Immunoblastic lymphadenopathy with purpura and cryoglobulinemia. 1977

H L Wechsler, and A Stavrides

Immunoblastic lymphadenopathy is a recently described lymphoproliferative disorder, presumably of B-cell origin. It is characterized by regional or generalized lymphadenopathy, usually associated with hypergammaglobulinemia or dysproteinemia. Other findings may be hepatosplenomegaly, dermatitis, fever, malaise, weight loss, and various altered immunologic reactions. Histologically, the involved lymph nodes show immunoblast, plasmacytoid, and plasma cell proliferation. This may be extranodal as well. The case reported here is one of the few followed up prospectively. The patient's purpuric eruption was an apparent manifestation of a type II mixed cryoglobulinemia. Differing from what has usually been reported, we noted hypogammaglobulinemia and findings in part of altered cell-mediated immunity. Despite leukopenia and anemia there were no infectious episodes. Although a satisfactory treatment regimen has not been established, there was beneficial response to prednisone and short courses of melphalan.

UI MeSH Term Description Entries
D007970 Leukopenia A decrease in the number of LEUKOCYTES in a blood sample below the normal range (LEUKOCYTE COUNT less than 4000). Leukocytopenia,Leukocytopenias,Leukopenias
D008206 Lymphatic Diseases Diseases of LYMPH; LYMPH NODES; or LYMPHATIC VESSELS. Lymphatism,Status Lymphaticus,Disease, Lymphatic,Diseases, Lymphatic,Lymphatic Disease
D008558 Melphalan An alkylating nitrogen mustard that is used as an antineoplastic in the form of the levo isomer - MELPHALAN, the racemic mixture - MERPHALAN, and the dextro isomer - MEDPHALAN; toxic to bone marrow, but little vesicant action; potential carcinogen. Medphalan,Merphalan,Phenylalanine Mustard,Sarcolysine,Sarkolysin,4-(Bis(2-chloroethyl)amino)phenylalanine,Alkeran,L-PAM,Mustard, Phenylalanine
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010265 Paraproteinemias A group of related diseases characterized by an unbalanced or disproportionate proliferation of immunoglobulin-producing cells, usually from a single clone. These cells frequently secrete a structurally homogeneous immunoglobulin (M-component) and/or an abnormal immunoglobulin. Gammapathy, Monoclonal,Gammopathy, Monoclonal,Monoclonal Gammopathies,Paraimmunoglobulinemia,Paraimmunoglobulinemias,Paraproteinemia,Plasma Cell Dyscrasias,Monoclonal Gammapathies,Monoclonal Gammopathy,Cell Dyscrasia, Plasma,Dyscrasia, Plasma Cell,Monoclonal Gammapathy,Plasma Cell Dyscrasia
D011241 Prednisone A synthetic anti-inflammatory glucocorticoid derived from CORTISONE. It is biologically inert and converted to PREDNISOLONE in the liver. Dehydrocortisone,delta-Cortisone,Apo-Prednisone,Cortan,Cortancyl,Cutason,Dacortin,Decortin,Decortisyl,Deltasone,Encorton,Encortone,Enkortolon,Kortancyl,Liquid Pred,Meticorten,Orasone,Panafcort,Panasol,Predni Tablinen,Prednidib,Predniment,Prednison Acsis,Prednison Galen,Prednison Hexal,Pronisone,Rectodelt,Sone,Sterapred,Ultracorten,Winpred,Acsis, Prednison
D011694 Purpura, Hyperglobulinemic Purplish or brownish red discoloration of the skin associated with increase in circulating polyclonal globulins, usually GAMMA-GLOBULINS. This syndrome often occurs on the legs of women aged 20 to 40 years. Benign Hyperglobulinemic Purpura of Waldenström,Hypergammaglobulinemic Purpura of Waldenstrom,Hyperglobulinemic Purpura,Hyperglobulinemic Purpura of Waldenström,Waldenstrom Hyperglobulinemic Purpura,Hyperglobulinemic Purpura, Waldenstrom,Hyperglobulinemic Purpuras,Purpura, Waldenstrom Hyperglobulinemic,Purpuras, Hyperglobulinemic,Waldenstrom Hypergammaglobulinemic Purpura,Waldenström Hyperglobulinemic Purpura
D003450 Cryoglobulins Abnormal immunoglobulins, especially IGG or IGM, that precipitate spontaneously when SERUM is cooled below 37 degrees Celsius. It is characteristic of CRYOGLOBULINEMIA. Cryoglobulin,Cryoprotein,Cryoproteins
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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