Adult T-cell leukemia/lymphoma

Adult T-cell leukemia/lymphoma
Human T-cell(normal)
Specialty	Oncology, hematology

Adult T-cell leukemia/lymphoma (ATL or ATLL) is a rare

Globally, the retrovirus HTLV-1 is estimated to infect 20 million people per year with the incidence of ATL approximately 0.05 per 100,000 per year with endemic regions such as regions of Japan, as high as 27 per 100,000 per year.[6] However, cases have increased in non-endemic regions with highest incidence of HTLV-1 in southern/northern islands of Japan, Caribbean, Central and South America, intertropical Africa, Romania, northern Iran. ATL normally occurs around the age of 62 years but median age at diagnosis does depend on prevalence of the HTLV-1 infection in the geographic location.^[7]

Current treatment regiments for ATL are based on clinical subtype and response to initial therapy. Some therapy modalities for treatment may not available in all countries therefore strategies differ across the world. All patients are referred to clinical trials if available. Beyond clinical trials, treatments are centered on multiagent chemotherapy, zidovudine plus interferon a (AZT/IFN), and allogenic hematopoietic stem cell transplantation (alloHSCT).^[6]

Signs and symptoms

ATL is usually a highly aggressive

Diagnosis is made based on the combination of clinical features, characteristic morphologic and immunophenotypic changes of malignant cells. As clinical features and prognosis can be diverse, the disease is subtype-classified into four categories according to the Shimoyama classification: acute, lymphoma, chronic, smoldering.[13] Normally, identification of at least 5 percent of tumor cells in peripheral blood and confirmation of human T-lymphotropic virus type-1 are sufficient for diagnosis of acute, chronic, and smoldering types. For the lymphoma type, histopathologic examination by biopsy of lymph nodes may be needed.^[14]

Treatment

Treatment options that have been tried include zidovudine and the CHOP regimen.^[11] Pralatrexate has also been investigated.^[15] Recently, it has been reported that the traditional glucocorticoid-based chemotherapy toward ATL are largely mediated by thioredoxin binding protein-2 (TBP-2/TXNIP/VDUP1), suggesting the potential use of a TBP-2 inducer as a novel therapeutic target.^[16][17]

In 2021, mogamulizumab was approved for relapsed/refractor treatment of ATL in Japan.^[18]

At a medical conference in December 2013, researchers reported anywhere from 21 to 50% of ATL patients have disease expressing CD30.^[19] Although not FDA approved, treatment with CD30-targeting brentuximab vedotin in CD 30+ cases may be beneficial and supported by current NCCN guidelines.^[20]

Epidemiology

HTLV-1 infection in the

Novel approaches to the treatment of PTCL in the relapsed or refractory setting are under investigation. Pralatrexate is one compound currently under investigations for the treatment of PTCL.^[15]

References