Hand Osteolysis in Patients with Adult T-Cell Leukemia-Lymphoma: Radiographic Characteristics

Abstract

Adult T-cell leukemia-lymphoma (ATLL) is caused by human T-cell lymphotrophic virus I (HTLV-I) infection. Among ATLL cases, 70% of patients present with leukemia and the remaining patients present with lymphoma. Hand osteolysis in the patients with ATLL is considered as paraneoplastic syndrome and caused by parathyroid hormone-related peptide (PTHrP) released from tumor cells. Radiographic features are similar to hyperparathyroidism, but the distribution of osteolysis in hands appears to be slightly different with the authors' experiences. The objective of this study was to identify radiographic characteristics of hand osteolysis associated with ATLL. We included six ATLL patients (5 men and 1 woman; age range, 45-71 years). All the patients presented with acute leukemia, and three were associated with hypercalcemia and pain in various locations including hands. Patterns of osteolysis on hand radiographs were evaluated and recorded independently by three musculoskeletal radiologists. We analyzed the distribution of the bone resorption in the ray distribution of the hand, finger predilection, and the difference between the ulnar and radial sides. The bone resorption was characterized by frequent involvement of the distal and proximal phalanges, predilection of ring fingers and prominent involvement on the ulnar side, compared with frequent involvement of proximal and middle phalanges, index and middle fingers, and on the radial side in the bone resorption of typical hyperparathyroidism. Such distribution may be a characteristic feature of hand osteolysis in patients with ATLL. The present findings are helpful for physicians to differentiate PTHrP-mediated osteolysis in ATLL from parathyroid hormone-mediated hyperparathyroidism.

Mouse model recapitulates the phenotypic heterogeneity of human adult T-cell leukemia/lymphoma in bone

“…While paraneoplastic bone loss is common in ATL patients, primary ATL bone tumors resulting in local solitary or multiple lytic lesions also have been described [14], [27], [46], [47], [48], [49]. While the role of the endocrine factors that mediate HHM and widespread bone resorption in ATL have been investigated, the description of the direct interaction between ATL and the cells of the bone microenvironment has largely been limited to case reports.…”

“…While the role of the endocrine factors that mediate HHM and widespread bone resorption in ATL have been investigated, the description of the direct interaction between ATL and the cells of the bone microenvironment has largely been limited to case reports. Bone lesions are observed in 5.5% of ATL patients, mainly in the tibia, ulna, scapula, femur, clavicle, and peripheral extremities [14], [49], [50]. Regardless of the presence or absence of tumor cells, bone biopsies of ATL patients consistently demonstrate a marked increase in number and activation of osteoclasts lining the bone trabeculae and cortices, resulting in either local or systemic bone loss [1].…”

“…The enhanced pathologic bone resorption in ATL patients is primarily controlled through humoral or endocrine-mediated mechanisms. However, several case reports have identified primary ATL bone tumors that also express osteoclast stimulatory factors, indicating a paracrine role for ATL factors in bone as well [14], [15], [16]. Few studies have focused on the mechanisms by which humoral or paracrine tumor-growth factors and cytokines from ATL cells induce osteolysis and promote tumor progression.…”