PMID- 18308388
OWN - NLM
STAT- MEDLINE
DCOM- 20080724
LR  - 20151119
IS  - 0145-2126 (Print)
IS  - 0145-2126 (Linking)
VI  - 32
IP  - 9
DP  - 2008 Sep
TI  - Long-term remission with imatinib mesylate in Philadelphia chromosome-positive 
      AML presenting as primary extramedullary myeloid sarcoma.
PG  - 1476-9
LID - 10.1016/j.leukres.2008.01.012 [doi]
AB  - In this case report, we describe the first account in the literature of a patient 
      who presented with extramedullary myeloid sarcomas (EMS) and Ph+ AML without 
      leukemic manifestations. EMS are rare, destructive, extramedullary tumor masses 
      that consist of immature leukemia cells. These tumors can occur anywhere in the 
      body and have to be differentiated from lymphoma, carcinoma or infectious 
      processes. We report a previously healthy 61-year-old male who presented with 
      progressive left-sided hip pain. Magnetic resonance imaging (MRI) of the left hip 
      joint revealed an extensive lytic lesion with pathological fracture of the femur. 
      The lesion was confirmed by biopsy to be EMS with bcr-abl rearrangement in tumor 
      cell nuclei by fluorescence in situ hybridization (FISH). Laboratory tests showed 
      normal metabolic and complete blood counts with normal differential. Peripheral 
      blood RT-PCR for bcr-abl was negative. Bone marrow examination revealed a 
      normocellular with progressive trilineage hematopoiesis and no evidence of 
      increased blast count. On the basis of available literature for the treatment of 
      EMS, our patient received standard AML induction chemotherapy consisting of 
      idarabucin and cytarabine. After cytogenetics studies of the bone marrow revealed 
      bcr-abl rearrangement, we discontinued further chemotherapy and managed the 
      patient with imatinib mesylate 400mg p.o.q. day. Follow-up at time of manuscript 
      preparation, the patient remains in complete cytogenetic remission (CCR) for 22 
      months. To the best of our knowledge, this is the first case report of Ph+ AML 
      presenting as a primary EMS without leukemic manifestations.
FAU - Ahmed, Mohamed S
AU  - Ahmed MS
AD  - Division of Neoplastic Diseases & Related Disorders Department of Medicine, 
      Medical College of Wisconsin, 9200 West Wisconsin Avenue, Milwaukee, WI 53226, 
      USA. mahmed@mcw.edu
FAU - Kroft, Steven H
AU  - Kroft SH
FAU - Davis, Nancy B
AU  - Davis NB
FAU - King, David M
AU  - King DM
FAU - Cheng, Yee Chung
AU  - Cheng YC
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20080304
PL  - England
TA  - Leuk Res
JT  - Leukemia research
JID - 7706787
RN  - 0 (Benzamides)
RN  - 0 (Piperazines)
RN  - 0 (Protein Kinase Inhibitors)
RN  - 0 (Pyrimidines)
RN  - 8A1O1M485B (Imatinib Mesylate)
RN  - EC 2.7.10.1 (Protein-Tyrosine Kinases)
RN  - EC 2.7.10.2 (Fusion Proteins, bcr-abl)
SB  - IM
MH  - Benzamides
MH  - Chromosome Aberrations
MH  - Cytogenetic Analysis
MH  - Fusion Proteins, bcr-abl/genetics
MH  - Humans
MH  - Imatinib Mesylate
MH  - In Situ Hybridization, Fluorescence
MH  - Karyotyping
MH  - Leukemia, Myelogenous, Chronic, BCR-ABL Positive/*drug therapy/pathology
MH  - Magnetic Resonance Imaging
MH  - Male
MH  - Middle Aged
MH  - Piperazines/*therapeutic use
MH  - Protein Kinase Inhibitors/*therapeutic use
MH  - Protein-Tyrosine Kinases/antagonists & inhibitors
MH  - Pyrimidines/*therapeutic use
MH  - Remission Induction
MH  - Reverse Transcriptase Polymerase Chain Reaction
MH  - Sarcoma, Myeloid/*drug therapy/pathology
EDAT- 2008/03/01 09:00
MHDA- 2008/07/25 09:00
CRDT- 2008/03/01 09:00
PHST- 2007/11/24 00:00 [received]
PHST- 2008/01/11 00:00 [revised]
PHST- 2008/01/14 00:00 [accepted]
PHST- 2008/03/01 09:00 [pubmed]
PHST- 2008/07/25 09:00 [medline]
PHST- 2008/03/01 09:00 [entrez]
AID - S0145-2126(08)00020-9 [pii]
AID - 10.1016/j.leukres.2008.01.012 [doi]
PST - ppublish
SO  - Leuk Res. 2008 Sep;32(9):1476-9. doi: 10.1016/j.leukres.2008.01.012. Epub 2008 
      Mar 4.