Phenotype / cell stem origin The lymphoma cell is a peripheral T lymphocyte in various stages of differentiation. The neoplastic clone expresses T-cell antigens and is usually CD4+. The malignant T-cells are believed to secrete cytokines responsible for the polyclonal B-cell hyperplasia observed in involved nodes. Clonality studies demonstrated a monoclonal rearrangement of the b-chain of the T-cell receptor (TCR) in the majority of cases. In some cases clonality could not be demonstrated. This led some authors to postulate the existence of at least two types of AILD, namely a reactive and benign type and a lymphomatous form. Etiology The disease is rare Clinics The disease preferentially affects elderly males (male-to-female ratio 3:1, median age around 60 years). Most patients present with generalized lymphadenopathy, hepatosplenomegaly, skin rash and general symptoms (fever, weight loss). Polyclonal hypergammaglobulinemia is a common finding. Pathology The lymph node architecture is effaced and no reactive germinal centres are usually observed. The infiltrate may involve the perinodal fat. There is a proliferation of high endothelial venules with clusters of follicular dendritic cells. The lymphoid infiltrate consists of small-to-large cells resembling immunoblasts and atypical clear cells with round nucleus and abundant pale cytoplasm. The latter cells may occur in small aggregates or sheets. Treatment Some patients respond to steroids; in steroid-unresponsive patients multiagent chemotherapy usually produces short lasting responses. Evolution Few patients present spontaneous or steroid-induced remission; the majority of cases feature an aggressive disease with short survival despite chemotherapy. Most patients die with infection and active disease. Prognosis Median survival is about 1-3 years. Cytogenetics Note A mixture of normal and abnormal cells is usually seen in the vast majority of cases. The cytogenetic picture at disease presentation may be normal in some cases which may develop clonal abnormalites during the course of the disease. The following karyotype pattern can be found Cytogenetics Morphological Clonal abnormalities defining a stemline, with one or more sidelines (approximately 30-50% of the cases) Normal karyotype (10-30% of the cases) Single cells with unrelated chromosome anomalies (10-20%) Unrelated clones with aberrant karyotypes, each carrying single unrelated additional anomalies (10-20%). Recurrent chromosome changes in those cases with an abnormal clone include trisomy 3, trisomy 5 and trisomy X A 14q+ chromosome is a recurrent structural defect. Recurrent breakpoints include 1p31-32; 3p24-25; 4p13; 9q21-22; 12q13; 14q11; 14q32 The presence of abnormal metapahses in unstimulated cultures was associated with failure to respond to therapy and with shorter survival, as was the case with +X, structural aberrations of chromosome 1, and complex karyotype. The latter cytogenetic parameter maintained prognostic predictivity at multivariate analysis. Cytogenetics Molecular Using probes for the detection of +3, +5 and +X, the vast majority of cases can be shown to carry aneuploidy

Angioimmunoblastic lymphadenopathy

CUNEO, Antonio
2002

Abstract

Phenotype / cell stem origin The lymphoma cell is a peripheral T lymphocyte in various stages of differentiation. The neoplastic clone expresses T-cell antigens and is usually CD4+. The malignant T-cells are believed to secrete cytokines responsible for the polyclonal B-cell hyperplasia observed in involved nodes. Clonality studies demonstrated a monoclonal rearrangement of the b-chain of the T-cell receptor (TCR) in the majority of cases. In some cases clonality could not be demonstrated. This led some authors to postulate the existence of at least two types of AILD, namely a reactive and benign type and a lymphomatous form. Etiology The disease is rare Clinics The disease preferentially affects elderly males (male-to-female ratio 3:1, median age around 60 years). Most patients present with generalized lymphadenopathy, hepatosplenomegaly, skin rash and general symptoms (fever, weight loss). Polyclonal hypergammaglobulinemia is a common finding. Pathology The lymph node architecture is effaced and no reactive germinal centres are usually observed. The infiltrate may involve the perinodal fat. There is a proliferation of high endothelial venules with clusters of follicular dendritic cells. The lymphoid infiltrate consists of small-to-large cells resembling immunoblasts and atypical clear cells with round nucleus and abundant pale cytoplasm. The latter cells may occur in small aggregates or sheets. Treatment Some patients respond to steroids; in steroid-unresponsive patients multiagent chemotherapy usually produces short lasting responses. Evolution Few patients present spontaneous or steroid-induced remission; the majority of cases feature an aggressive disease with short survival despite chemotherapy. Most patients die with infection and active disease. Prognosis Median survival is about 1-3 years. Cytogenetics Note A mixture of normal and abnormal cells is usually seen in the vast majority of cases. The cytogenetic picture at disease presentation may be normal in some cases which may develop clonal abnormalites during the course of the disease. The following karyotype pattern can be found Cytogenetics Morphological Clonal abnormalities defining a stemline, with one or more sidelines (approximately 30-50% of the cases) Normal karyotype (10-30% of the cases) Single cells with unrelated chromosome anomalies (10-20%) Unrelated clones with aberrant karyotypes, each carrying single unrelated additional anomalies (10-20%). Recurrent chromosome changes in those cases with an abnormal clone include trisomy 3, trisomy 5 and trisomy X A 14q+ chromosome is a recurrent structural defect. Recurrent breakpoints include 1p31-32; 3p24-25; 4p13; 9q21-22; 12q13; 14q11; 14q32 The presence of abnormal metapahses in unstimulated cultures was associated with failure to respond to therapy and with shorter survival, as was the case with +X, structural aberrations of chromosome 1, and complex karyotype. The latter cytogenetic parameter maintained prognostic predictivity at multivariate analysis. Cytogenetics Molecular Using probes for the detection of +3, +5 and +X, the vast majority of cases can be shown to carry aneuploidy
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in SFERA sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11392/1190772
 Attenzione

Attenzione! I dati visualizzati non sono stati sottoposti a validazione da parte dell'ateneo

Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus ND
  • ???jsp.display-item.citation.isi??? ND
social impact