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1165 Epidemiology, Clinical Features, and Outcome of HTLV-1-Related Adult T-Cell Leukemia/Lymphoma in Latin America: A Study from the Latin American Group of Lymphoproliferative Disorders (GELL)

Program: Oral and Poster Abstracts
Session: 624. Hodgkin Lymphoma and T/NK Cell Lymphoma—Clinical Studies: Poster I
Hematology Disease Topics & Pathways:
Adult, Diseases, Elderly, Non-Hodgkin Lymphoma, T-Cell Lymphoma, Young Adult, Lymphoid Malignancies, Study Population, Clinically relevant
Saturday, December 5, 2020, 7:00 AM-3:30 PM

Luis E Malpica Castillo, MD1, Daniel J Enriquez, MD2, Denisse A. Castro, MD3,4, Camila Peña, MD5, Henry Idrobo, MD6*, Lorena Fiad, MD7*, Maria Prates, MD8, Victoria Otero, MD9*, Mirna Churin, MD10*, Milagros Altamirano, MD11*, Gustavo Sandival Ampuero, MD2, Ursula Aviles-Perez12*, Kelly Meza, MD13*, Laura Aguirre-Martinez, MS14*, Cristaldo Nancy, MD15*, Juan L. Maradei, MD16*, Luciana Guanchiale, MD17*, Pablo Soto, MD18*, Jose Luis Viñuela, MD19*, Maria Elena Cabrera, MD20*, Sally Rose Paredes, MD3,21*, Eloisa Riva, MD22, Marcos Di Stefano, MD23*, Andrea Noboa, MD24*, Juan Antonio Choque, MD25*, Myrna Candelaria, MD, PharmD26, Alana Von Glasenapp, MD27*, Fabiola Valvert, MD28, Marialejandra Torres Viera, MD29*, Guilherme Fleury Perini, MD30*, Eduardo Sotomayor, MD31, Jorge J. Castillo, MD32, Juan Carlos Ramos, MD, BS33, Luis Villela Villela, MD, MC34* and Brady E. Beltrán, MD3,4*

1Department of Lymphoma and Myeloma, Division of Cancer Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX
2Instituto Nacional de Enfermedades Neoplasicas, Lima, Peru
3Departamento de Oncología y Radioterapia, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru
4Centro de Investigación de Medicina de Precisión, Universidad de San Martin de Porres, Lima, Peru
5Hematology Department, Hospital Del Salvador, Santiago, Chile
6Hospital Universitario del Valle, Cali, Colombia
7Hematología, Hospital Italiano de La Plata, La Plata, Argentina
8Hematology, Hospital Italiano La Plata, La Plata, Argentina
9Sección Hematología, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina
10Grupo HTLV INBIRS (UBA-CONICET), Universidad de Buenos Aires, Buenos Aires, Argentina
11Medical Oncology Department, Hospital Nacional Guillermo Almenara Irigoyen, Lima, Peru
12Universidad Nacional Federico Villareal, Lima, Peru
13Department of Pediatrics, Weill Cornell Medicine, New York, NY
14Facultad de Salud, Universidad del Valle, Cali, Colombia
15Hospital Italiano de Buenos Aires (HIBA), Buenos Aires, Argentina
16Servicio de Hematologia, Hospital Municipal Emilio Ferreyra, Necochea, Buenos Aires, Argentina
17Hospital Privado Universitario de Córdoba, Cordoba, Argentina
18Hospital Dr. Eduardo Schütz Schroeder, Puerto Montt, Chile
19Servicio de Hematologia, Hospital Sótero de Rio, Santiago de Chile, Chile
20Hematology Department, Hospital del Salvador, Santiago, Chile
21Centro de Investigacion de Medicina de Precision, Universidad de San Martin de Porres, Lima, Peru
22Cátedra de Hematología, Hospital de Clínicas, Facultad de Medicina, Montevideo, Uruguay
23Hospital Solón Espinoza Ayala (SOLCA, Quito)/ Universidad San Francisco de Quito (USFQ), Quito, Ecuador
24Servicio de Hematologia, Instituto Oncológico Nacional Dr. Juan Tanca Marengo, Guayaquil, Ecuador
25Servicio de Hematologia, Caja Nacional de Salud - Hospital de Especialidades Materno Infantil La Paz, La Paz, Bolivia (Plurinational State of)
26Research Division, Instituto Nacional de Cancerología, Mexico City, Mexico
27Department of Hematology, Instituto de Prevision Social, Asuncion, Paraguay
28Instituto De Cancerología y Hospital Dr. Bernardo Del Valle (INCAN), Ciudad de Guatemala, Guatemala
29Universidad Central de Venezuela, Caracas, Venezuela (Bolivarian Republic of)
30Hospital Israelita Albert Einstein, Sao Paulo, PA, Brazil
31George Washington University Cancer Center, Washington, DC
32Bing Center for Waldenström Macroglobulinemia, Dana Farber Cancer Institute, Boston, MA
33Sylvester Comprehensive Cancer Center, University of Miami Miller School of Medicine, Miami, FL
34Internal Medicine division/Hematology and blood bank service, Centro Medico Dr. Ignacio Chavez/ISSSTESON/HermosilloSonora, Hermosillo, Mexico

INTRODUCTION: Adult T-cell leukemia/lymphoma (ATLL) is a mature peripheral T-cell neoplasm caused by the Human T-cell Leukemia Virus Type 1 (HTLV-1). HTLV-1 infects up to 10 million people worldwide and is most endemic in Southwestern Japan, the Caribbean basin, South America, and Western Africa. In Latin America (LA), Peru and Brazil have the highest prevalence of HTLV-1-related diseases, however, data on ATLL in other LA countries is scarce. ATLL carries a dismal prognosis and is essentially incurable by conventional drugs. We describe the epidemiology, clinical features, treatment, and disease outcome of ATLL encountered in 11 countries in LA.

METHODS: We retrospectively analyzed patients (pts) diagnosed with ATLL between January 1995 and December 2019. ATLL cases were classified according to the Shimoyama criteria into acute (A), lymphomatous (L), chronic (C) and smoldering (S). Treatment approaches used as first-line therapy were: 1) chemotherapy alone; 2) combined chemotherapy with zidovudine/interferon-alpha (AZT-IFN); and 3) AZT-IFN alone, as previously done with Miami cohort (Malpica and Ramos et al. Blood Advances 2018). Treatment response was assessed according to Tsukasaki et al. (JCO 2009) criteria. To be classified as complete response (CR), partial response and stable disease, these had to persist for a period of at least 4 weeks. Survival curves were estimated using the Kaplan-Meier method and Log rank test.

RESULTS: A total of 253 pts with ATLL were identified. Two hundred twenty six pts (L=122, A=73, C=26, S=5) had sufficient data for analysis. Demographic and clinical features are shown in Figure 1 and Table 1. Median age at diagnosis was 57 years, with a female predominance in A (58%) and S (100%) types. Most ATLL pts were from Peru (n=159, 63%) followed by Chile (n=44, 17%), Argentina (n=20, 8%) and Colombia (n=17, 7%). B symptoms were high present in A, L and C types (73%, 72%, 58% vs. 8% S type, respectively, p=0.011). Hypercalcemia was highly associated with A type (57% vs. L 27%, p=0.014). The PIT score yielded to a more aggressive risk classification compared to the IPI score (high-risk: 55% vs. 29%, respectively, p<0.001). Strongyloidiasis (n=5) and pneumocystis jirovecii pneumonia (n=5) were the most commonly observed co-infections at diagnosis. Commonly affected extranodal sites other than bone marrow in all subtypes were skin 25% (n=63) and liver 9% (n=24). The therapy approach used during the first 2 therapy evaluations are summarized in Table 2. The median survival (MS) times were 4.3 months, 7.9 months, 21.1 months, and not reached for A, L, C and S form, with 4-year survival of 8%, 22%, 40% and 80%, respectively (Figure 2). First-line AZT-IFN resulted in overall response (OR) rate of 63% (CR 24%) for A (n=8) and 75% (CR 50%) for L (n=8), respectively (Table 3). The OR rates after first-line multi-agent chemotherapy alone for A vs. L were 21% (CR 8%) and 41% (CR 29%), respectively (Table 3). The most commonly used regimens were CHOP/CHOP-like (n=117, 59%) and CHOEP (n=40, 20%) regimens with OR rates of 29% (CR 12%) and 60% (CR 42%), respectively (Table 3). Progression-free survival (PFS) rates in pts with aggressive ATLL who achieved CR after chemotherapy vs. AZT-IFN (alone or in combination with chemotherapy) were 2.8 months vs. 30.4 months for A (n=8) type and 67.1 months vs. 17.7 months for L (n=30) type, respectively (Figure 3). Only 2 pts with L type underwent allogeneic hematopoietic stem cell transplant (allo-HSCT) with PFS of 12 and 17 months (Table 4).

CONCLUSION: ATLL continues to carry a dismal outcome with conventional therapies thus urging the development of novel approaches. Our study found that Latin American ATLL variant presents at a younger age, has a female predominance, high incidence of L type, low incidence of indolent types and lower survival rates, suggesting that Latin American ATLL variant presents earlier and more aggressively than in Japanese pts. AZT-IFN produced durable responses in A type patients who achieved CR as compared to chemotherapy alone. Chemotherapy responses were more durable in L types who achieved CR as compared to A type. In conclusion, in the management of aggressive ATLL, chemotherapy remains the preferred choice for L type (with consideration of allo-HSCT upfront), while AZT-IFN is a good option to attempt for A type upfront.

Disclosures: Peña: Janssen: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Sandoz: Membership on an entity's Board of Directors or advisory committees; Roche: Membership on an entity's Board of Directors or advisory committees; Abbvie: Membership on an entity's Board of Directors or advisory committees; Amgen: Speakers Bureau; BindingSite: Research Funding. Idrobo: Amgen: Honoraria, Speakers Bureau; Janssen: Honoraria, Speakers Bureau; Tecnofarma: Honoraria, Speakers Bureau; Takeda: Honoraria, Speakers Bureau; Abbvie: Honoraria, Speakers Bureau. Altamirano: Hospital Nacional Guillermo Almenara Irigoyen: Other: Servicio de Hematologia. Perini: Abbvie: Speakers Bureau; Janssen: Honoraria, Speakers Bureau; Takeda: Honoraria. Castillo: TG Therapeutics: Research Funding; Beigene: Consultancy, Research Funding; Abbvie: Research Funding; Janssen: Consultancy, Research Funding; Pharmacyclics: Consultancy, Research Funding; Kymera: Consultancy. Ramos: NIH: Research Funding. Villela: Roche: Other: advisory board, Speakers Bureau; amgen: Speakers Bureau.

*signifies non-member of ASH