Double Philadelphia chromosome-positive B acute lymphoblastic leukaemia in an elderly patient
- Author:
Tang Yee-Loong
;
Raja Zahratul Azma Raja Sabudin
;
Leong Chooi-Fun
;
Clarence Ko Ching-Huat
- Publication Type:Journal Article
- Keywords:
Double Philadelphia chromosomes;
acute lymphoblastic leukaemia;
imatinib;
BCR/ABL
- From:The Malaysian Journal of Pathology
2015;37(3):275-279
- CountryMalaysia
- Language:English
-
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.
- Full text:P020160315421157693506.pdf