1/12. Monocytic leukemia cutis diagnosed simultaneously with refractory anemia with monocytosis: a case report.A case of leukemia cutis (LC) of monocytic lineage in a patient with myelodysplastic syndrome (MDS) is presented. Cutaneous infiltrates were recognized concurrent with diagnosis of refractory anemia (RA) with monocytosis. skin infiltrates subsequently spontaneously regressed although MDS progressed with increasing monocytosis, anemia, and thrombocytopenia. death occurred 6 months after diagnosis with evolution of acute monoblastic leukemia complicated by sepsis. This case supports previous observations of poor prognosis associated with leukemia cutis. LC associated with MDS is reviewed including the role of monocytes.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
2/12. erythropoietin-dependent transformation of myelodysplastic syndrome to acute monoblastic leukemia.Acute monoblastic leukemia (acute myeloid leukemia [AML], French-American-British type M5a) with leukemia cutis developed in a patient 6 weeks after the initiation of erythropoietin (EPO) therapy for refractory anemia with ringed sideroblasts. AML disappeared from both marrow and skin after the discontinuation of EPO. Multiparameter flow cytometric analysis of bone marrow cells demonstrated coexpression of the EPO receptor with CD45 and CD13 on the surface of blasts. The incubation of marrow cells with EPO, compared to without, resulted in 1.3- and 1.6-fold increases, respectively, in tritiated thymidine incorporation and bromodeoxyuridine incorporation into CD13( ) cells. Clinical and laboratory findings were consistent with the EPO-dependent transformation of myelodysplastic syndrome (MDS) to AML. It is concluded that leukemic transformation in patients with MDS treated with EPO may be EPO-dependent and that management should consist of the discontinuation of EPO followed by observation, if clinically feasible.- - - - - - - - - - ranking = 5keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
3/12. Granulocytic sarcoma presenting with malignant anasarca in a patient with secondary acute myeloid leukemia.Granulocytic sarcomas (GS) are rare extramedullary tumor masses composed of immature cells derived from the hematopoietic myeloid series. GS occur in 3% to 7% of cases of acute myeloid leukemia (AML) and can present before, during, or even after the diagnosis of AML. GS can involve different organs, individually or simultaneously, including the skin, lymph nodes, bone, breast, central nervous system, and lung among others. GS involving peritoneal and pleural fluids is a rare presentation. We present an unusual case of a patient with myelodysplastic syndrome whose disease progressed into a secondary AML and developed GS in the ascitic and pleural effusions as the predominant manifestation of disease progression.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
4/12. Acute nonlymphocytic leukemia after thiotepa instillation into the bladder: report of 2 cases and review of the literature.We report 2 cases of acute nonlymphocytic leukemia after thiotepa instillation into the bladder for superficial bladder carcinoma and review 4 additional cases from the literature. Intravesical thiotepa is absorbed systemically in patients with bladder carcinoma and such treatment may be associated with the rare occurrence of acute nonlymphocytic leukemia and/or the myelodysplastic syndrome.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
5/12. Monocytic involvement by monosomy 7 preceded acute myelomonocytic leukemia in a patient with myelodysplastic syndrome.Novel techniques were used to detect which cell lineages were affected by monosomy 7 in a patient who had myelodysplastic syndrome and later developed acute leukemia. The patient had had paroxysmal nocturnal hemoglobinuria for 20 years before developing refractory anemia with excess of blasts. cytogenetic analysis at the myelodysplastic stage disclosed monosomy 7 in bone marrow mitoses. Restriction fragment length polymorphism analysis of fractionated white blood cells with the chromosome 7-specific probes MetH and MetD revealed that blood monocytes and most bone marrow erythroblasts but not blood granulocytes or lymphocytes were affected by monosomy 7. The patient later developed acute myelomonocytic leukemia with blast cells positive for markers of the myelomonocytic lineage but negative for granulocytic markers in a standard surface marker analysis. The leukemic blast cells had monosomy 7 as determined by direct cytogenetic investigation. Thus, the monocytes were found to be affected by monosomy 7 in this patient 8 months before her myelodysplastic syndrome progressed to acute myelomonocytic leukemia, and the affected cells had the same biologic markers at both stages of the disease.- - - - - - - - - - ranking = 6.2696929838146keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
6/12. Establishment of a leukaemic cell line from a patient with acquisition of chromosomal abnormalities during disease progression in myelodysplastic syndrome.A cell line designated SKM-1 was newly established from leukaemic cells of a 76-year-old Japanese male patient with monoblastic leukaemia following myelodysplastic syndrome (MDS). The cells were obtained from peripheral blood of the patient when he lost multiple point mutations of ras genes with acquisition of chromosomal abnormalities during disease progression in MDS. The cells grew as a single floating cell, and have been continuously growing with the morphological characteristics of immature monoblasts by serial passages during the past 42 months with a doubling time of about 48 h. By cytochemical analysis, the cloned cells were positive for butyrate esterase, but negative for the Epstein-Barr virus associated nuclear antigen. Phenotypic analysis revealed the expression of myelomonocyte specific antigens such as CD4, CD13, CD33 and HLA-DR. Cells from the primary peripheral blood and those from 50 passages of the SKM-1 cell line both possessed no activated ras genes but showed karyotype abnormalities with 46,XY, del(9)(q13;q22), der(17) t(17;?)(p13;?). The SKM-1 cells have two mutations in p53 gene and overexpress the p53 products. This cell line may contribute to a better understanding of molecular mechanisms in the progression from MDS to myelogenous leukaemia.- - - - - - - - - - ranking = 5keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
7/12. Whole arm translocation t(17;18): a non-random abnormality of myeloid cell proliferation.Whole arm translocation t(17;18) was detected in two patients, one with acute monocytic leukemia and the other with acute transformation of chronic myelocytic leukemia. Dual-color fluorescence in situ hybridization (FISH) to interphase nuclei with alphoid probes specific to chromosomes 17 and 18 showed the presence of two very close spots. This feature was interpreted as the conservation of the pericentromeric region of the two chromosomes involved in the translocation. The present cases add to eight previously reported other patients with whole arm translocation t(17;18) (one with FISH studies). Since these patients had either myeloid leukemia or myelodysplastic syndrome, it is suggested that the t(17;18)(p10;q10) translocation is a new non-random abnormality associated with myeloid cell proliferations.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
8/12. Translocation (12;14)(q13;q32) in myelodysplastic syndrome.We report a patient diagnosed with refractory anemia with excess blasts in transformation (RAEB-t) who underwent an evolution to a nonlymphocytic acute leukemia (ANLL-M5a). Initial cytogenetic study showed a diploid karyotype; however, when ANLL-M5a was diagnosed, the bone marrow (BM) cells showed a t(12;14)(q13;q32), which to our knowledge has not been described previously in a myelodysplastic syndrome (MDS).- - - - - - - - - - ranking = 5keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
9/12. t(3;21)(q26;q22) with AML1 rearrangement in a de novo childhood acute monoblastic leukaemia.t(3;21)(q26;q22) is a recurrent chromosomal abnormality in philadelphia-positive chronic myeloid leukaemia in blast crisis and in treatment-related myelodysplastic syndrome and acute myeloid leukaemia. The molecular consequences of the t(3;21) are presently being unravelled; various transcripts between the AML1 gene in 21q22 and several unrelated genes, i.e. EAP, EVI1 and MDS1, in 3q26 are generated, resulting in the formation of a chimaeric transcription factor. The t(3;21) has only rarely been described in de novo leukaemias and never before in an acute leukaemia in a child. We here present the clinical, cytogenetic and molecular genetic findings in a boy with a de novo acute monoblastic leukaemia with t(3;21)(q26;q22) and AML1 rearrangement.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
10/12. Two acute monocytic leukemia (AML-M5a) cell lines (MOLM-13 and MOLM-14) with interclonal phenotypic heterogeneity showing MLL-AF9 fusion resulting from an occult chromosome insertion, ins(11;9)(q23;p22p23).We describe two new human leukemia cell lines, MOLM-13 and MOLM-14, established from the peripheral blood of a patient at relapse of acute monocytic leukemia, FAB M5a, which had evolved from myelodysplastic syndrome (MDS). Both cell lines express monocyte-specific esterase (MSE) and MLL-AF9 fusion mRNA. gene fusion is associated with a minute chromosomal insertion, ins(11;9)(q23;p22p23). MOLM-13 and MOLM-14 are the first cell lines with, and represent the third reported case of, MLL gene rearrangement arising via chromosomal insertion. Both cell lines carry trisomy 8 which was also present during the MDS phase, as well as the most frequent trisomies associated with t(9;11), ie, 6, 13, 19 variously present in different subclones. Despite having these features in common, differences in antigen expression were noted between the two cell lines: that of MOLM-13 being CD34 , CD13-, CD14-, CD15 , CD33 ; whereas MOLM-14 was CD4 , CD13 , CD14 , CD15 , CD33 . Differentiation to macrophage-like morphology could be induced in both cell lines after stimulation with INF-gamma alone, or in combination with TNF-alpha, which treatment also induced or upregulated, expression of certain myelomonocyte-associated antigens, including CD13, CD14, CD15, CD64, CD65 and CD87. Together, these data confirm that both cell lines are likely to be novel in vitro models for studying monocytic differentiation and leukemogenesis.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
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