Acute leukemia characterized by trisomy 8 in down's syndrome

Pathology ◽  
1985 ◽  
Vol 17 (1) ◽  
pp. 111-114 ◽  
Author(s):  
Alan F. Broomhead ◽  
Y.L Kwan ◽  
N.A. Zell ◽  
P.R.L. Lam-Po-Tang ◽  
D. O'Gorman Hughes
Blood ◽  
1990 ◽  
Vol 76 (11) ◽  
pp. 2348-2353 ◽  
Author(s):  
S Kojima ◽  
T Matsuyama ◽  
T Sato ◽  
K Horibe ◽  
S Konishi ◽  
...  

Abstract The clinical, hematologic, and immunophenotypic features in 20 patients with Down's syndrome (DS) and acute leukemia were analyzed. Of the 20 patients, all 14 patients who were 3 years old and less were diagnosed as having acute megakaryoblastic leukemia (AMKL) by use of platelet- specific monoclonal antibodies and platelet peroxidase (PPO) reaction in electron microscopy. They were characterized by the presence of bone marrow fibrosis, having a history of myelodysplastic syndrome (MDS) and a poor response to chemotherapy. Only one patient has remained in continuous complete remission for more than 1 year. Acute leukemia in six patients who were older than 4 years was classified as common acute lymphoblastic leukemia antigen (CALLA)-positive acute lymphoblastic leukemia (ALL). In one of six patients classified as ALL, the leukemic blasts simultaneously expressed myeloid-associated surface antigens. All six patients achieved a complete remission and have remained in continuous complete remission and have remained in continuous complete remission from 10 to 52 months from the initial diagnosis. Although it has been suggested that the distribution of types of acute leukemia in patients with DS is similar to that in normal children, the present study shows that the distribution of acute leukemia types is quite different from that in patients without Down's syndrome.


1986 ◽  
Vol 21 (2) ◽  
pp. 93-98 ◽  
Author(s):  
Frederick Hecht ◽  
Barbara K. Hecht ◽  
Rodman Morgan ◽  
Avery A. Sandberg ◽  
Michael P. Link

1987 ◽  
Vol 28 (1) ◽  
pp. 155-162 ◽  
Author(s):  
N. Wang ◽  
J. Leung ◽  
R.P. Warrier ◽  
M. Schorin ◽  
D. Kirkpatrick ◽  
...  

Blood ◽  
1988 ◽  
Vol 72 (1) ◽  
pp. 15-23
Author(s):  
Y Hayashi ◽  
M Eguchi ◽  
K Sugita ◽  
S Nakazawa ◽  
T Sato ◽  
...  

Cytogenetic, immunologic, and electron microscopic studies were performed on the blast cells of 28 pediatric patients with Down's syndrome, 13 with acute leukemia (DS-AL) and 15 with transient myeloproliferative disorders (DS-TMD). Clonal chromosome abnormalities were found in the cells of all patients with DS-AL but not those with DS-TMD. The younger ages and higher hemoglobin concentrations, platelet counts, and WBC counts of DS-TMD patients provided a clinical contrast with the frankly leukemic cases. Myelodysplastic syndrome, characterized by a small percentage of leukemic blast cells, was observed in 11 of the 13 patients with DS-AL compared with none in the DS-TMD group. Electron microscopy disclosed a positive platelet peroxidase reaction in each of the 11 DS-TMD patients and in nine of the 13 DS-AL patients. Immunologic studies revealed antiplatelet- megakaryocyte antigens on the blast cells of the majority of patients in both study groups. Our findings suggest that the blast cells in cases of DS-AL and DS-TMD arise from cells of the megakaryocytic lineage or from a myeloid progenitor with the capacity for megakaryocytic differentiation. The high risk of the development of AL in patients with DS who are less than 3 years old may be related to increased megakaryocyte proliferation in this age group.


Author(s):  
O.V. Petrova ◽  
O.I. Murygina ◽  
S.A. Shashin ◽  
D.M. Nikulina ◽  
D.G. Tarasov

Blood ◽  
1981 ◽  
Vol 58 (3) ◽  
pp. 459-466 ◽  
Author(s):  
Y Kaneko ◽  
JD Rowley ◽  
D Variakojis ◽  
RR Chilcote ◽  
JW Moohr ◽  
...  

Chromosome and cytologic studies were performed on three Down's syndrome (DS) patients with acute nonlymphocytic leukemia (ANLL). All three patients had an aneuploid clone in their leukemic cells: 50, XX, +6, +19, +21, +22, +8, XX, +21, and 47,XY, +8, - 21 +dic(21;21)(p13;p11). Every patient appeared to have acute undifferentiated leukemia when the blast cells were examined with Wright-Giemsa stain; cytochemistry studies, however, showed that the leukemic blasts were in an early stage of myeloid differentiation. The two patients with +8 had a preleukemic phase; the blast cells of the patient with an extra no. 19 and no.22 could not be differentiated morphologically from those of the two patients with an extra no. 8. Our findings and a review of data on 40 other patients suggest that most DS children with ANLL have hyperdiploidy, which is usually related to gains of C, F, and /or G chromosomes, and that the abnormalities of +8 and of +19, +22 in DS children may be associated with acute leukemia (AL) in an early stage of myeloid differentiation.


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