Idiopathic bone marrow failure anemia(BMF),a group of severe hematopoietic diseases including acquired aplastic anemia,pure red cell aplasia,immune related pancytopenia,immune related anemia,paroxysmal nocturnal hemogobinurea,myelodysplastic syndromes and idiopathic cytopenia of undetermined significances,is quite easy to be misdiagnosed clinically.The proper diagnoses of these diseases should be made on the complete and detailed information about the patient's symptoms and signs,results of regular blood tests,bone marrow morphology examination and investigations on the pathogenesis of BMF,and response to the tentative therapy if it is necessary.High curative rate of these diseases will be achieved with prompt and long enough therapies for supporting patient's life,blocking the pathogenic process and stimulating hematopoiesis.

Aplastic anemia(AA)is a life-threatening blood disease.The British guideline and Chinese experts' experiences indicate that it is related to the abnormality of cellular immunity.Diagnosis by adequate exclusion,strict indication for stem cell transplantation and more immunosuppression plus hematopoietic stimulating therapies should be paid more attention in the treatment of this disease.

There were great break through in the studies on aplastic anemia in China during the past 20 years.The annual incidence of aplastic anemia in China was about 0.73 per one hundred thousand population.The risk factors,particularly virus infection,were associated with the onset of aplastic anemia. The pathogenesis of aplastic anemia was recognized as that the marrow failure was resulted mainly by autoimmunic T cell attack through lymphokine induced apoptosis.Aplastic anemia,an autoimmunic marrow failure rather than marrow failure syndromes,should be diagnosed based on marrow failure,T cell over function and exclusion of other similar diseases.Long enough intensive immunosuppressive therapy combined with hemopoietic stimulating factors was proven to be effective on aplastic anemia which might be cured.

Anemia is one of the most common complication and a negative prognostic factor of lymphoid neoplasma.Different cases with lymphoid neoplasma associated anemia(LNAA)have various pathogenesis such as hemorrhagic anemia, anemia of chronic disease,autoimmune hemolytic anemia and treatment related anemia, though they all have the same symptoms. So different regimens are given to different patients according to their pathogenesis of LNAA.

[Abstrct] Based on field studying at University of California, Los Angeles (UCLA),discussing with the teacher and student representatives and reviewing related literatures, we found that the suc-cessful experience of PBL at UCLA were small group teaching, the use of different sources of tutor, serious case preparation, and good hardware support. The major difficulties of PBL at UCLA were high teaching costs, unstable teacher group and high hardware requirements. Because of the cultural differ-ences between East and West, Chinese students are not good at active learning, so we recommended gradually carrying out PBL teaching in our country, making more PBL skills training to teachers and students, strengthening the hardware construction, seeking school administrative support, and giving timely repair according to the feedback of scientific research.

Abnormal epigenetics play important roles in the pathogenesis of myelodysplastic syndromes (MDS). DNA hypermeth-ylation is the most common epigenetic abnormality in MDS. Demethylating DNA hypermethylation may improve the quality of life of MDS patients and prolong their overall survival. Azacitidine and decitabine are the demethylating drugs approved for MDS treatment. These drugs showed clinical effects on all subgroups of MDS patients.

Myelodysplastic syndrome (MDS) comprises a heterogeneous group of malignant disorders.The diagnosis of MDS has always been the focus of attention of hematologists all over the world.From the FAB classification to 2008 WHO classification,the diagnosis of MDS has changed from completely morphological diagnosis to multi-parameter diagnosis.Nowadays,the diagnosis of MDS should involve multiple parameters concerning morphology,histochemistry,cytogenetics and immunology.

Objective To study the clinical and laboratory features of the patients with immunorelated pancytopenia(IRP).Methods The risk factors,manifestations,blood cell counts,bone marrow phenotypes,autoantibodies and immunosuppressive therapy response of 72 patients with IRP were analyzed.Then they were followed up for(6 ~36)months,to see their long-term outcome and the prognostic factors.Results The infection,anaphylaxis and pregnancy were highly suspected to be the risk factors of IRP.Most of these patients were with pancytopenia72.2%(52/72);91.7%(66/72)of them were anemic with large or normal MCV;75.0%(54/72)of them were with leukopenia;18.1%(13/72)of them had fever.Thrombocytopenia was common,but serious bleeding rare.68.1% of these cases were with normal or decreased bone marrow cellularities and increased normoblasts.They were all found to have positive results of bone marrow mononuclear cell Coombs test,negative results of routine hemolysis tests and no evidence of malignant clonal hematopoiesis.42.9%(18/42)of these patients had C3 decreased,and 19.0%(8/42)C4 decreased.Immunosuppressive therapy was administered to 72 IRP patients.The response rate at 36 months was 87.5%(28/32).Conclusion IRP is a blood disease induced by acquired autoantibodies mediated bone marrow inhibition or destruction.It manifests pancytopenia with relative higher percentages of reticulocytes and neutrophils,and responds well to immunosuppressive therapy.

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 explore the differences of clinical features and relationship between aplastic anemia paroxysmal nocturnal hemoglobinuria syndrome(AA PNH syndrome)and typical paroxysmal nocturnal hemoglobinuria(t PNH).Methods A case control study on the discrepancies of clinical and laboratory features between patients with AA PNH syndrome and t PNH was carried out.Results Compared with t PNH,AA PNH syndrome showed following features:①Lower frequencies of venous thrombosis,jaundice and enlarged liver or spleen.②Higher percentages of pancytopenia and bone marrow hypoplasia.③Lower percentages of positive hemolysis tests.The percentages of CD55 and CD59 of peripheral blood cells were not significantly different in most cases of both groups.④Immunoglobulins and subgroups of T lymphocytes were normal in cases of both groups.⑤Adrenocortical hormone was effective in cases of both groups.Conclusion AA PNH syndrome shares a same pathophysiology with t PNH;CD55 and CD59 tests can improve the diagnosis of AA PNH syndrome.