A chronic "postinfectious" fatigue syndrome associated with benign lymphoproliferation, B-cell proliferation, and active replication of human herpesvirus-6.
A 17-year-old, previously healthy woman developed an acute "mononucleosis-like" illness with an associated "atypical" pneumonitis, followed by years of debilitating chronic fatigue, fevers, a 10-kg weight loss, night sweats, and neurocognitive symptoms. Thereafter, her sister developed a similar but less severe illness. The patient developed marked, chronic lymphadenopathy and splenomegaly, with associated persistent relative lymphocytosis and atypical lymphocytosis and with thrombocytopenia. After 3 years of illness, a splenectomy was performed, which resulted in some symptomatic improvement, prompt weight gain, and resolution of all hematologic abnormalities. Serial immunologic studies revealed a strikingly elevated number of activated B lymphocytes and a T lymphopenia, which improved but did not return to normal postsplenectomy. No causal association was found with any of several infectious agents that could produce such a lymphoproliferative illness. However, both the patient and her sister had evidence of active infection with the recently discovered human herpesvirus-6. Seven years after the onset of the illness, the patient and her sister remain chronically ill.
Buchwald
D
D
University of Washington School of Medicine, Seattle 98121.
Freedman
A S
AS
Ablashi
D V
DV
Sullivan
J L
JL
Caligiuri
M
M
Weinberg
D S
DS
Hall
C G
CG
Ashley
R L
RL
Saxinger
C
C
Balachandran
N
N
D000293:Adolescent; D000328:Adult; D000914:Antibodies, Viral / Q000097:blood; D000954:Antigens, Surface / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology*; D015673:Fatigue Syndrome, Chronic / Q000276:immunology* / Q000601:surgery; D005260:Female; D006566:Herpesviridae Infections / Q000276:immunology*; D004854:Herpesvirus 4, Human / Q000276:immunology; D015654:Herpesvirus 6, Human / Q000276:immunology / Q000502:physiology*; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000276:immunology; D008213:Lymphocyte Activation / Q000276:immunology; D008232:Lymphoproliferative Disorders / Q000209:etiology*; D013156:Splenectomy; D013601:T-Lymphocytes / Q000276:immunology; D014779:Virus Replication / Q000276:immunology
D000914:Antibodies, Viral; D000954:Antigens, Surface