Monoclonal B cell proliferation in lymphoproliferative disease associated with herpes virus type 6 infection.
Clonal gene rearrangements and aneuploidy are commonly recognized as characteristics of malignancy. We challenge this by an observation of a 3 1/2 year old boy with chronic benign lymphadenopathy, connatal splenomegaly and peripheral lymphocytosis. His brother and mother suffered from similar disease without progression. Cervical lymph node biopsy from our patient revealed a benign histology corresponding to the clinical course; however, clonal rearrangements of the JH and TCR gamma gene were demonstrated in lymph node cells and DNA aneuploidy in 11% of bone marrow cells. Immunohistology showed an inverse T-helper/T-suppressor cell ratio with polyclonal B cell increase and extensive lymphocyte activation. In situ hybridization demonstrated foci of lymphoid cells in the paracortical zone with expression of herpes virus type 6 genome. In addition, the patient, his brother and his mother showed serological evidence of active infection with the human herpes virus-6 (HHV-6). The association of HHV-6 infection, monoclonality and benign lymphoproliferation is discussed.
Berthold
F
F
Children's Hospital, University of Cologne, F.R.G.
Krueger
G R
GR
Tesch
H
H
Hiddemann
W
W
D000917:Antibody Formation; D001402:B-Lymphocytes / Q000276:immunology*; D015139:Blotting, Southern; D001853:Bone Marrow / Q000382:microbiology / Q000473:pathology; D002675:Child, Preschool; D003165:Complement System Proteins / Q000032:analysis; D004279:DNA, Viral / Q000302:isolation & purification; D005434:Flow Cytometry; D005814:Genes, Viral; D006561:Herpes Simplex / Q000150:complications / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D008198:Lymph Nodes / Q000276:immunology / Q000473:pathology; D008213:Lymphocyte Activation; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000276:immunology* / Q000473:pathology; D008297:Male; D018139:Simplexvirus / Q000235:genetics / Q000302:isolation & purification
D004279:DNA, Viral; D007136:Immunoglobulins; D003165:Complement System Proteins