title: Double Philadelphia chromosome-positive B acute lymphoblastic leukaemia in an elderly patient.
abstract: A rare case of double Philadelphia chromosome-positive B Acute lymphoblastic Leukaemia (B-ALL) is reported here. A 60-year-old lady presented with one month history of fever, submandibular lymphadenopathy, loss of appetite and weight loss. Physical examination revealed multiple palpable cervical lymph nodes. Blood film showed leucocytosis with 72% blasts. Bone marrow assessment confirmed a diagnosis of B-ALL with presence of double Philadelphia (Ph) chromosomes. As she was very ill, she was initially treated with an attenuated regimen of induction chemotherapy consisting of rituximab, cyclophosphamide, vincristine and prednisolone (R-CVP) along with intrathecal chemotherapy comprising methotrexate, cytarabine and hydrocortisone. Bone marrow examination post-induction chemotherapy showed >5% blasts. She was subsequently re-induced with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone (R-CHOP) along with intrathecal chemotherapy, following which she went into complete remission. Consolidation chemotherapy consisting of methotrexate, methylprednisolone, cytarabine, intrathecal chemotherapy and imatinib was subsequently administered followed by maintenance chemotherapy consisting of vincristine, prednisolone and imatinib (IDEAMOP). She developed spontaneous bruises and relapsed four months into her maintenance chemotherapy with 90% blasts in the bone marrow which was treated with fludarabine, cytarabine and granulocyte colony stimulating factor (FLAG). Unfortunately she developed neutropenic sepsis which was complicated by invasive lung aspergillosis. Bone marrow examination post-FLAG showed 80% blasts. Despite aggressive antifungal therapy, her lung infection worsened and she finally succumbed to her illness 13 months after the initial diagnosis. We highlight a rare case of elderly B-ALL with double Ph chromosomes which carries a poor prognosis despite aggressive treatment for the disease and its complications.
authors.affiliation: Universiti Kebangsaan Malaysia Medical Centre, Faculty of Medicine, Department of Pathology, Jalan Yaacob Latif, Bandar Tun Razak 56000 Cheras, Kuala Lumpur, Malaysia. tangyl@ppukm.ukm.edu.my.
authors.forename: Yee-Loong
authors.initials: YL
authors.lastname: Tang
authors.forename: Raja Zahratul Azma
authors.initials: RZ
authors.lastname: Raja Sabudin
authors.forename: Chooi-Fun
authors.initials: CF
authors.lastname: Leong
authors.forename: Clarence Ching-Huat
authors.initials: CC
authors.lastname: Ko
authors.forename: Wai-Kit
authors.initials: WK
authors.lastname: Chia
authors.forename: Shuib
authors.initials: S
authors.lastname: Salwati
authors.forename: Chieh-Lee
authors.initials: CL
authors.lastname: Wong
mesh_terms: D059786:Abnormal Karyotype; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008875:Middle Aged; D010677:Philadelphia Chromosome; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000235:genetics*