Acute megakaryoblastic leukemia(AML-M7)is a rare neoplastic hematologic disorder,the pathogenesis is not clearly.Recent research has found many eytogenetic changes in this disease;and also some genes mutation,such as GATAI,p53,RUNXI play very important roles in AMKL.The aim of this article is to review the cytogenetic and molecule mechanism of acute megakaryoblastic leukemia.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Autoantibodies ; biosynthesis ; Case-Control Studies ; Child ; Female ; Humans ; Immunoglobulin G ; biosynthesis ; Male ; Middle Aged ; Pancytopenia ; immunology ; pathology ; Receptors, Erythropoietin ; physiology ; Young Adult

Hemoglobinuria, Paroxysmal ; metabolism ; Humans ; Membrane Proteins ; biosynthesis

Objective To probe the clinical features and possible mechanisms of autoimmunic hemocytopenia complicated with thrombosis.Methods From 2007 to 2008,the clinical and laboratory data of seven patients with autoimmunic hemocytopenia complicated with thrombosis in the hematology department General Hospital tianjin medical university were analysed.Results One of the patients was with Evans syndrome,and another six patients with immuno-related pancytopenia.Three were complicated with lower extremity Veins embolism;two with cerebral vessel and pulmonary artery embolisms respectively;one with splenic artery and coronary artery embolisms respectively.Among them,three were complicated embolism of two parts;six patients had higher D-Dimer level.They were treated with glucocorticoids,cyclosporin A,and immunoglobulin for immunic hemocytopenia and with anti-coagulation therapy(aspirin、heprin and warfarin) for thrombosis.Seven patients' hemogram,signs and symptoms improved gradually since they received immunosuppression and anticoagulation therapy.The symptoms of thrombosis disappeared two weeks later.The degree and extent of thrombosis also alleviated;at the same time,the function of blood coagulation recovered partly or completely.Conclusion Thrombosis is a rare but severe complication of autoimmunic hemocytopenia.Promptly anti-coagulation and immunosuppressive therapies can get ideal therapeutic effects.

Idiopathic cytopenia of uncertain significance is a new term which describes a cytopenia in one or more myeloid lineages that is constant,does not meet the minimal criteria of an MDS and cannot be explained with any other hematologic or non-hematologic diseases.The proposal,diagnosis and therapy of ICUS are reviewed in this paper.

Objective To analyse the occurrence and prognosis related factors of renal dysfunction in patients with multiple myeloma(MM).Methods During Janaury 1991 and Janaury 2007 seventy-four cases of MM in Department of Hematology,Gernal Hospital of Tianjin Medical University were enrolled in this study.The occurrence,the risk factors and prognosis were analyzed.Results The incidence of renal dysfunction(RD)in MM was 56.8%.Age,hypertention,hemoglobin,serum ALB,GLO,calcium,phosphonium,?2-MG,the percentage of plasma and immature plasma cells in bone marrow,Durie-Salmon stage and type of MM were associated with the incidence to RD with MM by monovariam analysis.Hypertention,serum ALB and ?2-MG were associated with the incidence of RD with MM by multivariant analysis.ALB was the protected factor and the other two were risk factors.The renal function improved rapidly in the patients who received CR or blood transfusion for severe and moderate anemia.Compared with patients of normal renal funtion,RD patients survived shorter and had higher early death rate.Conclusion Hypertention and high tumor burden are risk factors of renal dysfunction in MM.Effective chemothemapy and supportive treatment helped renal function recovery.

Objective To examine mutations in the WT1 and PLCE1 gene in three Chinese families with autosomal recessive steroid-resistant nephrotic syndrome (SRNS) once mutations in NPHS2 had been excluded. Methods Peripheral blood samples were collected for genetic analysis from three probands of three Chinese families and their parents, and two probands' siblings, and 50 adult volunteers with normal urinalysis. Genomic DNA was isolated from peripheral blood leucocytes. Ten exons and exon-intron boundaries of WT1, and 31 exons and exon-intron boundaries of PLCE1 were amplified by polymerase chain reaction (PCR). Mutational analysis was performed by DNA sequencing directly and RFLP (restriction fragment length polymorphism) and/or PCR. Results No mutation in both WT1 and PLCE1 was identified in three probands from three Chinese families with autosomal recessive SRNS. However, three variants of WT1, 126C>T, ⅣS5-64A>G and 903A>G, and 13 variants of PLCE1, -134A>G, 810T>C, 960G>A, ⅣS11-28C>G, ⅣS15+26A>C, 4724G>C, ⅣS20+40C>T, ⅣS21+64G>A, ⅣS22-26T> A, 5320C>T, 5780A>G, ⅣS27+24A>G and ⅣS31 +48_49insT, were detected in three probands and some controls, indicating that all these variants were gene polymorphisms. WT1 polymorphism ⅣS5-64A>G, and PLCE1 polymorphism ⅣS22-26T>A were novel. Conclusion All the encoding exons and exon-intron boundaries of both WT1 and PLCE1 in three probands are examined, and no causative mutations in WT1 and PLCE1 axe found, suggesting that mutation in WT1 and PLCE1 genes is not a major cause of the Chinese families with autosomal recessive SRNS.

Child ; Child, Preschool ; Female ; Humans ; Infant ; Intracellular Signaling Peptides and Proteins ; genetics ; Male ; Membrane Proteins ; genetics ; Nephrotic Syndrome ; ethnology ; genetics ; WT1 Proteins ; genetics

Cells, Cultured ; Comet Assay ; DNA Damage ; drug effects ; Dose-Response Relationship, Drug ; Humans ; Lymphocytes ; drug effects ; Styrene ; adverse effects

Adolescent ; Anticonvulsants ; therapeutic use ; Body Mass Index ; Child ; Child, Preschool ; Epilepsy ; blood ; drug therapy ; Female ; Galanin ; blood ; Humans ; Infant ; Male ; Valproic Acid ; blood ; therapeutic use