Document 0176
 DOCN  M95B0176
 TI    Proteinaceous (angiocentric sclerosing) lymphadenopathy: a polyclonal
       systemic, nonamyloid deposition disorder.
 DT    9511
 AU    Michaeli J; Niesvizky R; Siegel D; Ladanyi M; Lieberman PH; Filippa DA;
       Division of Hematologic Oncology, Memorial Sloan-Kettering Cancer;
       Center, New York, NY 10021, USA.
 SO    Blood. 1995 Aug 1;86(3):1159-62. Unique Identifier : AIDSLINE
       MED/95345467
 AB    Proteinaceous lymphadenopathy with hypergammaglobulinemia (PLWH) is an
       exceedingly rare disease of unknown etiology. Described primarily as a
       pathologic entity, relatively little is known about its clinical
       manifestations or its response to therapy. The disease is often referred
       to and treated as an unusual form of plasma cell dyscrasia or light
       chain deposition disease. We have recently encountered a young patient
       with PLWH who presented with generalized lymphadenopathy, marked liver
       function abnormalities, hypocomplementemia, cryoglobulinemia, decreased
       T4/T8 ratio, and ophthalmopathy. Contrary to the notion that PLWH is a
       clonal disorder, we found no evidence of clonality in this patient. The
       most characteristic finding in this and in another patient, previously
       seen at our institution, was marked angiocentric hyaline sclerosis of
       the small and mid-sized blood vessels of involved lymph nodes and
       organs. Based on these findings, we propose the term angiocentric
       sclerosing lymphadenopathy, which more accurately defines this
       clinicopathologic entity that appears to be distinct from light chain
       deposition disease and other plasma cell dyscrasias.
 DE    Adult  Amyloid/METABOLISM  Case Report  Clone Cells  CD4-CD8 Ratio
       Female  Human  Hypergammaglobulinemia/COMPLICATIONS  Liver
       Cirrhosis/PATHOLOGY  Lymph Nodes/PATHOLOGY  Lymphoproliferative
       Disorders/METABOLISM/*PATHOLOGY  JOURNAL ARTICLE

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