Granular lymphocyte proliferative disorders: a multicenter study of 20 cases.
A series of 20 patients with granular lymphocyte proliferative disorders (GLPD) is reported. The criterion of inclusion was presence of persistent (> or = 6 months) granular lymphocytosis in the absence of any causative illness. Diagnoses made upon analytical control in half the patients of splenomegaly (25%) and hepatomegaly (25%) were infrequent. Clinical course was nonprogressive in 17/20 patients, but two developed high-grade NHL several years later and one showed progressive disease. Actuarial probability of survival at 5 years was 85%. Granular lymphocyte morphology was relatively homogeneous, and peripheral blood counts were preserved in the most patients. Bone marrow lymphocytic infiltration was low, as assessed by bone marrow aspiration and/or biopsy. Eosinophilia was an outstanding feature in eight cases. Ultrastructurally, all cases showed parallel tubular arrays; cytoplasmic granules and numerous short microvilli were noticed. The lymphoid phenotype was heterogeneous, the most common being CD2+CD3+CD4-CD8+, but six patients (30%) were CD4+ with variable expression of natural killer-associated antigens. Chromosomal analysis was abnormal in 4/10 patients [trisomy 19, t(5;6); inv(14) and inv(10)]. The study of beta-chain of the T-cell receptor revealed clonal rearrangements in 14 (78%), restricted to CD3+ patients (92%). In vitro culture of myeloid precursors showed decreased CFU-GM in 5/6 patients. Virological studies for HTLV-I and II were negative. In conclusion, the presence of a clonal proliferation was not correlated with the clinical course or an associated disease.
Woessner
S
S
Catalonian Group of Hematological Cytology, Hospital La Aliança, Barcelona, Spain.
Feliu
E
E
Villamor
N
N
Zarco
M A
MA
Domingo
A
A
Millá
F
F
Florensa
L
L
Rozman
M
M
Abella
E
E
Soler
J
J
D000368:Aged; D000369:Aged, 80 and over; D003114:Colony-Forming Units Assay; D015480:Deltaretrovirus Antibodies / Q000097:blood; D005260:Female; D005838:Genotype; D006412:Hematopoietic Stem Cells; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000276:immunology*; D008232:Lymphoproliferative Disorders / Q000097:blood* / Q000235:genetics / Q000276:immunology; D008297:Male; D008875:Middle Aged
D015480:Deltaretrovirus Antibodies