Dicentric dic(7;9)(p11;p11): a new case in childhood ALL

Elvira 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)

Previous history

Preleukaemia
-
Malignant disease
-
Inborn condition
-
Main items
30 days of asthenia, petechia and ecchymosis.

Clinics case report

Age
13 yrs
Sex
F
Liver
+
Spleen
+
Lymph nodes
+
Cns involv
-

Blood data

Wbc
28.5
Hb
6.6
Platelets
110
Blasts
64
Bone marrow
72.4% of lymphoid blast cells

Cyto path

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 data

Date diagnosis
02-2009
Treatment
Chemotherapy with BFM95 for intermediate risk
Complete remission
+ remission was obtained after the first induction cycle
Treatment relat death
-
Relapse
-
Status
A
Date last follow
04-2009
Survival
1

Karyotype

Sample
Bone marrow
Culture time
24 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 relapse
not applied
Mol cytogenet 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 molec studies

Technics
not done

Images

Atlas Image
Figure 1: G-banding karyotype showing the first clone with monosomy 7 and one marker (recognized as a der(9) after sky study).
Atlas Image
Figure 2: Partial G- banding karyotypes showing the second clone with dic(7;9)(p11;p11).
Atlas Image
Figure 3: Sky study showing the first clone with monosomy 7 and a der(9).
Atlas Image
Figure 4: Sky study showing the second clone with dic(7;9)(p11;p11).

Comments section

Comments
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.

Bibliography

Pubmed IDLast YearTitleAuthors
20188381991New recurring chromosomal translocations in childhood acute lymphoblastic leukemia.Raimondi SC et al
19950901991Philadelphia chromosome and monosomy 7 in childhood acute lymphoblastic leukemia: a Pediatric Oncology Group study.Russo C et al
104776771999Association of chromosome arm 9p abnormalities with adverse risk in childhood acute lymphoblastic leukemia: A report from the Children's Cancer Group.Heerema NA et al
169385752006Dicentric (7;9)(p11;p11) is a rare but recurrent abnormality in acute lymphoblastic leukemia: a study of 7 cases.Pan J et al
166161162006Three adults with acute lymphoblastic leukemia and dic(7;9)(p11.2;p11).Smith A et al

Citation

Elvira 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

Dicentric dic(7;9)(p11;p11): a new case in childhood ALL

Atlas Genet Cytogenet Oncol Haematol. 2009-09-01

Online version: http://atlasgeneticsoncology.org/case-report/208841/dicentric-dic(7;9)(p11;p11)-a-new-case-in-childhood-all