1/122. Transient leukemoid disorder in a newborn with down syndrome followed 19 months later by an acute myeloid leukemia: demonstration of the same structural change in both instances with clonal evolution.A transient leukemoid disorder (TLD) was observed in a newborn with down syndrome (DS), demonstrating a clonal abnormality: 47,XX,der(X;15)(p10;q10), 21(c). Spontaneous remission was observed, but 19 months later an acute leukemia from the myeloid series was discovered. Cytogenetic study revealed the same structural change as at birth, with karyotypic evolution corresponding to addition of one chromosome 8 and a fourth chromosome 21. These findings demonstrate, at least in our patient, that TLD and the subsequent acute leukemia are closely related and that TLD, closely related to DS, must be viewed as a preleukemic disorder undergoing spontaneous remission. A review of literature data shows that most cytogenetic studies reported so far are related to either TLD or acute leukemia in DS. Serial studies performed in the same patient are quite infrequent and, to the best of our knowledge, there is only one other report demonstrating a cytogenetic relation between TLD and the subsequent acute leukemia.- - - - - - - - - - ranking = 1keywords = leukemia, myeloid leukemia (Clic here for more details about this article) |
2/122. trisomy 21 as the sole acquired karyotypic abnormality in acute myeloid leukemia and myelodysplastic syndrome.We report five cases of myeloid disorders in which trisomy 21 ( 21) was found as the sole acquired karyotypic abnormality, comprising two cases of acute myeloid leukemia (AML) and three cases of myelodysplastic syndrome (MDS). In this series, MDS patients with 21 presented as high grade disease, which included two cases of refractory anemia with excess of blasts (RAEB) and one case of refractory anemia with excess of blasts in transformation (RAEBt), and showed rapid disease progression. Significant thrombocytopenia was observed in all three patients, and bone marrow examination showed a marked reduction in megakaryocytes. AML patients with 21 included one case each of AML-M2 and M4. Despite the poor prognosis reported in AML patients with 21 as the sole abnormality, the patient in our series who was able to complete intensive treatment was cured of disease. The role of 21 in leukemogenesis is reviewed.- - - - - - - - - - ranking = 0.63070603776677keywords = leukemia, myeloid leukemia (Clic here for more details about this article) |
3/122. Transient myeloproliferative disease of the newborn: case report with placental, cytogenetic, and flow cytometric findings.Transient myeloproliferative disease (TMD) of the newborn is a rare hematologic abnormality associated with trisomy 21. It is frequently difficult to distinguish the disorder from true congenital leukemia (TCL). Unlike leukemia, which has a clinically aggressive course, TMD generally resolves within weeks to months. We present a case of TMD of the newborn diagnosed on the basis of peripheral blood studies and describe the pertinent pathological findings within the placenta. Flow cytometric analysis of the blasts in the peripheral blood showed phenotypic heterogeneity with features consistent with megakaryocytic differentiation. Cytogenetic studies showed trisomy 21 within the blastic cells. The placenta showed villous dysmaturity with associated chorangiosis and prominent intravascular aggregates of primitive-appearing cells with focal, early vascular wall invasion. The neonate recovered fully and shows no evidence of disease at 2 years of age.- - - - - - - - - - ranking = 0.24771758489329keywords = leukemia (Clic here for more details about this article) |
4/122. Immunophenotype of a transient myeloproliferative disorder in a newborn with trisomy 21.Cytologic, immunologic, and cytogenetic studies were performed on the blast cells of a newborn with down syndrome and transient myeloproliferative disease. This hematologic disorder is uncommon, and occurs primarily in infants with down syndrome. This boy presented with a high white blood cell count and a high percentage of blast cells, without anemia or thrombocytopenia. Chromosome analysis showed a constitutional trisomy 21 without any other clonal abnormality. A three-color flow cytometric analysis was performed and revealed two different CD45 dim, CD34( ), CD117( ), CD56( ) immature subpopulations: the normal immature myeloid precursor and an immature blast cell population that expressed CD41, CD42, CD61, CD36, CD13, CD1a, and CD2. We postulate that this population could be the leukemic precursor involved in the acute megakaryoblastic leukemia frequently observed in children with down syndrome.- - - - - - - - - - ranking = 0.12385879244665keywords = leukemia (Clic here for more details about this article) |
5/122. Duplication of 1q in a child with down syndrome and myelodysplastic syndrome.cytogenetic analysis of bone marrow cells was performed on a 2-year-old African-American male with down syndrome (DS) and myelodysplastic syndrome (MDS), specifically refractory anemia with excess blasts in transformation (RAEB-T). Chromosome analysis showed, in addition to the constitutional trisomy 21, a trisomy of chromosome 11 and a dup(1)(q23q31). This duplication of 1q is apparently a new chromosomal abnormality in a child with MDS. Partial trisomy of the long arm of chromosome 1 has been reported by several authors and appears to represent a nonrandom chromosomal anomaly in patients with MDS/acute myelogenous leukemia and DS.- - - - - - - - - - ranking = 0.1253457190567keywords = leukemia, myelogenous (Clic here for more details about this article) |
6/122. Hodgkin's disease in association with down syndrome: a case report.The increased incidence of malignancies, especially acute leukemia, in down syndrome has been clearly established. The association of Hodgkin's disease with down syndrome has not been extensively documented, and only a few cases have been reported. We present here a case report of Hodgkin's disease in an 11-year-old female child with down syndrome. The child presented with a stage IVB nodular sclerotic Hodgkin's disease and died of progressive disease. We also present a brief review of the mechanisms of development of malignancy in down syndrome.- - - - - - - - - - ranking = 0.12385879244665keywords = leukemia (Clic here for more details about this article) |
7/122. Down's syndrome with myelodysplastic syndrome showing t(7;11)(p13;p14).We report a boy with Down's syndrome (DS) who developed myelodysplastic syndrome (MDS) after spontaneous remission of transient myeloproliferative disorder (TMD) at birth. Chromosomal analysis of the blasts in the MDS phase demonstrated t(7;11)(p13;p14) which had not been detected in the TMD phase. NUP98-HOXA9 chimera mRNA, which is known to be involved in t(7;11)(p15;p15) translocation in acute myeloid leukemia (AML), was not detected by reverse transcriptase-polymerase chain reaction, and NUP98 rearrangement was not detected by Southern blot analysis of the blasts in the MDS phase. Reciprocal translocation is very rare in AML/MDS in DS, and the t(7;11)(p13;p14) found in our patient was different from the recurrent translocation t(7;11)(p15;p15) previously reported.- - - - - - - - - - ranking = 0.12614120755335keywords = leukemia, myeloid leukemia (Clic here for more details about this article) |
8/122. A t(1;22)(p13;q13) in four children with acute megakaryoblastic leukemia (M7), two with down syndrome.We report four children with acute megakaryoblastic leukemia (AML-M7) and t(1;22)(p13;q13), two of them with down syndrome; their ages were 7 months, and 6, 7, and 10 years. These findings differ from those reported in children with M7 and t(1;22) at the age of presentation (exclusively under 1-year-old) and in the two cases associated with down syndrome (t[1;22], 21c) that may be due to the high heterogeneity of the chromosomal changes in children with AML. We cannot disregard ethnic difference distribution of chromosomal changes and age of presentation in Mexican children with AML.- - - - - - - - - - ranking = 0.61929396223323keywords = leukemia (Clic here for more details about this article) |
9/122. Acute megakaryoblastic leukemia in down syndrome: orbital infiltration.PURPOSE: To describe an uncommon ocular presentation of acute megakaryoblastic leukemia in a child with down syndrome. METHOD: Case report. Initial manifestation of disease was bilateral proptosis with secondary exposure keratitis caused by leukemic infiltration of the orbits. RESULTS: Bone marrow biopsy and immunophenotyping established the diagnosis of acute megakaryoblastic leukemia (FAB-M7). The leukemia was treated successfully with chemotherapy, with resolution of proptosis. The patient remained in remission more than 1 year after cessation of treatment. CONCLUSIONS: Bilateral proptosis can be a presenting sign of acute megakaryoblastic leukemia, a malignancy associated with down syndrome.- - - - - - - - - - ranking = 0.99087033957316keywords = leukemia (Clic here for more details about this article) |
10/122. Isolated pericardial effusion and transient abnormal myelopoiesis in a fetus with Down's syndrome.Isolated pericardial effusion was detected in a fetus at 34 weeks of gestation. A male infant weighing 2,044 g was born by cesarean section because of a non-assuring fetal heart rate pattern at 35 weeks of gestation. Transient leukocytosis (36,100/microl) with 49% blast cells was seen in this neonate. The infant's karyotype was 47, XY 21. The pericardial effusion disappeared after treatment with prednisolone at a dose of 2 mg/kg/day. hypothyroidism was subsequently found. Thus, the subject patient with Down's syndrome developed isolated pericardial effusion, transient abnormal myelopoiesis (TAM), and hypothyroidism. Because more than 20% of the infants with TAM and Down's syndrome develop acute nonlymphocytic leukemia in early childhood, he is being closely observed.- - - - - - - - - - ranking = 0.12385879244665keywords = leukemia (Clic here for more details about this article) |
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