ALL-004 Philadelphia Chromosome-Positive T-Cell Acute Lymphoblastic Leukemia/Lymphoblastic Lymphoma: A Rare Case Report

Abstract

T-acute lymphoblastic leukemia/lymphoblastic lymphoma (T-ALL/LBL) is a rare and aggressive leukemia. Philadelphia chromosome-positive (Ph+) T-ALL is exceedingly rare and has therapeutic and prognostic significance. The case study herein is a very rare presentation. A case report of a 52-year-old Egyptian female, who presented in 2012 with a 2-month history of fatigue, bone aches, and abnormal vaginal bleeding. Oncology Center Mansoura University. The patient appeared pale with significant splenomegaly. Complete blood count: white blood cell count 6.9 k/µL, microcytic hypochromic anemia with hemoglobin 10.4 g/dL, and platelet count 149 k/µL. A peripheral blood smear showed neutrophil toxic granulation. Bone marrow aspirate (BMA) was infiltrated with 45% blasts, and myeloid and erythroid series were suppressed. No significant dysplasia was noted. Flow cytometry analysis performed on BMA showed an abnormal population of blasts expressing TdT (20%), cytoplasmic CD3 (35%), CD7 (64%), CD2 (62%), CD5 (61%), CD1a (0.38%), CD4 (27%), CD8 (32%), CD10 (3.6%), CD117 (5.4%), CD 34 (2.41%), and CD79a (27%), revealing a case of ALL (T-cell lymphoblastic lymphoma/leukemia). The fluorescence in-situ hybridization (FISH) analysis performed on interphase nuclei obtained from a BMA sample revealed a dual-fusion hybridization signal pattern for BCR and ABL1 probes on chromosomes 9 and 22, indicating t(9:22) in 100%. Cytogenetic analysis revealed an abnormal complex karyotype, 46,XY,t(9;22),(q34;q11.2)[25], in all counted cells. BCR-ABL1 gene rearrangement by quantitative real-time PCR was 100% IS. The patient was diagnosed with a case of T-ALL/LBL with t(9;22);BCR-ABL1 (Ph+). The patient started the MRCUK protocol in combination with imatinib 400 mg/d. She finished maintenance therapy on 10/12/2016. The patient is still on imatinib 400 mg/d. The patient is still in complete remission and in deep molecular response with no detectable minimal residual disease. Ph+ T-ALL is rare. Differentiation between Ph+ T-ALL/LBL and T-LB crisis of chronic myeloid leukemia (CML) was difficult because it is diagnosed with no prior history of CML. The addition of tyrosine kinase inhibitors to intensive chemotherapy has greatly improved the outcomes of Ph+ ALL patients.