PMID- 10847463
OWN - NLM
STAT- MEDLINE
DCOM- 20001006
LR  - 20200203
IS  - 0923-7534 (Print)
IS  - 0923-7534 (Linking)
VI  - 11
IP  - 4
DP  - 2000 Apr
TI  - Clinical, hematological and cytogenetic characteristics of atypical chronic 
      myeloid leukemia.
PG  - 441-4
AB  - BACKGROUND: Atypical chronic myeloid leukemia (aCML) is an infrequent chronic 
      myeloproliferative disorder characterized by leukocytosis, absence of 
      Philadelphia chromosome or BCR-ABL rearrangement, and marked myeloid dysplasia. 
      Some cases have an absolute monocytosis but can be distinguished from chronic 
      myelomonocytic leukemia (CMML) by the presence of a higher percentage (> 15%) of 
      circulating immature granulocytes. PATIENTS AND METHODS: In a series of 11 
      patients with a diagnosis of aCML according to the FAB proposals we have analyzed 
      the most relevant clinical, hematological and cytogenetic characteristics. 
      RESULTS: The median age was 65 years (16-84). All but one case showed, at time of 
      diagnosis, leukocytosis (median WBC was 36 x 10(9)/l), 55% had moderate anemia 
      and 36% had thrombocytopenia. Most cases had marked dysplasia, particularly in 
      the granulocytic lineage (82% of the cases), and all cases showed bone marrow red 
      hypoplasia. Cytogenetic abnormalities were present in 9 out of the 11 patients. 
      Trisomy 8 was observed in three cases and other clonal chromosomal abnormalities 
      included deletions of 5q, 13q, 17p, 12q, and 11q as well as a t(6;8)(p23;q22) 
      translocation. Fluorescence in situ hybridization (FISH) studies failed to 
      demonstrate ETV-6 gene involvement. The median survival time from diagnosis was 
      only 14 months (range 3-56 months). CONCLUSIONS: These data suggest that aCML is 
      a rare disease which is characterized by leukocytosis, with dysgranulopoiesis, BM 
      erythroid hypoplasia, chromosomal, though not recurrent, abnormalities and poor 
      prognosis.
FAU - Hernandez, J M
AU  - Hernandez JM
AD  - Department of Hematology, Hospital Universitario and Centro de Investigacion del 
      Cancer, Universidad de Salamanca-CSIC, Spain. jmhernandezr@aehh.org
FAU - del Canizo, M C
AU  - del Canizo MC
FAU - Cuneo, A
AU  - Cuneo A
FAU - Garcia, J L
AU  - Garcia JL
FAU - Gutierrez, N C
AU  - Gutierrez NC
FAU - Gonzalez, M
AU  - Gonzalez M
FAU - Castoldi, G
AU  - Castoldi G
FAU - San Miguel, J F
AU  - San Miguel JF
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - Ann Oncol
JT  - Annals of oncology : official journal of the European Society for Medical 
      Oncology
JID - 9007735
SB  - IM
MH  - Adolescent
MH  - Aged
MH  - Anemia
MH  - Bone Marrow Cells/pathology
MH  - Chromosome Aberrations
MH  - Female
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Leukemia, Myelogenous, Chronic, BCR-ABL 
      Positive/classification/genetics/*pathology
MH  - *Leukocytosis
MH  - Male
MH  - Middle Aged
MH  - Prognosis
MH  - Survival Analysis
MH  - Thrombocytopenia
EDAT- 2000/06/10 09:00
MHDA- 2000/10/14 11:01
CRDT- 2000/06/10 09:00
PHST- 2000/06/10 09:00 [pubmed]
PHST- 2000/10/14 11:01 [medline]
PHST- 2000/06/10 09:00 [entrez]
AID - S0923-7534(19)55020-7 [pii]
AID - 10.1023/a:1008393002748 [doi]
PST - ppublish
SO  - Ann Oncol. 2000 Apr;11(4):441-4. doi: 10.1023/a:1008393002748.