Characteristics of Adult T-Cell Leukemia/Lymphoma Patients with Long Survival: Prognostic Significance of Skin Lesions and Possible Beneficial Role of Valproic Acid.

Plumelle Yves,Panelatti Gérard,Banydeen Rishika,Delaunay Christine,Michel Stephane

doi:10.1155/2015/476805

Plumelle Yves, Panelatti Gérard + Show 3 more

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https://doi.org/10.1155/2015/476805

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Abstract

We describe the clinical and biological features of ten patients with a survival superior to ten years (long survival), out of 175 patients diagnosed with Adult T-cell Leukemia/Lymphoma (ATL) in Martinique (1983–2013). There were 5 lymphoma and 5 chronic subtypes. Five of them (3 chronic, 2 lymphoma) had been treated with valproic acid (VA) for neurological disorders developed before or after ATL diagnosis, suggesting a beneficial role for VA as a histone deacetylase inhibitor (HDI) in ATL treatment. Total duration of uninterrupted VA treatment ranged from 8 to 37 years. Overall, the 175 incident ATL cases presented with a median survival of 5.43 months. The five VA-treated (VA+) patients presented with longer survival compared to VA treatment-free patients (VA−). For chronic subtypes, survival periods were of 213 months for 3 VA+ patients and of 33 months for 11 VA− patients (p = 0.023). For lymphoma subtypes, survival periods were of 144 months for 2 VA+ patients versus 6 months for 49 VA− patients (p = 0.0046). ATL cases with skin lesions, particularly lymphoma subtypes, had a longer survival (13.96 months) compared to those without skin lesions (6.06 months, p = 0.002). Eight out of the 10 patients presenting with long survival had skin lesions.

Highlights

The human T-lymphotropic virus 1 (HTLV-1) is the causative agent of ATL (Adult T-cell Leukemia/Lymphoma) [1, 2] and of HAM/TSP (HTLV-1-associated myelopathy/tropical spastic paraparesis), a neurodegenerative disorder [3,4,5]
Median survival was 5.43 months for all cases: 3.06 months for those presenting with acute subtypes, 8.13 months for lymphoma subtypes, and 45.16 months for chronic subtypes (Figure 1)
Patients with skin lesions registered a significantly longer survival compared to patients without skin lesions (13.96 months versus 6.06 months, p = 0.002) (Figure 2)

Summary

Introduction

The human T-lymphotropic virus 1 (HTLV-1) is the causative agent of ATL (Adult T-cell Leukemia/Lymphoma) [1, 2] and of HAM/TSP (HTLV-1-associated myelopathy/tropical spastic paraparesis), a neurodegenerative disorder [3,4,5]. Based on the importance of lymphocytosis, tumor syndrome, hypercalcemia, and lactate dehydrogenase (LDH) values, the Shimoyama classification (Lymphoma Study Group (LSG) classification) recognizes four ATL subtypes: acute and lymphoma aggressive forms, chronic and smoldering indolent forms [6]. The ATL cell further has an intrinsic resistance to various chemotherapies. All these factors contribute to a very poor prognosis for the ATL disease, with a median survival of 6.2, 10.2, and 24.3 months for acute, lymphoma, and chronic subtypes, respectively [6]. Some of them had received long-term valproic acid (VA) treatment for neurological disorders having developed before or after ATL diagnosis, suggesting a beneficial role for VA, as a histone deacetylase inhibitor (HDI), in ATL clinical management

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