Large granular lymphocyte proliferative disease: 21 Belgian cases and review of the literature.
We report the findings in 21 Belgian patients (12 males and 9 females, median age 61 years) with LGLPD. Symptoms at presentation included infection (n = 9), weight loss (n = 5), asthenia (n = 9), pruritus (n = 2) and arthralgia (n = 7). Four patients were asymptomatic. The main clinical findings were hepatomegaly (n = 5), splenomegaly (n = 8), lymph node enlargement (n = 3) and arthritis (n = 5). All patients had an increased LGL count associated with anemia (n = 12), neutropenia (n = 17), often less than 0.5.10(9)/L (n = 10) and thrombocytopenia (n = 6). Three patterns of lymphocyte surface markers were observed: CD3+CD4-8+ (14 patients), CD3+CD4-8+ (5 patients) and CD3+CD4+8- (1 patient). An abnormal karyotype was found in 2 patients. T-cell receptor gene was rearranged in all cases tested (9/9).
Pierre
P G
PG
Hôpital de Jolimont, La Louvière, Bruxelles.
Bosly
A
A
De Bock
R
R
Debusscher
L
L
Ferrant
A
A
Feremans
W
W
Lacor
P
P
Michaux
J L
JL
Van Camp
B
B
Van Hoof
A
A
D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D007621:Karyotyping; D008232:Lymphoproliferative Disorders / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D013601:T-Lymphocytes / Q000473:pathology*