Case of chronic-phase chronic myelogenous leukemia with an abdominal hematopoietic tumor of leukemic clone origin.
We report a 59-year-old man with chronic myelogenous leukemia (CML) in chronic phase who presented with a large abdominal tumor. Biopsy revealed proliferation of granulocytic-, erythroid-, and megakaryocytic-lineage cells in a retroperitoneal lymph node. The BCR/ABL fusion gene was detected on a paraffin-embedded tissue section of the lymph node by double-color fluorescence in situ hybridization, indicating an extramedullary hematopoietic tumor of CML origin. This patient has achieved a complete cytogenetic response for 19 months with imatinib mesylate (STI571; Gleevec), in association with the regression of the tumor. However, the development of an extramedullary tumor in chronic-phase CML generally indicates a poor prognosis, because it commonly consists of blast proliferation and is followed by blast crisis in the marrow within a few months. This case, therefore, points to the importance of histological examination of extramedullary tumors in CML for evaluation of disease status and for therapeutic decisions.
Sakakura
Miho
M
Second Department of Internal Medicine, Mie University School of Medicine, Mie, Japan.
Ohishi
Kohshi
K
Nomura
Kenichi
K
Katayama
Naoyuki
N
Nishii
Kazuhiro
K
Masuya
Masahiro
M
Nakase
Kazunori
K
Shiku
Hiroshi
H
D001549:Benzamides; D001752:Blast Crisis / Q000473:pathology; D016044:Fusion Proteins, bcr-abl / Q000235:genetics; D006411:Hematopoiesis, Extramedullary; D006801:Humans; D000068877:Imatinib Mesylate; D015466:Leukemia, Myeloid, Chronic-Phase / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D008198:Lymph Nodes / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010879:Piperazines / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D012074:Remission Induction; D012186:Retroperitoneal Neoplasms / Q000473:pathology*
D001549:Benzamides; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate; D016044:Fusion Proteins, bcr-abl