PMID- 17701956
OWN - NLM
STAT- MEDLINE
DCOM- 20071024
LR  - 20070925
IS  - 0008-543X (Print)
IS  - 0008-543X (Linking)
VI  - 110
IP  - 7
DP  - 2007 Oct 1
TI  - Therapy may unmask hypoplastic myelodysplastic syndrome that mimics aplastic 
      anemia.
PG  - 1520-6
AB  - BACKGROUND: Rarely, patients who present with pancytopenia and are diagnosed 
      initially with aplastic anemia (AA) subsequently develop a myelodysplastic 
      syndrome (MDS). There has been controversy regarding whether the initial 
      diagnosis of AA is correct or whether these patients have hypocellular MDS at the 
      onset of pancytopenia. METHODS: The authors studied bone marrow (BM) specimens 
      from patients who were diagnosed initially with AA and subsequently with MDS from 
      a cohort of 128 consecutive patients who had AA during the period from 1993 to 
      2004. Cytogenetic and fluorescence in situ hybridization (FISH) analyses were 
      performed to assess for monosomy 7 retrospectively in a subset of patients. 
      RESULTS: Twelve patients were identified (age range, 26-79 years). At the time 
      they were diagnosed with AA, there was no evidence of dysplasia, the median BM 
      cellularity was 5% (range, from <1% to 15%), and all patients had a normal 
      karyotype. Therapy for 11 patients included immunomodulating agents, which were 
      accompanied by growth factors in 4 patients and 1 patient underwent BM 
      transplantation. One patient received growth factors only. The median interval to 
      the diagnosis of MDS was 9 months (range, 2-43 months). The median BM cellularity 
      was 30% (range, 5-90%), and dysplastic changes were observed in all patients. 
      Nine patients had an abnormal karyotype, and monosomy 7 was the most common 
      abnormality (n = 5 patients). FISH detected monosomy 7 in 6 samples at the time 
      MDS was diagnosed and in 2 samples at the time AA was diagnosed. CONCLUSIONS: The 
      detection of monosomy 7 in specimens that were considered AA and the short time 
      interval to a subsequent diagnosis of MDS suggests that these patients had 
      hypoplastic MDS at the onset of pancytopenia. Therapy may allow the detection of 
      MDS by enhancing cell growth.
FAU - Konoplev, Sergej
AU  - Konoplev S
AD  - Department of Hematopathology, The University of Texas M. D. Anderson Cancer 
      Center, Houston, Texas 77030, USA.
FAU - Medeiros, L Jeffrey
AU  - Medeiros LJ
FAU - Lennon, Patrick A
AU  - Lennon PA
FAU - Prajapati, Sapana
AU  - Prajapati S
FAU - Kanungo, Anuradha
AU  - Kanungo A
FAU - Lin, Pei
AU  - Lin P
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Cancer
JT  - Cancer
JID - 0374236
RN  - 0 (Immunologic Factors)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Anemia, Aplastic/blood/*diagnosis/drug therapy/genetics/*therapy
MH  - Bone Marrow Transplantation
MH  - *Chromosomes, Human, Pair 7
MH  - Cytogenetic Analysis
MH  - Diagnosis, Differential
MH  - Female
MH  - Hematologic Tests
MH  - Humans
MH  - Immunologic Factors/therapeutic use
MH  - In Situ Hybridization, Fluorescence
MH  - Karyotyping
MH  - Male
MH  - Middle Aged
MH  - *Monosomy
MH  - Myelodysplastic Syndromes/blood/*diagnosis/drug therapy/genetics/*therapy
MH  - Retrospective Studies
EDAT- 2007/08/19 09:00
MHDA- 2007/10/25 09:00
CRDT- 2007/08/19 09:00
PHST- 2007/08/19 09:00 [pubmed]
PHST- 2007/10/25 09:00 [medline]
PHST- 2007/08/19 09:00 [entrez]
AID - 10.1002/cncr.22935 [doi]
PST - ppublish
SO  - Cancer. 2007 Oct 1;110(7):1520-6. doi: 10.1002/cncr.22935.