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

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

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

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

OBJECTIVE: Pregnancy-associated aplastic anemia remains a rare occurrence. The aim of this study was to examine the maternal and fetal outcomes of pregnancy-associated aplastic anemia treated with supportive care. METHODS: From January 1995 to December 2004, a total of 14 women newly diagnosed with pregnancy-associated aplastic anemia were recruited for the study. RESULTS: Eleven (78%) of the 14 women were diagnosed with pregnancy-associated aplastic anemia during the second or third trimester. There were eight severe cases; three of which were diagnosed at the initial presentation. All 14 women had conservative management with transfusions but not specific immunological or hormonal therapies during pregnancy. Blood transfusions were performed prenatally in seven mothers and perinatally in 13. Of the 12 patients eligible for follow-up, one achieved complete remission and another eight showed partial remission after delivery. During the follow up period, there was no case of maternal-fetal death in our series. The pregnancies were continued uneventfully in most cases. CONCLUSIONS: This study demonstrated favorable maternal and neonatal outcomes with transfusion support alone for pregnancy-associated aplastic anemia. Therefore, pregnancy continuation with meticulous blood support should be considered, rather than therapeutic termination, for women with pregnancy-associated aplastic anemia.
Anemia, Aplastic* ; Blood Transfusion ; Female ; Follow-Up Studies ; Humans ; Mothers ; Pregnancy ; Pregnancy Trimester, Third ; Prognosis

Aplastic anemia is a rare complication of pregnancy that is associated with significant maternal and fetal morbidity and mortality. The relationship between aplastic anemia and pregnancy is not yet clear. Aplastic anemia in pregnancy is difficult to manage because the risk of bleeding and infection is high, especially in refractory to platelet transfusion. There is no therapeutic guide-line and prognosis for aplastic anemia in pregnancy refractory to platelet transfusion. We recently experienced one case of aplastic anemia in pregnancy refractory to platelet transfusion without no peripartum complication. So we report this case with brief review of the literature.
Anemia, Aplastic* ; Blood Platelets* ; Hemorrhage ; Mortality ; Peripartum Period ; Platelet Transfusion* ; Pregnancy* ; Prognosis

Aplastic anemia is a hematopoietic disorder characterized by marked reduction or absence of erythoid, granulocytic, and megakariocytic cells in the bone marrow with resultant pancytopenia. To control of infection & bleeding secondary to leukopenia and thrombocytopenia, the inflammatory lesions in oral & maxillofacial area should be removed. MATERIALS AND METHOD: The extractions were performed on 21 patients with severe aplastic anemia. The initial, pre-operative and postoperative CBCs were checked up. Amount and kind of transfused platelet in each patient and increment of platelet level were recorded. The complications were documented. RESULT: A mean of 2.9 teeth were extracted from each patient(ranging between 1 and 13). Furthermore, surgical extractions including ostectomy and odontectomy of the third molar were performed on 11 patients. The preoperative WBC levels presented between 600/muL and 5000/muL(mean 2376/muL). The WBC values decreased by an average of 145/muL per patient after extractions had been performed. The teeth of 16 patients were extracted under 10.0g/dL, and the mean change in postoperative hemoglobin levels in comparison with preoperative hemoglobin levels was -0.06 per patient. The initial platelet levels were between 1000/(L and 81,000/muL(mean 20,174/muL). In five patients, extractions were performed with platelet levels less than 50,000/muL. CONCLUSION: The results suggest that more active and preventive treatments in the oral and maxillofacial area are possible and are necessary to remove the infectious foci on the patients with severe aplastic anemia. We report the results of our experiences and literature reviews in treatment of the patients with severe aplastic anemia in our department.
Anemia, Aplastic* ; Blood Platelets ; Bone Marrow ; Hemorrhage ; Humans ; Leukopenia ; Molar, Third ; Pancytopenia ; Surgery, Oral* ; Thrombocytopenia ; Tooth