experimental features can be served as a reliable marker for the timely diagnosis of adult EBV+T/NK-LPD.
Adult ; Aged ; Epstein-Barr Virus Infections ; pathology ; Female ; Humans ; Immunophenotyping ; Killer Cells, Natural ; virology ; Lymphoproliferative Disorders ; pathology ; virology ; Male ; Middle Aged ; Retrospective Studies ; T-Lymphocytes ; virology ; Young Adult

OBJECTIVETo investigate the expression of microRNA-155 and microRNA-146a in the CD19(+) B cells of chronic lymphocytic leukemia (CLL), mantle cell lymphoma (MCL), splenic marginal zone lymphoma (SMZL), and to analyze its clinical significance.

METHODSPeripheral blood (PB) (78 cases) and bone marrow (BM) samples (9 cases) from 53 CLL patients, 13 MCL patients, 19 SMZL patients, and 12 healthy donors were collected. Mononuclear cells were isolated and B cells were purified with a CD19(+) magnetic-bead system. Total RNA was extracted from purified CD19(+) cells and microRNAs expression were measured using the TaqMan microRNA quantitative PCR. The results combined with the clinic data of patients were analysed.

RESULTS(1) The expression of microRNA-155 in CLL (4.49 ± 0.83) was significantly higher than in MCL (3.83 ± 0.45) and SMZL (3.80 ± 0.61) (P < 0.05); (2) The level of microRNA-146a in SMZL (3.81 ± 0.59) was significantly higher than in CLL (2.58 ± 0.90) and MCL (2.27 ± 0.88) (P < 0.01); (3) The level of microRNA-155 was significantly higher in IgVH unmutated patients than in mutated patients in CLL (P = 0.012); (4) The microRNAs expression had no statistical difference between two prognostic groups in CLL.

CONCLUSION(1) The expression of microRNA-155 and microRNA-146a is different in malignant lymphoproliferative disorders (LPD); (2) Deregulation of the microRNAs expression might play a critical role in the pathogenesis and prognosis in the LPD.

B-Lymphocytes ; metabolism ; Case-Control Studies ; Chronic Disease ; Humans ; Leukemia, Lymphocytic, Chronic, B-Cell ; genetics ; pathology ; Lymphoproliferative Disorders ; genetics ; pathology ; MicroRNAs ; metabolism

Molecular pathologic testing plays an important role for the diagnosis, prognostication and decision of treatment strategy in lymphoproliferative disease. Here, we briefly review the molecular tests currently used for lymphoproliferative disease and those which will be implicated in clinical practice in the near future. Specifically, this guideline addresses the clonality test for B- and T-cell proliferative lesions, molecular cytogenetic tests for malignant lymphoma, determination of cell-of-origin in diffuse large B-cell lymphoma, and molecular genetic alterations incorporated in the 2016 revision of the World Health Organization classification of lymphoid neoplasms. Finally, a new perspective on the next-generation sequencing for diagnostic, prognostic, and therapeutic purpose in malignant lymphoma will be summarized.
Classification ; Cytogenetics ; Diagnosis ; In Situ Hybridization, Fluorescence ; Lymphoma ; Lymphoma, B-Cell ; Lymphoproliferative Disorders* ; Molecular Biology ; Pathology, Molecular ; T-Lymphocytes ; World Health Organization

Epstein-Barr virus (EBV) is a ubiquitous herpesvirus, affecting >90% of the adult population. EBV targets B-lymphocytes and achieves latent infection in a circular episomal form. Different latency patterns are recognized based on latent gene expression pattern. Latent membrane protein-1 (LMP-1) mimics CD40 and, when self-aggregated, provides a proliferation signal via activating the nuclear factor-kappa B, Janus kinase/signal transducer and activator of transcription, phosphoinositide 3-kinase/Akt (PI3K/Akt) and mitogen-activated protein kinase pathways to promote cellular proliferation. LMP-1 also induces BCL-2 to escape from apoptosis and gives a signal for cell cycle progression by enhancing cyclin-dependent kinase 2 and phosphorylation of retinoblastoma (Rb) protein and by inhibiting p16 and p27. LMP-2A blocks the surface immunoglobulin-mediated lytic cycle reactivation. It also activates the Ras/PI3K/Akt pathway and induces Bcl-xL expression to promote B-cell survival. Recent studies have shown that ebv-microRNAs can provide extra signals for cellular proliferation, cell cycle progression and anti-apoptosis. EBV is well known for association with various types of B-lymphocyte, T-lymphocyte, epithelial cell and mesenchymal cell neoplasms. B-cell lymphoproliferative disorders encompass a broad spectrum of diseases, from benign to malignant. Here we review our current understanding of EBV-induced lymphomagenesis and focus on biology, diagnosis and management of EBV-associated B-cell lymphoproliferative disorders.
B-Lymphocytes/*pathology/*virology ; Diagnosis, Differential ; Disease Management ; Epstein-Barr Virus Infections/*complications ; Herpesvirus 4, Human/*physiology ; Humans ; Lymphoproliferative Disorders/*diagnosis/*etiology/therapy