[Adult T-cell leukemia/lymphoma. Clinical aspects].
BACKGROUND
Adult T-cell leukemia/lymphoma (ATL) is a malignant proliferation of activated CD4+ T lymphocytes. The disease is almost exclusively found in patients living in retrovirus HTLV-1 endemic areas.
In ATL, monoclonal HTLV-1 provirus is integrated into atypical lymphocytes, called clover-leaf lymphocytes. The pathogenic mechanism leading to HTLV-1-induced leukemogenesis remains obscure. The disease generally occurs after a long latency period.
The diversity of the clinical presentation has led to the classification of ATL into four subtypes: acute or prototype, lymphoma, chronic, and painless. In the acute form of ATL there is a tumor syndrome associated with paraneoplastic hypercalcemia and a high rate of opportunistic infections due to the immunodepression predominated by cellular immunity.


METHODS
Prognosis is poor for the acute and lymphomatous forms with a median survival of 6 and 10 months respectively. Infectious episodes are frequent, often caused by Pneumocystis carinii, and require systematic prophylaxis. Screening for anguilulosis and prophylaxis is also necessary.
Besson
C
C
Service d'Hématologie clinique, Hôpital Necker, 149, rue de Sèvres, F75743 Paris.
Plumelle
Y
Y
Arnulf
B
B
Gonin
C
C
Panelatti
G
G
Bazarbachi
A
A
Hermine
O
O
D000328:Adult; D018450:Disease Progression; D006801:Humans; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000145:classification / Q000473:pathology* / Q000821:virology; D010146:Pain / Q000209:etiology; D010257:Paraneoplastic Syndromes / Q000209:etiology; D011379:Prognosis; D016019:Survival Analysis