Humans ; Anaplastic Lymphoma Kinase ; Histiocytosis, Langerhans-Cell ; Receptor Protein-Tyrosine Kinases ; Lymphoma, Large-Cell, Anaplastic/pathology*

Humans ; Lymphoma, Large-Cell, Anaplastic/pathology* ; PAX5 Transcription Factor/genetics*

ALK negative anaplastic large cell lymphoma (ALK(-) ALCL) lacks the specific expression of ALK protein, although it also strongly expresses CD30 and resembles the morphologic characteristics of ALK positive anaplastic large cell lymphoma (ALK(+) ALCL). Recently, some new researches indicate that there exist molecular and genetic differences between these two types of ALCL. Moreover, the treatment response, prognosis, and long-term survival of ALK(-) ALCL are far worse than that of ALK(+) ALCL, such as ALK(-) ALCL is associated with older age persons, B group syndrome, disease advanced stage, high International Prognostic Index (IPI) and poor prognosis (< 49% 5-year survival). As a consequence, some new advances on basis (cell morphology and tissue pathology, immunophenotypes, cell genetics and molecular marker), diagnosis and treatment of ALK(-) ALCL are summarized in this review.
Biomarkers, Tumor ; Humans ; Lymphoma, Large B-Cell, Diffuse ; classification ; pathology ; Lymphoma, Large-Cell, Anaplastic ; classification ; pathology

Humans ; Lymphoma, Large-Cell, Anaplastic/pathology* ; Receptor Protein-Tyrosine Kinases ; Anaplastic Lymphoma Kinase ; Central Nervous System/pathology*

Objective: To investigate the clinicpathological characteristics of ALK-positive anaplastic large cell lymphoma (ALCL) of the gastrointestinal tract, and to discuss its diagnosis and differential diagnosis. Methods: Five cases of gastrointestinal ALK-positive ALCL diagnosed and treated in Xijing Hospital of the Fourth Military Medical University, between 2011 and 2019 were collected. There were three male and two female patients, aged 5-42 years (mean 25 years). These patients clinically presented with fever and night sweats, weight loss, abdominal pain, abdominal mass, ulcers, bleeding, or intestinal obstruction, and underwent surgical resection of the tumors or endoscopic biopsy. The clinical manifestations, auxiliary examinations, histopathological characteristics, immunophenotypes and genetic alterations were analyzed. Results: In this cohort, one case was common type, two cases were monomorphic variant of common type, and two cases were small cell variant. The tumor cells in all cases expressed ALK, CD30, and one or more T lymphocyte markers, while all the markers of B lymphocyte and plasmacyte were negative. Clonality analysis showed that two cases had clonal T cell receptor (TCR) and immunoglobulin (Ig) gene rearrangement, one case had no clonal TCR but Ig gene rearrangement, and one case had no clonal TCR and Ig gene rearrangements. During the 4 to 67 months' follow-up, two patients died of the disease, two were alive with free of disease and one had a relapse. Conclusions: ALK-positive ALCL of the gastrointestinal tract is extremely rare, and has poor prognosis. Lymphoma originating from this site with CD30 and ALK-positive phenotypes may be considered to be ALCL; however differentiation from other tumors that had anaplastic features, expressed CD30 and or ALK, in particular, ALK positive large B-cell lymphoma is necessary.
Male ; Female ; Humans ; Lymphoma, Large-Cell, Anaplastic/pathology* ; Receptor Protein-Tyrosine Kinases/genetics* ; Anaplastic Lymphoma Kinase ; Gastrointestinal Tract/pathology* ; Lymphoma, Large B-Cell, Diffuse/genetics*

Adolescent ; Female ; Humans ; Lymphoma, Large-Cell, Anaplastic ; pathology ; Neutrophils ; pathology ; Skin Neoplasms ; pathology

OBJECTIVETo investigate the frequency of different types of mature T- and NK-cell lymphomas diagnosed in a 4-year period at Sun Yat-sen University Cancer Center, and to study baseline CD30 for potential anti-CD30 targeted therapy in mature T- and NK-cell lymphoma.

METHODSAll cases of mature T- and NK-cell lymphoma diagnosed at Sun Yat-sen University Cancer Center from September 1, 2009 to August 31, 2013, were reviewed. Paraffin-blocks of available 164 consecutive cases were stained for CD30 immunohistochemistry using EnVision protocol.

RESULTSA total of 625 cases of mature T- and NK-cell lymphomas were diagnosed and the most common type was extranodal NK/T cell lymphoma (ENKTL), nasal type 319 (51.0%) cases, followed by angioimmunoblastic T-cell lymphoma (AITL) (119 cases, 19.0%), peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) (81 cases, 13.0%), and anaplastic large-cell lymphoma (ALCL), including 48 cases (7.7%) of systematic ALCL and 11 cases (1.8%) of primary cutaneous ALCL. Besides ALCL, ENKTL had the highest expression rate of CD30 among the 164 cases, with positivity observed in 41 cases (62.1%, 41/66). Only 1 case of PTCL-NOS was CD30 positive. CD30 was not expressed in all 28 cases of AITL and other rare types of mature T- and NK-cell lymphoma.

CONCLUSIONSThe frequency of different types of mature T- and NK-cell lymphoma encountered at Sun Yat-sen University Cancer Center was similar to that seen in other areas of China and other Asia countries. CD30 expression is different among several types of mature T- and NK-cell lymphoma. In addition to ALCL, ENKTL has the highest expression rate of CD30, which may be a candidate disease for anti-CD30 targeted therapy.

China ; epidemiology ; Humans ; Immunohistochemistry ; Killer Cells, Natural ; Lymphoma, Extranodal NK-T-Cell ; epidemiology ; pathology ; Lymphoma, Large-Cell, Anaplastic ; epidemiology ; pathology ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; epidemiology ; pathology ; Lymphoma, T-Cell, Peripheral ; epidemiology ; pathology ; T-Lymphocytes

Primary cutaneous anaplastic large cell lymphoma is a rare non-Hodgkin's lymphoma.The tumor cells have the characteristics of anaplastic cells,expressing CD30 but not anaplastic lymphoma kinase.In this study,we reported a case of primary cutaneous anaplastic large cell lymphoma in a Tibetan child and summarized the clinicopathological features,aiming to strengthen the understanding of this disease.
Child ; Humans ; Ki-1 Antigen ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Skin Neoplasms/pathology*

Dendritic Cells, Follicular ; pathology ; Diagnosis, Differential ; Humans ; Leukemia, Lymphocytic, Chronic, B-Cell ; pathology ; Lymphoma ; pathology ; Lymphoma, Extranodal NK-T-Cell ; pathology ; Lymphoma, Follicular ; pathology ; Lymphoma, Large B-Cell, Diffuse ; pathology ; Lymphoma, Large-Cell, Anaplastic ; pathology ; Lymphoma, Mantle-Cell ; pathology ; Lymphoma, T-Cell, Peripheral ; pathology ; Neoplasm Invasiveness ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; pathology

OBJECTIVETo explore the clinicopathologic features of CD30-positive sinusoidal large B-cell lymphoma (CD30 + SLBCL) and its relative correlation with Epstein-Barr virus (EBV).

METHODSTwo cases of CD30 + SLBCL, a 65-year-old men and a 85-year-old women were morphologically and immunophenotypically analyzed. EBV status was also evaluated through not only the polymerase chain reaction (PCR) amplification to the EBV Bam HIW DNA sequence, but also an immunohistochemical detection of the latent membrane protein 1 (LMP1).

RESULTSThe patients presented with similarly superficial lymphadenopathy. One of them died of the tumor within 10 months. Microscopically, both of the neoplasms were characterized by a cohesive sinus growth pattern and the monomorphic cytology of the tumor cells. Immunohistochemically, They were both positive for CD45, CD30, and CD20 or CD79alpha, whereas neither expressed EMA, ALK1, nor any histiocytic/T-lineage markers. No evidence of EBV-infection could be found either.

CONCLUSIONSCD30 + SLBCL is a morphologically and immunophenotypically distinctive variant of diffuse large B-cell lymphoma, which should be distinguished from T/null cell type anaplastic large cell lymphoma and some other nodal lesions with a predominantly sinusoidal infiltrative pattern. CD30 + SLBCL may not be correlation with EBV.

Aged ; Aged, 80 and over ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Ki-1 Antigen ; analysis ; Lymphoma, B-Cell ; diagnosis ; pathology ; Lymphoma, Large-Cell, Anaplastic ; diagnosis ; pathology ; Male ; Middle Aged