[Lymphoproliferative syndrome with granular lymphocytes of CD8+ phenotype: a clonal pathology with a chronic course].
The syndrome of CD8 hyperlymphocytosis with neutropenia is a heterogeneous disorder ranging from reactive benign state to neoplastic pathology. The prognosis for LGL (Large Granular Lymphocyte) leukemia depends likely on its phenotype:-NK phenotype, extremely poor prognosis and rapidly fatal-T phenotype (CD8+), chronic disease with slow progression. Here, we report four cases of CD8+ hyperlymphocytosis with neutropenia, which are CD2+/-, CD3+, CD4-, CD8+, CD16-, CD56+/-, CD57+ phenotype. These lymphocytic proliferations were associated with clonal rearrangement of T-cell receptor b gene. In two cases, characteristic blood hyperlymphocytosis appeared only after splenectomy, but retrospective bone marrow analysis showed that the CD8+, CD57+ lymphocyte proliferation previously existed. These lymphocytes had a low natural killer activity against K562 cell line. HTLV1 proviral sequence was not integrated in leukemic cell DNA. This monoclonal pathology has a chronic clinical course, with a thirteen year evolution in one case. Splenectomy did not correct neutropenia but allowed the control of hemolytic anemia and auto-immune thrombocytopenia in one case.
Pellegrin
J L
JL
Clinique de Médecine Interne et Maladies Infectieuses, Hôpital Haut-Lévêque, CHU Bordeaux, Pessac.
Merlio
J P
JP
Cony-Makhoul
P
P
Ferrer
A M
AM
Astier-Gin
T
T
De Mascarel
A
A
Beylot
C
C
Broustet
A
A
Reiffers
J
J
Leng
B
B
D000328:Adult; D015139:Blotting, Southern; D016827:CD8 Antigens / Q000276:immunology*; D002908:Chronic Disease; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000209:etiology; D007945:Leukemia, Lymphoid / Q000150:complications* / Q000276:immunology / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009503:Neutropenia / Q000209:etiology; D013156:Splenectomy; D013163:Splenomegaly / Q000209:etiology / Q000601:surgery; D013997:Time Factors
D016827:CD8 Antigens
Syndrome lymphoprolifératif à lymphocytes granuleux de phénotype CD8+: une pathologie clonale d'évolution chronique.