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A 28-year-old male patient presented with multiple lymphadenopathies and extranodal masses. He was diagnosed with stage IVB ALK-positive anaplastic large cell lymphoma after the right axillary lymph node biopsy. A partial metabolic response with fluorodeoxyglucose accumulation was observed in the residual disease of the upper left hilar lymph node after eight courses of brentuximab vedotin, cyclophosphamide, adriamycin, and prednisolone. We started alectinib at 600 mg daily, which achieved a complete metabolic response (CMR) after three months. The CMR was maintained and alectinib was continuously administered without adverse events at the last follow up. Alectinib showed high efficacy and tolerability, though the optimal period and long-term adverse effects of administration remain unclear. Therefore, further studies are necessary.

A 59-year-old woman with anaplastic large cell lymphoma (ALCL), ALK-negative, was treated with brentuximab vedotin (BV) against relapse after 6 regimens of systemic chemotherapy and radiation. Despite achieving an initial response, skin lesions worsened after 11 courses. A skin biopsy after the development of resistance to BV confirmed loss of CD30 expression by the tumor cells, suggesting a possible cause of resistance. This case shows that down-regulation of CD30 does occur during BV treatment, resulting in resistance to this drug. Because of this possibility, in the future, expression of CD30 should be carefully monitored with extended use of BV against ALCL.

The randomized international trial for childhood anaplastic large cell lymphoma, (ALCL99-R1) involving European study groups and a Japanese group, compared six courses of methotrexate 1 g/m(2) over 24 hours with an intrathecal injection (IT) (MTX1 arm) with six courses of methotrexate 3 g/m(2) over 3 hours without IT (MTX3 arm). In this report, data from the Japanese portion of the trial are compared with the results of the international study. Overall, 352 patients were recruited for the international study, and 44 of these patients were from Japan. Median follow-up times were 3.8 and 3.5 years, respectively, in the international and Japanese studies. The two-year event-free and 2-year overall survival rates of the international study were 74% and 93%. The corresponding figures for those registered in Japan were 81% and 96%, respectively. Clinical characteristics and outcomes of patients were similar in the two groups. Incidences of grade 4 hematologic toxicity, infection, and grade 3 to 4 stomatitis, which were reported to be statistically significantly higher after the MTX1 arm in the international study, were also statistically significantly higher after the MTX1 arm for those registered in Japan. Results of ALCL99-R1 treatment in Japan were essentially the same as in the international study. The international study is anticipated to contribute to establishing an optimal treatment for ALCL, a rare childhood lymphoma.

A 35-year-old man was admitted with continuous general fatigue and low grade fever. He was HIV-positive, and had gastric diffuse large B-cell lymphoma and renal T-cell anaplastic large cell lymphoma (T-ALCL). We diagnosed double lymphomas related to AIDS. The patient received anti-retroviral therapy, and started the CHOP regimen for the double lymphomas, resulting in transient improvement. However, fever again appeared during HAART and CHOP treatment, and a right inguinal subcutaneous lesion appeared. Biopsy specimen demonstrated null cell ALCL, and this patient demonstrated multiple lymphomas. This case suggested that cancer generation was promoted by low immunity, although it is known that ambivalent tumors such as non-Hodgkin lymphomas can occur frequently.

A 58-year-old woman complicated with rheumatoid arthritis (RA) was admitted to our hospital with right axillar lymphadenopathy and splenomegaly in November 1992. She was diagnosed as an anaplastic large-cell lymphoma (Ki-1 +) (stage IIIB) on the histological findings of the right axillar lymph nodes. She was treated with 11 courses of CHOP regimen between February 1992 and May 1993, and with mitoxantrone, etoposide (VP-16) and predonisolone in April 1992 and May 1993. The right axillar lymph nodes and spleen were irradiated at a dose of 36Gy in October 1992 and May 1993 respectively. In May 1993, peripheral blood showed WBC 89,000/microliter with 96% myeloblasts, Hb 8.3 g/dl, and Plt 124,000/microliter. Bone marrow aspirate revealed hypercellularity with 90% myeloblasts, which were positive for CD13 and HLA-DR. She was diagnosed as AML (M1). The karyotype showed normal. Southern blot analysis did not reveal the rearrangement of the MLL gene. She received the BHAC-DMP regimen and obtained complete remission. However, she relapsed during consolidation therapy, and died of cerebral bleeding. An autopsy revealed absence of a residual tumor. The mean interval from exposure to alkylating agent to the onset of secondary leukemia has been reported to be about 5 years, in contrast to a shortened interval of about 2 years for VP-16-induced leukemia. In our patient, it took only 1 year to have AML following chemotherapy for Ki-1 lymphoma. This suggests that her AML might be induced not only by treatments for RA and Ki-1 lymphoma, but also by immunological background such as RA.