Background: Patients who received multiple transfusions of blood and blood products may produce antibodies against antigens of erythrocytes, leukocytes, platelets etc, resulting in many clinical implications. Objectives: To detect frequencies of antineutrophil antibodies in multitransfused patients at National Institute of Hematology and Blood Transfusion (NIHBT). Subjects and methods: The study was conducted on 30 multitransfused patients. Among them there were 12 with thrombocytopenia and 18 with aplastic anemia. Results: 6 cases had anti - neutrophil antibodies, of which 5 had more than 5 times of transfusion, 4 with aplastic anemia and 2 with thrombocytopenia. The sera were further tested with neutrophil panel, revealing 4 samples with anti - NA 1 (13.3%) and 1 sample with anti - NA2 (3.3%). The frequency of anti - neutrophil antibodies in multitransfused patients at IHBT in the study is 20%. Conclusion: Frequency of anti-NA1 was higher than anti-NA2 in multitransfused patients at NIHBT and directly proportional by frequency of NA1 and NA2 antigens in this group. The technical process to identify and classify antineutrophil antibodies in this study can be applied for patients who received multiple transfusions of blood and blood products in Viet Nam
Anemia ; Aplastic/ blood ; complications ; pathology ; Neutrophils

From 11/1995 to 4/1999 we treated by prednisone for 15 patients and CsA for 16 ones with idiopathic aplastic anemia. Results:- Prednison did not change significantly TCD3, TCD4 and TCD8 colony count comparing with pre-treatment (P>0.05).- CsA decreased clearly TCD3 colony count after 1st, 6th month (P<0,05). However, TCD4 colony count decreased not significantly (P>0.05). Conclusions: CsA has more suppressive effect on T-cell colonies, mainly TCDz3 and TCD8 than Prednisone and therefore it may be chosen to treat patients with idiopathic aplastic anemia without indication for bone marrow transplantation.
Anemia, Aplastic ; therapy ; blood ; therapeutics ; cyclosporine

Anemia, Aplastic ; therapy ; Fetal Blood ; transplantation ; Humans ; Mesenchymal Stromal Cells

OBJECTIVETo investigate the abnormalities of iron metabolism, the prevalence and risk factors of iron overload and clinical characteristics of patients with aplastic anemia (AA).

METHODSA cross-sectional study was conducted on 520 newly diagnosed AA patients.

RESULTSIron overload was observed in 66(13%) of 520 AA patients,in which a higher prevalence of iron overload was seen not only in patients with infections(19/86, 22%)than those without infections (47/434, 11%, P<0.01), but also in patients with hepatitis associated AA(HAAA) (6/22, 19%) than the idiopathic cases (60/488, 12%, P>0.05). Excluded the patients with infections and/or HAAA, 43 of 405(11%)cases had iron overload, including 14 of 248(6%) cases without history of blood transfusion and 29 of 157 patients (18%, P<0.01) with transfusion. In univariate analysis, higher levels of serum ferritin (SF), serum iron (SI) and transferrin saturation (TS) were mainly observed in adult male patients with severe AA (SAA) and significantly upward with increasing blood transfusion (P<0.01). No differences of soluble transferrin receptor (sTfR) were observed between adults and children, males and females, hepatitis and idiopathic AA. However, patients with infections had significantly lower level of sTfR (0.50 mg/L) than cases without infections (0.79 mg/L, P<0.01). The level of sTfR in SAA patients (0.70 mg/L) was only half of that in non-SAA (NSAA) (1.36 mg/L, P<0.01). Patients with increasing blood transfusion had significantly downward levels of sTfR (P<0.01). In multivariate analysis, more than 8 U blood transfusion (OR=10.52, P<0.01), adults (OR=3.48, P<0.01), males (OR=3.32, P<0.01) and infections (OR=2.09, P<0.01) were independent risk factors.

CONCLUSIONAA patients had higher iron burden and were high-risk populations occurring iron overload. The iron overload occurred in 18% of patients with blood transfusion and in 6% of patients without transfusion.

Anemia, Aplastic ; complications ; physiopathology ; Blood Transfusion ; Ferritins ; blood ; Hepatitis ; complications ; Humans ; Iron ; blood ; metabolism ; Iron Overload ; physiopathology ; Risk Factors

OBJECTIVEThrombocytopenic hemorrhage is one of the major appearance in pediatric hemorrhagic diseases, in which, idiopathic thrombocytopenic purpura (ITP) is the most common disease. Thrombocytopenia is the earliest phenomenon or the only one in certain phases of hemorrhagic diseases, such as ITP, aplastic anemia (AA) and myelodysplastic syndrome (MDS). By now, the pathogenesis of thrombocytopenia in different diseases has not been clearly determined. At present, it is very difficult to diagnose these diseases and estimate their prognosis with current clinical data. In this study, morphological characteristics and hematopoiesis function of bone marrow megakaryocyte in pediatric patients with ITP, AA and MDS were observed and the cause and mechanism of different thrombocytopenias were analyzed.

METHODSThere were 16 children with ITP, 17 with AA and 16 with MDS in this study. CD41 McAb immunohistochemical technique was used to detect micromegakaryocyte on bone marrow smears. Plasma clot culture and CD41 McAb immunohistochemical technique were used for the MK-colony forming assay. The colony formation rate of colony formation unit-megakaryocyte (CFU-MK) and burst formation unit-megakaryocyte (BFU-MK) were counted.

RESULTSThere was no statistical difference on the positive rates of micromegakaryocyte and type I lymphoid small micromegakaryocyte between groups of ITP and control. The number of micromegakaryocyte and the formation rates of CFU-MK in ITP group were significantly higher than those in control group. Among AA patients, the numbers of MK, micromegakaryocyte and the formation rates of CFU-MK, BFU-MK in vitro significantly decreased. There was no significant difference in the positive rate of micromegakaryocyte between groups of MDS and control, but the number of micromegakaryocyte and the positive rate of type I lymphoid micromegakaryocyte were significantly higher than those of control group. There was no statistical difference of the formation rate of CFU-MK between groups of MDS and control. But in 63% childhood patients, the formation rate of CFU-MK decreased, 25% increased,and 13% was normal; BFU-MK formation rate decreased significantly in MDS group.

CONCLUSIONOverproliferation of bone MKs may exist in most ITP patients. For obviating the nosogenetic factors, the normal MK releasing platelet could be easily found in the culture system. But the colony formation rate of MK decreased in a few patients with CITP. The abnormality of MK might be one of the reasons for thrombocytopenia in partial patients with ITP. Underproliferation of MKs may exist in AA, but no pathosishemogenesis was found. The dysfunction of early phase MK progenitor and stem cell might be the major reason for AA, but not the abnormality of hematopoietic microenvironment. There may be two kinds of megakaryocyte clones in bone marrow of children with MDS. One may be pathologic and potentially malignant micromegakaryocytes, the other may be the normal megakaryocytic precursors. The increase of pathologic MK resulted in abnormal development and maturation of MK in bone marrow. The change of megakaryopoiesis showed different in ITP, AA or MDS. Using bone marrow smear megakaryocyte counting, small micromegakaryocyte immunohistochemical detecting and the formation rate of bone marrow MK colony assay, the different thrombocytopenia can be diagnosed during the early stage of ITP, AA or MDS.

Adolescent ; Anemia, Aplastic ; blood ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Megakaryocytes ; pathology ; physiology ; Myelodysplastic Syndromes ; blood ; Purpura, Thrombocytopenic ; blood

This case was an acquired aplastic anemia patient who required a major operation for excision of a mediastinal mass. The authors previewed that the coagulation abnormalities would be developed due to major operation. Thus we decided to monitoring the coagulation function using the thromboelastography during the perioperative period and checked the complete blood count, concommitantly. The total blood volume lost during operation was 1800 ml, so we gave him a transfusion of 10 U's of platelet concentrate, 10 U's of pheretic platelet rich plasma and 5 U's of whole blood. The thromboelastography was a good guide that helped us to avoid excessive treatment of the coagulation abnormalities. We concluded that the thromboelastograhy was a reliable and effective monitoring system at the intraoperative coagulation management.
Anemia, Aplastic* ; Anesthesia, General ; Blood Cell Count ; Blood Platelets ; Blood Volume ; Humans ; Perioperative Period ; Platelet-Rich Plasma ; Thrombelastography

OBJECTIVETo study the expression of Foxp3 and NFAT1 protein in peripheral blood (PB) in children with aplastic anemia (AA) and their roles in the pathogenesis of AA.

METHODSThe expression levels of Foxp3 and NFAT1 protein of mononuclear cells in PB were measured by Western blot in 68 children with AA before and after treatment and in 60 normal children (control group). The correlation between Foxp3 and NFAT1 protein expression and the correlation of the Foxp3 and NFAT1 protein expression with blood Hb, WBC and platelet levels were analyzed.

RESULTSThe expression levels of Foxp3 and NFAT1 protein in PB in the acute phase in the AA group were significantly lower than in the control group (P<0.05). After treatment (recovery phase) the expression levels of Foxp3 and NFAT1 protein increased obviously compared with those in the acute phase (P<0.05). The Foxp3 protein level was positively correlated with the NFAT1 protein level (r=0.812, P<0.05). Both the Foxp3 and NFAT1 protein levels were positively correlated with blood Hb, WBC and platelet levels in children with AA in the recovery phase (r=0.537, 0.579, 0.655 respectively; P<0.05).

CONCLUSIONSThe Foxp3 and NFAT1 protein levels in PB are reduced in children with AA, suggesting that they are involved in the pathogenesis of AA. The measurement of Foxp3 and NFAT1 protein levels may be useful in the severity evaluation of AA.

Adolescent ; Anemia, Aplastic ; blood ; etiology ; Child ; Child, Preschool ; Female ; Forkhead Transcription Factors ; blood ; Humans ; Male ; NFATC Transcription Factors ; blood

OBJECTIVETo evaluate bone marrow hematopoietic cells genetic instability (BMHCGI) in patients with aplastic anemia (AA) and to explore its influence on immunosupressive therapy for AA and significance on late clonal hematologic disorders.

METHODSGenetic instability of bone marrow mononuclear cells (BMMNC) was measured by Comet assay. The relationship between bone marrow failure parameters and genetic instability results was evaluated. The reciprocity of genetic instability and treatment responses to immunosuppressive therapy (IST) was investigated.

RESULTSComet assay parameters \[tail moment (TM), olive TM (OTM), comet %\] of AA patients were significantly higher than that of control group (P < 0.05). There was no statistic correlation of comet parameters of severe AA (SAA) BM hematopoietic cells with age, gender and peripheral blood cell count (P > 0.05). For the treatment response rate at six months after IST there was no statistical difference between comet cells of < 21.64% and of >/= 21.64%, and so did between OTM < 1.58 and >/= 1.58 in SAA patients. IST had no effect on SAA BMHCGI, whereas, the Comet%, TM and OTM in SAA PR patients and Comet% in CR patients were significantly decreased than those before treatment. Comet parameters of two SAA patients were significantly increased before the development of clonal cytogenetic abnormalities.

CONCLUSIONSIncreased BMHCGI may be one of the elements in the pathogenetic mechanisms in AA. The genetic instability is irrelevant to the SAA patients overall response rate of IST at six months, but IST can alleviate the genetic instabilities in responded SAA patients.

Anemia, Aplastic ; therapy ; Blood Cell Count ; Bone Marrow Cells ; Humans ; Immunosuppression ; Pancytopenia

OBJECTIVETo evaluate the impact of recombinant human thrombopoietin (rhTPO) on short-term response of immunosuppressive therapy (IST) in patients with newly diagnosed acquired severe aplastic anemia (SAA).

METHODSThe clinical data of forty adult acquired SAA patients, who treated with IST combined with rhTPO, were retrospective analyzed and the hematologic recovery were compared with patients by the IST alone during the same period. The factors affecting the short-term response were also analyzed.

RESULTSAt 3 months after IST, both the total response rate and CR+GPR rate in rhTPO group were much higher than those in control group (75.0% vs 50.0%, P=0.022; and 17.5% vs 2.5%, P=0.025). At 6 months after IST, there was no difference of total hematologic response rate in rhTPO group and control group (77.5% vs 57.5%, P=0.058), while the CR+GPR rate was still higher in rhTPO group (45.0% vs 22.5%, P=0.033). The median time of platelet transfusion independence was much shorter in rhTPO group [33(0-90) vs 53(0-75) d, P=0.019]. Patients in rhTPO group needed less platelets transfusion support. The median platelet count in rhTPO group was 29(4-95)×10⁹/L at 3 months after IST, which was much higher than that in control group [29(4-95)×10⁹/L, P=0.006]. There was no significant difference regarding overall survival between the two groups (100.0% vs 91.0%, P=0.276).

CONCLUSIONrhTPO is effective in promoting platelet recovery and improving the hematopoietic response for SAA patients with IST.

Anemia, Aplastic ; Blood Platelets ; Humans ; Immunosuppression ; Platelet Count ; Platelet Transfusion ; Recombinant Proteins ; Retrospective Studies ; Thrombopoietin

Aplastic anemia is a serious hematological disorder characterized by pancytopenia and bone marrow hypocellularity. Pregnancy associated with aplastic anemia is fortunately uncommon considering the significant morbidity and mortality for both mother and the fetus. The risk to the mother is mainly in the form of hemorrhage and sepsis, while the fetus may suffer from growth restriction and even intrauterine death. Maternal mortality has been reported variously from 20% to 60%. The relationship between pregnancy and aplastic anemia remains controversial. We experienced a patient with severe aplastic anemia in pregnancy who was treated with supportive transfusion of red blood cells and platelets. Here, we present a case with a brief review of the literature.
Anemia, Aplastic ; Blood Platelets ; Bone Marrow ; Erythrocytes ; Fetus ; Hemorrhage ; Humans ; Maternal Mortality ; Mothers ; Pancytopenia ; Pregnancy ; Sepsis