International Journal of Hematology

DOI: 10.1007/s12185-017-2338-6 Pages: 1-7

Prognostic impact of minimal disseminated disease and immune response to NPM-ALK in Japanese children with ALK-positive anaplastic large cell lymphoma

1. National Hospital Organization Nagoya Medical Center, Department of Clinical Trials and Research, Clinical Research Center

2. St. Marianna University School of Medicine, Department of Pediatrics

3. Saitama Children’s Medical Center, Department of Clinical Research

4. National Hospital Organization Kyushu Cancer Center, Department of Pediatrics

5. National Center for Child Health and Development

6. Aichi Medical University

7. Sapporo Hokuyu Hospital, Department of Hematology/Oncology for Children and Adolescents

Correspondence to:
Yuka Iijima-Yamashita
Tel: +81 52 951 1111
Email: yuka.iijima@nnh.go.jp

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Abstract

The prognostic impact of minimal disseminated disease (MDD) and anti-anaplastic lymphoma kinase (ALK) antibody titer in children with ALK-positive anaplastic large cell lymphoma (ALCL) was reported by an Italian/German group. Here, we examine their prognostic value in Japanese children with ALK-positive ALCL. We evaluated nucleophosmin (NPM)-ALK transcripts in 60 patients at diagnosis by RT-PCR and real-time PCR (qPCR). The antibody titer was assessed in 35 patients. Fifty-two percent were MDD positive by RT-PCR and 37% had more than 10 copies of NPM-ALK per 104 copies of ABL (10NCNs) by qPCR. Fifty-one percent of 35 patients had high antibody titer (> 1/750). Progression-free survival (PFS) of the patients with > 10 NCNs or low antibody titers was significantly poorer than that of patients with ≤ 10 NCNs or high antibody titers (> 1/750) (P = 0.016, 0.029), respectively, although we observed no difference in PFS associated with positive MDD on RT-PCR. On stratification using a combination of MDD and antibody titer, PFS for patients with > 10 NCNs and low antibody titer was extremely low (30.0%). Combined evaluation of MDD and anti-ALK antibody titer at diagnosis may thus be valuable for stratification of treatment for childhood ALCL.

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