Angio-immunoblastic lymphadenopathy: a clinical, immunological and molecular study. 1987

T S Ganesan, and H S Dhaliwal, and M S Dorreen, and A G Stansfeld, and J A Habeshaw, and T A Lister

Twenty four patients with angio-immunoblastic lymphadenopathy (AILD) presenting between 1974 and 1985 have been reviewed. Clinical features at presentation included rash, fever, lymphadenopathy and hepatosplenomegaly in 75% of patients. Polyclonal hypergammaglobulinaemia was seen in 19/20 patients; 5 had normal immunoglobulin levels. Combination chemotherapy with MVPP was the optimal treatment with 6/7 patients achieving complete remission. Duration of remission ranged from 9 months to 4 years and was significantly longer in patients achieving complete as opposed to partial remission. In 6 patients phenotype studies were performed on single cell suspensions and immunoperoxidase studies on frozen sections of 7 lymph nodes. There was a reversal of the normal T suppressor/helper cell ratio with a predominance of T suppressor cells. Loss of normal B follicles was observed histologically in all except one lymph node. Germline configuration of the beta B-chain of the T cell receptor was observed in lymph nodes of 4 patients with AILD, and a rearranged T cell receptor was observed in 1 patient in whom a second lymph node biopsy had shown alteration of the histological picture to that of T-zone lymphoma. Frozen sera of 15 patients were screened for antibodies to HTLV I and III and were found to be negative.

UI MeSH Term Description Entries
D007119 Immunoblastic Lymphadenopathy A disorder characterized by proliferation of arborizing small vessels, prominent immunoblastic proliferations and amorphous acidophilic interstitial material. Clinical manifestations include fever, sweats, weight loss, generalized lymphadenopathy and frequently hepatosplenomegaly. Angioimmunoblastic Lymphadenopathy,Lymphadenopathy, Immunoblastic,Angioimmunoblastic Lymphadenopathies,Immunoblastic Lymphadenopathies,Lymphadenopathies, Angioimmunoblastic,Lymphadenopathies, Immunoblastic,Lymphadenopathy, Angioimmunoblastic
D007136 Immunoglobulins Multi-subunit proteins which function in IMMUNITY. They are produced by B LYMPHOCYTES from the IMMUNOGLOBULIN GENES. They are comprised of two heavy (IMMUNOGLOBULIN HEAVY CHAINS) and two light chains (IMMUNOGLOBULIN LIGHT CHAINS) with additional ancillary polypeptide chains depending on their isoforms. The variety of isoforms include monomeric or polymeric forms, and transmembrane forms (B-CELL ANTIGEN RECEPTORS) or secreted forms (ANTIBODIES). They are divided by the amino acid sequence of their heavy chains into five classes (IMMUNOGLOBULIN A; IMMUNOGLOBULIN D; IMMUNOGLOBULIN E; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M) and various subclasses. Globulins, Immune,Immune Globulin,Immune Globulins,Immunoglobulin,Globulin, Immune
D007958 Leukocyte Count The number of WHITE BLOOD CELLS per unit volume in venous BLOOD. A differential leukocyte count measures the relative numbers of the different types of white cells. Blood Cell Count, White,Differential Leukocyte Count,Leukocyte Count, Differential,Leukocyte Number,White Blood Cell Count,Count, Differential Leukocyte,Count, Leukocyte,Counts, Differential Leukocyte,Counts, Leukocyte,Differential Leukocyte Counts,Leukocyte Counts,Leukocyte Counts, Differential,Leukocyte Numbers,Number, Leukocyte,Numbers, Leukocyte
D008198 Lymph Nodes They are oval or bean shaped bodies (1 - 30 mm in diameter) located along the lymphatic system. Lymph Node,Node, Lymph,Nodes, Lymph
D008297 Male Males
D008466 Mechlorethamine A biologic alkylating agent that exerts its cytotoxic effects by forming DNA ADDUCTS and DNA interstrand crosslinks, thereby inhibiting rapidly proliferating cells. The hydrochloride is an antineoplastic agent used to treat HODGKIN DISEASE and LYMPHOMA. Chlorethazine,Chlormethine,Mechlorethamine Oxide,Mustine,Nitrogen Mustard,Nitrogen Mustard N-Oxide,Bis(2-chloroethyl)methylamine,Caryolysine,Cloramin,Embichin,Mechlorethamine Hydrochloride,Mechlorethamine Hydrochloride N-Oxide,Mechlorethamine N-Oxide,Methylchlorethamine,Mitomen,Mustargen,NSC-10107,NSC-762,Nitrogranulogen,Nitromin,Hydrochloride N-Oxide, Mechlorethamine,Hydrochloride, Mechlorethamine,Mechlorethamine Hydrochloride N Oxide,Mechlorethamine N Oxide,N-Oxide, Mechlorethamine Hydrochloride,N-Oxide, Nitrogen Mustard,NSC 10107,NSC 762,NSC10107,NSC762,Nitrogen Mustard N Oxide
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010641 Phenotype The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment. Phenotypes
D011239 Prednisolone A glucocorticoid with the general properties of the corticosteroids. It is the drug of choice for all conditions in which routine systemic corticosteroid therapy is indicated, except adrenal deficiency states. Di-Adreson-F,Predate,Predonine,Di Adreson F,DiAdresonF
D011344 Procarbazine An antineoplastic agent used primarily in combination with mechlorethamine, vincristine, and prednisone (the MOPP protocol) in the treatment of Hodgkin's disease. Matulane,Natulan,Procarbazine Hydrochloride,Procarbazine Monohydrobromide,Procarbazine Monohydrochloride,Hydrochloride, Procarbazine,Monohydrobromide, Procarbazine,Monohydrochloride, Procarbazine

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