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2968 Impact of Cutaneous Involvement on the Clinical Outcome of Adult T-Cell Leukemia/Lymphoma: 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 III
Hematology Disease Topics & Pathways:
Adult, Diseases, Therapies, Combinations, Elderly, Non-Hodgkin Lymphoma, T-Cell Lymphoma, Young Adult, Lymphoid Malignancies, Study Population, Clinically relevant
Monday, December 7, 2020, 7:00 AM-3:30 PM

Luis E Malpica Castillo, MD1, Denisse A. Castro, MD2,3, Camila Peña, MD4, Henry Idrobo, MD5,6*, Daniel J Enriquez, MD7, Lorena Fiad, MD8*, Maria Prates, MD9, Victoria Otero, MD10*, Mirna Churin, MD11*, Milagros Altamirano, MD12*, Kelly Meza, MD13*, Celia Moises, MD14*, Roberto Oviedo-Pecho, MD14*, Antonio Paredes, MD14*, Gadwyn Sanchez14*, Laura Aguirre-Martinez, MS15*, Bryan Valcarcel, MD16*, Cristaldo Nancy, MD17*, Juan L. Maradei, MD18*, Luciana Guanchiale, MD19*, Pablo Soto, MD20*, Jose Luis Viñuela, MD21*, Maria Elena Cabrera, MD22*, Ursula Aviles-Perez23*, Gustavo Sandival Ampuero, MD7, Sally Rose Paredes, MD3,24*, Eloisa Riva, MD25, Marcos Di Stefano, MD26*, Andrea Noboa, MD27*, Juan Antonio Choque, MD28*, Myrna Candelaria, MD, PharmD29, Alana Von Glasenapp, MD30*, Fabiola Valvert, MD31, Marialejandra Torres Viera, MD32*, Guilherme Fleury Perini, MD33*, Eduardo Sotomayor, MD34, Jorge J. Castillo, MD35, Juan Carlos Ramos, MD, BS36, Luis Villela Villela, MD, MC37* and Brady Beltran, MD38,39*

1Department of Lymphoma and Myeloma, Division of Cancer Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX
2Centro de Investigación de Medicina de Precisión, Universidad de San Martin de Porres, Lima, Peru
3Departamento de Oncología y Radioterapia, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru
4Hematology Department, Hospital Del Salvador, Santiago, Chile
5Facultad de Medicina., Universidad Del Valle, Cali, Colombia
6Hospital Universitario del Valle, Cali, Colombia
7Instituto Nacional de Enfermedades Neoplasicas, Lima, Peru
8Hematología, Hospital Italiano de La Plata, La Plata, Argentina
9Hematology, Hospital Italiano La Plata, La Plata, Argentina
10Sección Hematología, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina
11Grupo HTLV INBIRS (UBA-CONICET), Universidad de Buenos Aires, Buenos Aires, Argentina
12Medical Oncology Department, Hospital Nacional Guillermo Almenara Irigoyen, Lima, Peru
13Department of Pediatrics, Weill Cornell Medicine, New York, NY
14Servicio de Dermatologia, Departamento de Medicina, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru
15Facultad de Salud, Universidad del Valle, Cali, Colombia
16Milken Institute School of Public Health, The George Washington University, Washington
17Hospital Italiano de Buenos Aires (HIBA), Buenos Aires, Argentina
18Servicio de Hematologia, Hospital Municipal Emilio Ferreyra, Necochea, Buenos Aires, Argentina
19Hospital Privado Universitario de Córdoba, Cordoba, Argentina
20Hospital Dr. Eduardo Schütz Schroeder, Puerto Montt, Chile
21Servicio de Hematologia, Hospital Sótero de Rio, Santiago de Chile, Chile
22Hematology Department, Hospital del Salvador, Santiago, Chile
23Universidad Nacional Federico Villareal, Lima, Peru
24Centro de Investigacion de Medicina de Precision, Universidad de San Martin de Porres, Lima, Peru
25Cátedra de Hematología, Hospital de Clínicas, Facultad de Medicina, Montevideo, Uruguay
26Hospital Solón Espinoza Ayala (SOLCA, Quito)/ Universidad San Francisco de Quito (USFQ), Quito, Ecuador
27Servicio de Hematologia, Instituto Oncológico Nacional Dr. Juan Tanca Marengo, Guayaquil, Ecuador
28Servicio de Hematologia, Caja Nacional de Salud - Hospital de Especialidades Materno Infantil La Paz, La Paz, Bolivia (Plurinational State of)
29Research Division, Instituto Nacional de Cancerología, Mexico City, Mexico
30Department of Hematology, Instituto de Prevision Social, Asuncion, Paraguay
31Instituto De Cancerología y Hospital Dr. Bernardo Del Valle (INCAN), Ciudad de Guatemala, Guatemala
32Universidad Central de Venezuela, Caracas, Venezuela (Bolivarian Republic of)
33Hospital Israelita Albert Einstein, Sao Paulo, PA, Brazil
34George Washington University Cancer Center, Washington, DC
35Bing Center for Waldenström Macroglobulinemia, Dana Farber Cancer Institute, Boston, MA
36Sylvester Comprehensive Cancer Center, University of Miami Miller School of Medicine, Miami, FL
37Internal Medicine division/Hematology and blood bank service, Centro Medico Dr. Ignacio Chavez/ISSSTESON/HermosilloSonora, Hermosillo, Mexico
38Universidad de San Martin de Porres, Centro de Investigación de Medicina de Precisión, Lima, Peru
39Departamento de Oncologia Medica y Radioterapia, Hospital Nacional Edgardo Rebagliati Martins, Lima, LIMA, Peru

INTRODUCTION: Adult T-cell leukemia/lymphoma (ATLL) is an aggressive 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, Western Africa, the Caribbean basin and South America. Cutaneous signs of ATLL are varied and may consist of macules (M), plaques (P), multiple papules (MP), tumoral nodules (TN), erythroderma (E) or mixed-lesions (≥2 predominant lesions, ML). M and P forms are believed to carry a better prognosis. However, data on cutaneous presentation of ATLL remains scarce. Herein, we report cases of ATLL with cutaneous involvement diagnosed in 4 Latin American countries over the last 3 decades.

METHODS: We retrospectively analyzed patients (pts) diagnosed with ATLL between January 1995 and December 2019. ATLL subtypes were classified according to the Shimoyama criteria into acute, lymphomatous, chronic and smoldering. Primary cutaneous tumoral (PCT) variant was classified according to the 2019 International Revised ATLL Consensus. We designed 2 cohorts: the first, ATLL pts with cutaneous involvement, and the second, matched cases without cutaneous involvement. We determined the type of skin lesion as well as the survival associated with the various types of skin lesions. We compared the frequency of clinical features using Fisher’s exact test. 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. We analyzed survival data according to ATLL subtype, cutaneous involvement status, and type of skin lesion using the Kaplan-Meier method and Log rank test.

RESULTS: A total of 169 pts with ATLL were identified; 63 had cutaneous involvement and 106 did not. Clinical features are shown in Table 1. In both groups the median age was 57 years with a female predominance. Cutaneous involvement was most frequently found in acute (41%) and lymphomatous (37%) ATLL pts. The E (24%) and P (22 %) types were the most frequent skin lesions. Disease stage, presence of B symptoms, hypercalcemia, ECOG ≥2, elevated LDH, and IPI/ PIT score were not different among groups. Table 2 and Table 3 summarize the first-line therapy used and response rates. The use of first-line zidovudine plus interferon alpha (AZT-IFN), regardless of the type of skin lesion, resulted in relatively high response rates [overall response (OR) 100%, n=8; CR 62.5%] as compared to multi agent-chemotherapy (OR 33.3%, n=12). Overall, the presence of cutaneous involvement was associated with better overall survival (OS) compared to non-cutaneous involvement (aHR 0.55 [95% CI: 0.37-0.82], p<0.01; 1-year OS 53% vs. 27%, respectively, p=0.012) (Figure 1). PCT pts had better outcome compared to acute and lymphomatous ATLL forms (1-year OS 75% vs. 39% vs. 25%, respectively, p=0.002). The presence of P and MP skin lesions was associated with better OS compared to other subtypes (1-year OS: P/MP 65% vs. others 41%, respectively, p=0.027) (Figure 2, supplemental figure 1). In a multivariate analysis, hypercalcemia was an independent poor prognostic factor for survival among ATLL pts with cutaneous involvement (aHR 3.99 [95% CI: 139-11.45], p=0.01) (supplemental figure 2). One patient with lymphomatous ATLL and plaque lesions underwent allogeneic stem cell transplant with high-dose chemotherapy after achieving CR with AZT-IFN; patient remains alive and progression-free for 17 months. Illustrative cases of cutaneous ATLL are shown in Figure 3.

CONCLUSION: In Latin American pts with aggressive ATLL, cutaneous involvement appears to be associated with better survival compared to non-cutaneous involvement. PCT subtype, an ATLL variant characterized by isolated skin lesions with no organ involvement and poor outcome, appeared to have a better prognosis compared to acute and lymphomatous ATLL forms. P and MP skin lesions were both associated with better survival. Hypercalcemia was found as an independent prognostic factor for survival in pts with cutaneous involvement. Finally, AZT-IFN appears to be reasonable first-line option for aggressive ATLL subtypes with cutaneous involvement regardless of the type of skin lesion at diagnosis, based on the relatively high response rates observed in this subset; further investigation in randomized clinical trials is needed.

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: Takeda: Honoraria, Speakers Bureau; Tecnofarma: Honoraria, Speakers Bureau; Abbvie: Honoraria, Speakers Bureau; Janssen: Honoraria, Speakers Bureau; Amgen: 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; Kymera: Consultancy; Beigene: Consultancy, Research Funding; Abbvie: Research Funding; Janssen: Consultancy, Research Funding; Pharmacyclics: Consultancy, Research Funding. Ramos: NIH: Research Funding. Villela: amgen: Speakers Bureau; Roche: Other: advisory board, Speakers Bureau.

*signifies non-member of ASH