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CASE REPORTS in HAEMATOLOGY
(Paper co-edited with the European LeukemiaNet)
Dicentric dic(7;9)(p11;p11): a new case in childhood ALL
 
Written2009-09Elvira D Rodrigues Pereira Velloso, Carolina Kassab, Silvia Helena A Figueira, Denise Tiemi Noguchi, Eliana Carla Armelin Benites, Cristóvão L P Mangueira, Fábio Morato de Oliveira
Clinical Laboratory, Hospital Israelita Albert Einstein, Sao Paulo, Brazil (EDRPV, CK, SHAF, CLPM); Instituto de Clinicas Pediatricas Bolivar Risso - GRENDACC, Sao Paulo, Brazil (DTN, ECAB); Cytogenetics and Onco-Hematology, FMRP USP, Brazil (FMDO)
Clinics
Age and sex : 13 year(s) old female patient.
Previous History : no preleukemia
no previous malignant disease
no inborn condition of note
Organomegaly : hepatomegaly ; splenomegaly ; enlarged lymph nodes ; no central nervous system involvement
Blood
WBC : 28.5 x 109/L ; Hb : 6.6 g/dL ; platelets : 110 x 109/L; blasts : 64 % .
Bone marrow : 72.4% of lymphoid blast cells
Cyto pathology classification
Cytology : ALL-L1
Immunophenotype : Blast cells positivity for: CD 34, HLA, CD10, CD19, CD22 and cCD79a.
Rearranged Ig Tcr : not done
Pathology : not done
Electron microscopy : not done
Precise diagnosis : Common B (B-II) ALL at diagnosis.
Survival
Date of diagnosis: 02-2009
Treatment : Chemotherapy with BFM95 for intermediate risk
Complete remission was obtained
Comments : remission was obtained after the first induction cycle
Treatment related death : -
Relapse : -
Status : Alive 04-2009
Survival : 1 month(s)
Karyotype
Sample : Bone marrow ; culture time : 24 h, and 48 h without stimulating agents ; banding : GAW- band
Results : 45,XX,-7,+mar[11]/45,XX,dic(7;9)(p11;p11)[9]/46,XX[4]
Karyotype at relapse : not applied
Other molecular cytogenetics technics : Spectral Karyotyping (SKY) using SkyPaint ASR (Applied Spectral Imaging). The marker seen in the first clone was elucidated as a derivative chromosome 9 (figure 3). The study confirm the der(7;9) seen in the second clone (figure 4).
Other molecular studies
technics : not done
Figure 1: G-banding karyotype showing the first clone with monosomy 7 and one marker (recognized as a der(9) after sky study).
Figure 2: Partial G- banding karyotypes showing the second clone with dic(7;9)(p11;p11).
Figure 3: Sky study showing the first clone with monosomy 7 and a der(9).
Figure 4: Sky study showing the second clone with dic(7;9)(p11;p11).
Comments
The case presented here is, to our knowledge, the 19th reported case of dic (7;9)(p11;p11) in acute lymphoblastic leukemia. From the literature review, 10 patients were less than 15 years old, seven with FAB L1 morphology, like our patient. She presented a enlarged liver and spleen, as ten and six of the cases reported in the studies, but without hyperleukocytosis, which was common for ALL patients with simultaneous dic(7;9) and t(9;22), present in 9 of 18 cases.
The prognostic significance of this abnormality remains controversial. Russo et al. suggested that the deletion of tumor suppressor genes located on 7p is associated with an adverse prognostic factor in ALL. Heerema et al. related that abnormalities in chromosome 9p are associated with increased relapse in children with ALL, probably because the inactivation of the tumor suppressor genes CDKN2 and CDKN1, mapped to 9p21-22. In the series of Pan et al. (2006), the prognosis of the patients with dic(7;9) and t(9;22) is worse than that of those with isolated dic(7;9).
From the 18 cases, 11 have no data of survival, 4 achieved long term remission and 3 died. Our children remain in clinical remission 45 days after induction therapy with intermediate risk BFM 95 (Berlin-Frankfurt-Munster) protocol.
Internal links
Atlas Carddic(7;9)(p11-13;p11)
Bibliography
New recurring chromosomal translocations in childhood acute lymphoblastic leukemia.
Raimondi SC, Privitera E, Williams DL, Look AT, Behm F, Rivera GK, Crist WM, Pui CH.
Blood. 1991 May 1;77(9):2016-22.
PMID 2018838
 
Philadelphia chromosome and monosomy 7 in childhood acute lymphoblastic leukemia: a Pediatric Oncology Group study.
Russo C, Carroll A, Kohler S, Borowitz M, Amylon M, Homans A, Kedar A, Shuster J, Land V, Crist W, et al.
Blood. 1991 Mar 1;77(5):1050-6.
PMID 1995090
 
Association of chromosome arm 9p abnormalities with adverse risk in childhood acute lymphoblastic leukemia: A report from the Children's Cancer Group.
Heerema NA, Sather HN, Sensel MG, Liu-Mares W, Lange BJ, Bostrom BC, Nachman JB, Steinherz PG, Hutchinson R, Gaynon PS, Arthur DC, Uckun FM.
Blood. 1999 Sep 1;94(5):1537-44.
PMID 10477677
 
Dicentric (7;9)(p11;p11) is a rare but recurrent abnormality in acute lymphoblastic leukemia: a study of 7 cases.
Pan J, Xue Y, Wu Y, Wang Y, Shen J.
Cancer Genet Cytogenet. 2006 Sep;169(2):159-63.
PMID 16938575
 
Three adults with acute lymphoblastic leukemia and dic(7;9)(p11.2;p11).
Smith A, Das P, O'Reilly J, Patsouris C, Campbell LJ.
Cancer Genet Cytogenet. 2006 Apr 1;166(1):86-8.
PMID 16616116
 

Citation

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