Extramedullary leukemia in children presenting with proptosis
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Abstract Background We highlight the orbital manifestations of acute myeloid leukemia and the role of peripheral blood smear in the diagnosis of these cases. A total of 12 patients who presented with proptosis and were subsequently diagnosed to have acute myeloid leukemia based on incision biopsy or peripheral blood smear were included in the study. Results A retrospective review of all cases of acute myeloid leukemia presenting to the Orbital clinic was performed. The age at presentation, gender, presenting features, duration of symptoms and fundus features were noted. In addition the temporal relationship of the orbital disease to the diagnosis of leukemia, laterality, location of the orbital mass, imaging features and the diagnostic tools used to diagnose leukemia were noted. The median age at presentation was 6 years. The male: female ratio was 0.7:1. None of these patients had been diagnosed earlier as having acute myeloid leukemia. The presenting features included proptosis in all patients, orbital mass in 5 (41.7%), visual symptoms in 2 (16.7%) and subconjunctival hemorrhage in one patient (8.3%). A diagnosis of acute myeloid leukemia was established by incision biopsy in 4 patients, subsequently confirmed by peripheral blood smear testing and bone marrow biopsy in 2 patients which revealed the presence of systemic involvement. Imprint smears of the biopsy identified blasts in 2 of 4 cases. In 8 patients presenting with ocular manifestations, diagnosis was established by peripheral blood smear examination alone which revealed a diagnosis of acute myeloid leukemia. Conclusion A peripheral blood smear should be performed in all cases of sudden onset proptosis or an orbital mass in children and young adults along with an orbital biopsy. It can always be complemented with a bone marrow biopsy especially in cases of aleukemic leukemia or when the blood smear is inconclusive.Keywords:
Bone marrow examination
Animal model
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Objective:To explore the possibility of secondary neoplasia following long term combined chemotherapy.Methods:Sister chromatid exchanges(SCE)were investigated in 44 patients with adult leukemia.Rusults:Frequencies of SCE in patients with de novo leukemia,relapsed leukemia and chronic myeloid leukemia(CML)were significant higher than health controls.The five patients(5/16)with complete remission showed higher SCE frequencies,those were mainly the patients received more than nine courses of combined chemotherapeutic regimens received more than nine courses of combined chemotherapeutic regimens.Conclusion:The patients with de novo leukemia,relapsed leukemia and CML have DNA damage in diagnosis,long term combined chemotherapy may cause the DNA instability in partial patients.
Sister chromatid exchange
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Pathogenesis of atypical leukemia (hypoplastic leukemia and smoldering acute leukemia) is still unknown. The author intended to find the factors inducing its unique clinical course by comparing 5 cases of atypical leukemia with the same numder of typical acute leukemia with respect to their blasts cell size, laveling index with (3)H-thymidine and pattern of tissue growth by the bone marrow culture method devised by us. And obtained the following results. 1) The size of 100 blasts chosen randomely out of the bone marrow smear prepared from 5 cases of atypical leukemia was compared with those of typical acute leukemia. The mean val-ue of the former group was 41.9±12.3, while that of the latter was 61.4±16.7. There fore, the size of blasts in the atypical leukemia proved to be somewhat smaller than those of the typical acute leukemia. 2) The mean value of laveling index with (3)H-thymidine of the blasts in the bone marrow of the former group was 6.4% , while that of the latter was 12.9% . 3) The growth pattern of the bone marrow tissue from the 5 patients with atypical leukemia was as follows: The bone marrow explants from these patients were generally hypoplastic, consisting of an increase of fat cells intermingled with several foci scattered made of leukemic blasts. The growth zone revealed much lower cell density than that of the typical acute leukemia. The margin of the growth zone, however, sharply bordered and similar to the pattern of typical acute leukemia. Cells observed predominantly in the growth zone composed of immature leukemic cells and mature lymphocytes throughout the culture period of 24-48 hours. From these results, it is assumed that most of leukemic blasts in the bone marrow of the patients with atpical leukemia are dormant cells, i.e. belong to a nondividing compartment, and reduced multiplication rate is closely related to their mild clinical courses.
Thymidine
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Both acute myeloid leukemia 1 and c‐Fos are regulatory factors of hematopoietic cell differentiation. We identified that the c‐fos promoter contains an acute myeloid leukemia 1 binding site at nucleotide positions −6–+14. c‐fos promoter activity was induced by transient overexpression of acute myeloid leukemia 1 in Jurkat T‐cells, but not by that of the short form of acute myeloid leukemia 1‐MTG8, a chimeric acute myeloid leukemia 1 protein. In 32Dcl3 myeloid cells, stable overexpression of acute myeloid leukemia 1‐MTG8 blocked the c‐fos gene transcription and cell differentiation, but that of acute myeloid leukemia did not. These data suggest that acute myeloid leukemia 1 and acute myeloid leukemia 1‐MTG8 reciprocally regulate the myeloid cell differentiation, possibly by the way of regulating c‐fos gene transcription.
RUNX1T1
IRF8
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Pathogenesis of atypical leukemia (hypoplastic leukemia and smoldering acute leukemia) is still unknown. The author intended to find the factors inducing its unique clinical course by comparing 5 cases of atypical leukemia with the same numder of typical acute leukemia with respect to their blasts cell size, laveling index with 3H-thymidine and pattern of tissue growth by the bone marrow culture method devised by us. And obtained the following results.1) The size of 100 blasts chosen randomely out of the bone marrow smear prepared from 5 cases of atypical leukemia was compared with those of typical acute leukemia. The mean value of the former group was 41.9±12.3, while that of the latter was 61.4±16.7. Therefore, the size of blasts in the atypical leukemia proved to be somewhat smaller than those of the typical acute leukemia.2) The mean value of laveling index with 3H-thymidine of the blasts in the bone marrow of the former group was 6.4%, while that of the latter was 12.9%.3) The growth pattern of the bone marrow tissue from the 5 patients with atypical leukemia was as follows: The bone marrow explants from these patients were generally hypoplastic, consisting of an increase of fat cells intermingled with several foci scattered made of leukemic blasts. The growth zone revealed much lower cell density than that of the typical acute leukemia. The margin of the growth zone, however, sharply bordered and similar to the pattern of typical acute leukemia. Cells observed predominantly in the growth zone composed of immature leukemic cells and mature lymphocytes throughout the culture period of 24-48 hours.From these results, it is assumed that most of leukemic blasts in the bone marrow of the patients with atpical leukemia are “dormant cells”, i.e. belong to a nondividing compartment, and reduced multiplication rate is closely related to their mild clinical courses.
Childhood leukemia
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Objective:To investigate the expression of MMP-2 and MMP-9 in acute leukemia and normal control and explore the relationship among MMP-2、MMP-9 and leukemia pathogenesis and infiltration.Method:The expression of MMP-2 and MMP-9 in bone marrow cells of untreated acute leukemia, relapse stage of acute leukemia, complete remission (CR) stage of acute leukemia and normal control were examined by in situ hybridization.Result:The positive rate of MMP-2 and MMP-9 in untreated, especially with infiltration and relapse stage of acute leukemia are significantly higher than that in CR stage of acute leukemia and normal control, the positive rate of MMP-2 and MMP-9 in untreated acute lymphoblastic leukemia(ALL) and acute nonlymphoblastic leukemia (ANLL) (M_4+M_5)group are significantly higher than that in ANLL (non M_4+M_5 )group.Conclusion:MMP-2 and MMP-9 may play an important role in leukemia pathogenesis and infiltration by enhancing the degradation of ECM.
Infiltration (HVAC)
Pathogenesis
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Chronic leukemia
White blood cell
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Childhood leukemia
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To investigate the expression levels of serum sICAM-1 and their clinical significances in patients with hyperleukocytic acute leukemia(HLAL).The serum levels of sICAM-1 in 30 hyperleukocytic acute leukemia and 20 non-hyperleukocytic acute leukemia were detected by ELISA,compared with 20 healthy persons as the control group.The expression of sICAM-1 in patients with hyperleukocytic acute leukemia and non-hyperleukocytic acute leukemia were significantly higher than those in control group,expecially in hyperleukocytic acute leukemia.Over expression of sICAM-1 may play a key role in the genesis and progression of hyperleukocytic acute leukemia.
Clinical Significance
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Objective:To investigate the expression of CD 56 on acute leukemia and its clinical manifestation.Methods:Immunophenotype of 92 cases with acute leukemia was measured by flow cytometry,the morphology,immunophenotype and clinic of CD 56 positive patients was analyzed.Results:15/92(16.3%)cases with acute leukemia expressed CD 56 ,one patient has been tesified to be myeloid/NK cell precursor acute leukemia,one patient has been testified to be blastic NK cell leukemia,one patient has been testified to be NK like T cell lymphoma/leukemia,12 patients are like to be acute myeloid leukemia with NK antigen expression or myeloid/NK cell acute leukemia.Conclusion:CD 56 positive acute leukemia is heterogeneous,its morphology,immunophenotype and clinical manifestation is different,they were associated with M 2?M 5?L 2;they had more infiltration out marrow and prognosis was poor.
Immunophenotyping
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