Acute Myeloblastic Leukemia M2 with the 8;21 Translocation Associated with Granulocytic Sarcoma

Abstract

A 13-year-old girl presented with 2 compact painless subcutaneous tumors of 3 cm on the scalp in June 1993. Peripheral blood count was Hb 95g/l, Er 3.2 time x 10¹²/leukocytes- 21 x 10⁹/1: blasts 74%, neutrophils 9%, lymphocytes 14%, monocytes 3%. After bone marrow puncture, AML FAB M2 was diagnosed. Cytogenetic analysis of bone marrow showed two clones: 1, 46 XX (20%) and clone 2, 46 XX t(8;21) (q22;q22) and t(l;17) (q21;pl3) (80%). CSF examination revealed 9 cells/ml WBC with blasts on the cytocentrifuged specimen. A tumor mass in the spinal canal (L2-L3) compressing the spinal cord was also revealed by CT scan. A complete remission was achieved after induction chemotherapy acccording to the BFMAML-87 protocol. The patient received the 6 week consolidation phase, but intensification of treatment with high-dose Ara-c and VP-16 was omitted due to the 10-fold increase in hepatic enzymes. Then the patient received maintenance therapy. Two months after completion of consolidation the patient was admitted with urine retention. Three separate large masses were palpable in the abdomen. Laparoscopy disclosed tumor masses in the uterus and both ovaries. Bone marrow examination revealed more than 50% of blasts. Relapse treatment consisted of: one course of Ara-C 1000mg/m² every 12 h for 4 days, VP-16 100mg/m² per day for 4 days and mitoxantrone 8 mg/m² per day for 3 days; one course of the same doses of Ara-C for 4 days plus amsacrine 100 mg/m² per day for 3 days and L-asparaginase 6000 IU/m² 6 h after the last dose of Ara-C. After the treatment the second remission was achieved in January 1994. Autologous BMT without ex vivo purging was performed in July 1994 after conditioning with melphalan 190 mg/m². After bone marrow reconstitution, immunotherapy with IL-2 (5 MU/m² s.c. for 5 days every 2 weeks) was begun and is being continued. The second remission has lasted for 12 months.