[Chronic immunoproliferative syndromes].
Immunoproliferative syndromes are etiologically unclear, rare diseases which involve polyclonal B- and/or T-cell proliferations and frequently develop into malignant non-Hodgkin-lymphoma. Typically, these diseases are characterized by lymphadenopathies, hepatosplenomegaly, general fatigue and autoimmune phenomena. Here we briefly outline 6 syndromes, two of which--the "pluripotential chronic immunoproliferative syndrome" (CPIS) and the "angioimmunoblastic lymphadenopathy" (AILD)--are discussed in more detail. We favor the notion that CPIS is a variant form of AILD clinically dominated by severe autoimmune hemolytic anemia. Both diseases share the high frequency of anti-cytoskeleton autoantibodies.
Abteilung für Rheumatologie und Klinische Immunologie, Medizinische Universitätsklinik Freiburg.
H H
HH
Peter
R
R
Hartel
B
B
Lang
S
S
Weber
D002908:Chronic Disease; D006801:Humans; D007119:Immunoblastic Lymphadenopathy; D007160:Immunoproliferative Disorders; D013577:Syndrome
Chronische immunproliferative Syndrome.