Adult T-Cell Leukemia

Abstract

Adult T-cell leukemia (ATL) is endemic in southwestern Japan, in the Caribbean islands, and in central Africa. Human T-cell lymphotropic virus type I (HTLV-I) is the etiologic agent of ATL. The clinical characteristics are (1) onset in adulthood, (2) subacute or chronic leukemia with rapidly progressive terminal course, (3) frequent skin lesions, (4) lymphadenopathy that characteristically spares the mediastinum, (5) hepatosplenomegaly, (6) hypercalcemia, and (7) a tendency toward geographical clustering. Although hypercalcemia and osteoclastic activity due to parathyroid hormone-related peptide are frequently reported histologically, radiographic abnormalities of bone are not common. Two major patterns of osteolytic lesions observed in ATL are "punched-out" lesions resembling multiple myeloma and osteolytic metastasis and subperiosteal bone resorptions similar to those in hyperparathyroidism.

HTLV-1-Associated Lymphoma Presented as Massive Lymphadenopathy

“…Chronic ATL presents with leukocytosis, lymphadenopathy, hepatomegaly, splenomegaly, skin, and pulmonary lesions with the absence of elevated LDH, hypercalcemia, and other visceral involvement. 5,9,13,14 Both of our cases presented with elevated LDH higher than 2 times the normal upper limit, lymphadenopathy, but no evidence of leukocytosis and no abnormal lymphocyte in peripheral blood. According to the Shimoyama classification, both cases were most likely diagnosed with ATL lymphoma type.…”

“…[16][17][18] Regrettably, the lymphoma relapsed with massive lymphadenopathy in the mediastinum that caused SVC syndrome. On reviewing the lymphadenopathy in HTLV-1-associated lymphoma, we found the lymphadenopathy was usually characterized as spares the mediastinum, 9,13,14 except one case report in 1984 that described massive mediastinal adenopathy. 19 Meanwhile, we found massive lymphadenopathy as a presentation of HTLV-1-associated lymphoma, has was not systemically reported in the typical ATL studies.…”

“…Chronic ATL presents with leukocytosis, lymphadenopathy, hepatomegaly, splenomegaly, skin, and pulmonary lesions with the absence of elevated LDH, hypercalcemia, and other visceral involvement. 5 , 9 , 13 , 14 …”