Title of article :
A Case with Neurofibromatosis and Chronic Myeloid Leukemia in Blastic Crisis Treated with Imatinib
Author/Authors :
Abdul Hamid، Gamal نويسنده Hematology-Oncology Department, Al-Gamhouria Teaching Hospital, Aden, Yemen , , Harize، Iman Bin نويسنده National Oncology Center, Aden, Yemen ,
Issue Information :
فصلنامه با شماره پیاپی 18 سال 2014
Abstract :
A 61-year-old female presented with complaints of fever, general weakness and
hepatosplenomegaly. She had a history of nonfamilial peripheral neurofibromatosis
diagnosed as von Recklinghausenʹs disease since 30 years previous.
Physical examination was remarkable for skin colored cutaneous circumscribed
nodules which appeared soft to the touch in both arms, the upper part of her abdomen,
back, and posterior thigh. The liver was palpable 10 cm below the inferior border of
the costal margin and she had evidence of significant splenomegaly. Laboratory results
were as follows: hemoglobin 7.9 g/dl; ESR142 mm/hour; leukocytes 22400x109/L;
neutrophils 35%; eosinophils 3%; basophils 4%; myelocytes40%; myeloblasts 14%;
promyelocytes 2%; and band form 2%. The bone marrow picture was chronic myeloid
leukemia in blastic form. Chest CT scan showed the presence of numerous cutaneous
nodules (neurofibromatosis). A biopsy of the tissue fragment from the nodules
confirmed the presence of diffuse neurofibromatosis. Bone marrow cytology that
included cytogenetic and immunophenotyping confirmed the presence of chronic
myeloid leukemia with a positive Philadelphia chromosome and diploidy female
clone in a blastic form (acute myeloid leukemia). Addition of 600 mg oral imatinib
mesylate daily for one month and reduced to 400 mg daily yields complete hematological
remission and complete cytogenetic responses.
This case illustrated an association between chronic myeloid leukemia, acute
myeloid leukemia and neurofibromatosis in an adult.
Journal title :
Middle East Journal of Cancer (MEJC)
Journal title :
Middle East Journal of Cancer (MEJC)