The malignant monoclonal population in hairy cell leukemia (HCL) has been variously ascribed to be of myeloid, B, or even T cell origin. Recent data have been interpreted as suggesting that hairy cells (HC) may concomitantly or serially express both B and T surface determinants, a phenomenon which, if verified, would be unique among the lymphoproliferative malignancies. Data described here, how...

Autoimmune lymphoproliferative syndrome is frequently caused by mutations in genes involved in the Fas death receptor pathway, but for 20-30% of patients the genetic defect is unknown. We observed that treatment of healthy T cells with interleukin-12 induces upregulation of Fas ligand and Fas ligand-dependent apoptosis. Consistently, interleukin-12 could not induce apoptosis in Fas ligand-defic...

We investigated the ability of an FcIgG receptor marker to discriminate between subtypes of malignant lymphoproliferative diseases that differed in their clinical presentations. A quantitative radioimmuno assay was established that enabled us to evaluate average receptor densities on a population basis. Surface receptors were first saturated with IgG complexes. The number of membrane associated...

Autoimmune lymphoproliferative syndrome (ALPS) is a rare, inherited disorder of immune dysregulation secondary to defective lymphocyte apoptosis. This leads to uninhibited proliferation of lymphoid tissue manifesting with lymphadenopathy, hepatosplenomegaly, autoimmune cytopenia, and an increased risk of lymphoid malignancy. We report a 2 year old boy with fever, generalized lymphadenopathy, he...

Delivery of cytotoxic agents specifically to malignant cells has always been a goal of cancer therapy. With the development of monoclonal antibodies for clinical use, this goal has become a reality, and antibody-drug conjugates are now part of standard therapy in diseases such as lymphoma and breast cancer. Brentuximab vedotin, which targets CD30, has contributed to the success of antibody-drug...

An adult patient with acute lymphoblastic leukemia associated with a 14q+ marker chromosome is presented. The abnormality resulted from a translocation of material from the long arm of chromosome 11. The leukemic cells were found to be B cells on the basis of surface immunoglobulins, lack of receptors for sheep erythrocytes, and a characteristically low level of adenosine deaminase activity. In...

EBER: Epstein-Barr encoded RNA EBV: Epstein-Barr virus LPD: lymphoproliferative disorder MTX: methotrexate RA: rheumatoid arthritis INTRODUCTION For more than 30 years, methotrexate (MTX) has been a mainstay in the treatment of various inflammatory disorders including rheumatoid arthritis (RA) and psoriasis. Off-label use of MTX has expanded in recent years, as it is found to be effective in tr...

Castleman's disease is a rare benign lymphoproliferative disorder that frequently affects lymph nodes of the mediastinal thorax and the neck. It very rarely affects the renal sinus. We report a case of Castleman's disease arising in the renal sinus in a 64-year-old man. The patient visited the hospital with the chief complaint of hematuria. Abdominal computed tomography revealed a homogeneous m...