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Hematology AND Aplastic anemia [keywords]
- A rare association of celiac disease and aplastic anemia: case report of a child and review of literature. [JOURNAL ARTICLE]
- Pediatr Dev Pathol 2014 Jul 30.
Abstract An association between severe aplastic anemia and other autoimmune diseases is rare and has been described in adults for eosinophilic fasciitis, thymomas, systemic lupus erythematosus and thyroid disorders. Herein we report a case of celiac disease on gluten free diet, who presented with progressive pallor, fever and weakness of 1 month duration. On investigation, he had pancytopenia, which on subsequent evaluation revealed aplastic anemia. Association between aplastic anemia and celiac disease has rarely been reported. To the best of authors knowledge, only single pediatric case of celiac disease associated with aplastic anemia has been published. This is the second report to suggest such an association in children.
- Clinical and Quality Evaluation of Red Blood Cell Units Collected Via Apheresis Versus Those Obtained Manually. [JOURNAL ARTICLE]
- Lab Med 2014; 45(3):238-243.
To evaluate the impact of collection procedure on the in vitro quality of red blood cells (RBC), we studied 30 units of apheresis-prepared RBC (ARBC) and 30 units of manually collected RBC (MRBC). We performed assays on day 1 and day 21 of the study, evaluating red cell mass volume (RCM); rate of hemolysis; pH, and levels of sodium, potassium, adenosine triphosphate (ATP), 2,3-diphosphoglycerate (2,3-DPG) and glucose. Eight patients with aplastic anemia received RBC transfusions of both components and their post-transfusion hematocrit (HCT) levels were compared. On day 21, we observed a significant drop of sodium and glucose levels in the ARBC group, compared with the MRBC group (P <.05). ARBC group demonstrated higher RCM that provided significantly higher HCT values to our group of anemic patients (P <.05). Hemolysis was significantly lower in the ARBC group, compared with the MRBC group (P <.05). At day 21, both groups had no detectable 2,3-DPG. Specimens from both groups retained ATP in sufficiently healthy amounts. The ARBC group demonstrated higher RCM and lower hemolysis levels compared with the MRBC group.
- Use of mesenchymal cells to modulate immune suppression and immune reconstruction in a patient with aplastic anemia complicated by invasive sino-orbital aspergillosis. [Journal Article]
- Turk J Haematol 2014 Jun; 31(2):181-3.
Cultured human bone marrow mesenchymal cells (MSCs) have immunomodulatory and tissue regenerative properties. This report summarizes the result of post-transplant treatment with MSCs of a 26-year-old patient with aplastic anemia complicated by invasive sino-orbital aspergillosis. The patient was treated with MSCs to benefit from the dual effects of MSCs in immune reconstitution: suppression against alloreactive T cells and facilitation of the re-engraftment process. The patient did not develop acute or chronic graft-versus-host disease. The aspergillus infection healed completely. The engraftment failure was also ended without any complications. During his last visit in his fourth year after transplantation, the patient was in hematological remission. Human bone marrow-derived MSCs seem to have an important role in preventing or overcoming immunological complications in patients who undergo stem cell transplantation.
- Paroxysmal Nocturnal Hemoglobinuria Clones in Children with Acquired Aplastic Anemia: A Multicentre Study. [JOURNAL ARTICLE]
- PLoS One 2014; 9(7):e101948.
A multicentre study evaluating the presence of glycosil phosphatidyl-inositol (GPI)-negative populations was performed in 85 children with acquired aplastic anemia (AA). A GPI-negative population was observed in 41% of patients at diagnosis, 48% during immune-suppressive therapy (IST), and 45% in patients off-therapy. No association was found between the presence of a GPI-negative population at diagnosis and the response to IST. In addition, the response rate to IST did not differ between the patients who were GPI-positive at diagnosis and later developed GPI-negative populations and the 11 patients who remained GPI-positive. Two patients with a GPI-negative population >10%, and laboratory signs of hemolysis without hemoglobinuria were considered affected by paroxysmal nocturnal hemoglobinuria (PNH) secondary to AA; no thrombotic event was reported. Excluding the 2 patients with a GPI-negative population greater than 10%, we did not observe a significant correlation between LDH levels and GPI-negative population size. In this study monitoring for laboratory signs of hemolysis was sufficient to diagnose PNH in AA patients. The presence of minor GPI-negative populations at diagnosis in our series did not influence the therapeutic response. As occasionally the appearance of a GPI-negative population was observed at cyclosporine (CSA) tapering or AA relapse, a possible role of GPI-negative population monitoring during IST modulation may need further investigation.
- [Clinical Observation of Haploidentical-Hematopoietic Stem Cell Transplantation Combined with Human Umbilical Cord-derived Mesenchymal Stem Cells for Severe Aplastic Anemia-II]. [English Abstract, Journal Article]
- Zhongguo Shi Yan Xue Ye Xue Za Zhi 2014 May; 22(3):774-8.
This study was purposed to investigate the efficacy and safety of haploidentical hematopoietic stem cells (allo-HSCT) transplantation combined with human umbilical cord-derived mesenchymal stem cell infusion (hUC-MSC) for severe aplastic anemia-II (SAA-II). Eight SAA-II patients received haploidentical allo-HSCT, the G-CSF mobilized peripheral hematopoietic stem cells and bone marrow haploidentical hematopoietic stem cells were selected as graft, the human umbilical cord-derived mesenchymal stem cells (hUC-MSC) were infused as the third party. Conditioning regimen consisted of rabbit anti-thymiclymphocytes protein(ATG), cyclophosphamide(CTX) and fludarabine(Flu). For two patients out of 8 SAA-II patients the conditioning regimen was combined with busulfan(BU). The graft versus host disease(GVHD) was prevented with CSA, MTX, ATG, CD25 and mycophenolate mofetial. The results showed that the average number of nucleated cells were 9.13×10(8)/kg, and number of CD34(+)cells were 3.76×10(6)/ kg. All the 8 SAA-II patients achieved hematopoietic reconstitution. The average time of neutrophils count>0.5×10(9)/L was 11.9 days, and average time of Plt level >20×10(9)/L was 14.6 days. The incidence of acute GVHD of I-II grade was 25%, and that of III-IVgrade was 12.5%, the transplantation-related mortality was 25%. It is concluded that haploidentical allo-HSCT combined with umbilical cord MSC infusion is an effective approach to cure SAA.
- [Effects of rapamycin on biological characteristics of bone marrow mesenchymal stem cells from patients with aplastic anemia]. [English Abstract, Journal Article]
- Zhongguo Shi Yan Xue Ye Xue Za Zhi 2014 May; 22(3):762-6.
This study was aimed to investigate the effects of rapamycin on biological function and autophagy of bone marrow mesenchymal stem cells (BM-MSC) from patients with aplastic anemia so as to provide experimental basis for the clinical treatment of aplastic anemia (AA) with rapamycin. BM-MSC were treated with different concentrations of rapamycin (0, 10, 50, 100 nmol/L) for 48 h, the expression of LC3B protein was detected by Western blot to observe the effect of rapamycin on cell autophagy; cell apoptosis and cell cycles were detected by flow cytometry; the proliferation of BM-MSC of AA patients was measured by cell counting kit-8; the adipogenic differentiation of BM-MSC were tested by oil red O staining after adipogenic induction for 2 weeks; the adipogenic related genes (LPL, CFD, PPARγ) were detected by real-time PCR. The results showed that the proliferation and adipogenesis of BM-MSC of AA patients were inhibited by rapamycin. Moreover, the autophagy and apoptosis of BM-MSC were increased by rapamycin in a dose-dependent way.Rapamycin arrested the BM-MSC in G0/G1 phase and prevented them into S phase (P < 0.05). It is concluded that rapamycin plays an critical role in inhibiting cell proliferation, cell cycles, and adipogenesis, these effects may be related with the autophagy activation and mTOR inhibition resulting from rapamycin.
- [Expression of PPARγ in bone marrow of model mice of immune-mediated aplastic anemia mice model]. [Journal Article]
- Zhonghua Xue Ye Xue Za Zhi 2014 Jun 14; 35(6):553-5.
- The polymorphisms of human leukocyte antigen loci may contribute to the susceptibility and severity of severe aplastic anemia in Chinese patients. [JOURNAL ARTICLE]
- Hum Immunol 2014 Jun 27.
The human leukocyte antigen (HLA) system has been reported to be involved in the development of aplastic anemia (AA). We compared and analyzed HLA-A, B, C, DRB1 and DQB1 alleles in 96 Chinese severe AA (SAA) patients to those in 600 healthy people chosen randomly from the China Marrow Donor Program to investigate the association of HLA class I and II allele polymorphisms with disposition of SAA and its severity degree in Chinese population. The DNA of patients was extracted and HLA high-resolution genotyping was conducted using polymerase chain reaction-sequence based typing technique. The gene frequencies of A∗02:01, A∗02:06, B∗13:01, DRB1∗07:01, DRB1∗09:01, DRB1∗15:01 and DQB1∗06:02 in SAA patients were significantly higher than in controls (all P<0.05), while the allelic frequencies of A∗02:07, A∗11:01 and B∗40:01 were notably lower in SAA patients than those in the controls (P=0.001, 0.002, 0.005, respectively). Comparison among different severity of SAA groups exhibited significant increases of DRB1∗15:01 (P=0.027) and DQB1∗06:02 (P=0.013), but obviously lower frequencies of B∗46:01 (P=0.023) and DRB1∗09:01 (P=0.020) in non-VSAA patients than in VSAA patients. Thus, our results identified several risk and protective HLA alleles for Chinese SAA patients. Moreover, DRB1∗15:01, DQB1∗06:02, B∗46:01 and DRB1∗09:01 may be associated with severity of SAA.
- CD8+HLA-DR+ T cells are increased in patients with severe aplastic anemia. [Journal Article]
- Mol Med Rep 2014 Sep; 10(3):1252-8.
The aim of the present study was to investigate the number and function of CD8+HLA-DR+ cells, which are considered to be activated cytotoxic T lymphocytes (CTLs), in peripheral blood to further examine the pathogenesis of severe aplastic anemia (SAA). Thirty-eight patients with SAA were included in the present study. Patients were screened for paroxysmal nocturnal hemoglobinuria by flow cytometry using anti-CD55 and anti-CD59 antibodies. The number of CD8+HLA-DR+ T cells was measured by three-color flow cytometry using anti-CD8-peridinin chlorophyll, anti-CD3-fluorescein isothiocyanate (FITC) and anti-HLA-DR-FITC antibodies. The expression of perforin, granzyme B, tumor necrosis factor-β (TNF-β) and FasL in CD8+HLA-DR+ T cells was detected by flow cytometry with the appropriate monoclonal antibodies. Total RNA was prepared from purified CD8+HLA-DR+ cells of healthy controls and SAA patients, and then polymerase chain reaction (PCR) was performed. Apoptosis of CD8+HLA-DR+ cells was detected by flow cytometry following staining with Annexin V. The proportion of CD8+HLA-DR+ T cells was analyzed by flow cytometry in peripheral blood and was identified to be significantly higher in untreated SAA than in remission patients and in the controls. The expression of perforin, granzyme B, TNF-β and FasL in CD8+HLA-DR+ T cells was analyzed by flow cytometry and PCR, which revealed increased expression in the untreated SAA group compared with that in the control group. Furthermore, the apoptosis of CD3- bone marrow cells from normal individuals was enhanced following co-culture with CD8+HLA-DR+ T cells from untreated SAA patients. In conclusion, the present study demonstrated that CD8+HLA-DR+ T cells may contribute to bone marrow failure in SAA.
- Fas/FasL in the immune pathogenesis of severe aplastic anemia. [Journal Article]
- Genet Mol Res 2014; 13(2):4083-8.
Fas/FasL protein expression of bone marrow hematopoietic cells was investigated in severe aplastic anemia (SAA) patients. Fas expression was evaluated in CD34(+), GlycoA(+), CD33(+), and CD14(+) cells labeled with monoclonal antibodies in newly diagnosed and remission SAA patients along with normal controls. FasL expression was evaluated in CD8(+) cells in the same manner. In CD34(+) cells, Fas expression was significantly higher in the newly diagnosed SAA group (46.59 ± 27.60%) than the remission (6.12 ± 3.35%; P < 0.01) and control (8.89 ± 7.28%; P < 0.01) groups. In CD14(+), CD33(+), and GlycoA(+) cells, Fas levels were significantly lower in the newly diagnosed SAA group (29.29 ± 9.23, 46.88 ± 14.30, and 15.15 ± 9.26%, respectively) than in the remission (47.23 ± 31.56, 67.22 ± 34.68, and 43.56 ± 26.85%, respectively; P < 0.05) and normal control (51.25 ± 38.36, 72.06 ± 39.88, 50.38 ± 39.88%, respectively; P < 0.05) groups. FasL expression of CD8(+) cells was significantly higher in the newly diagnosed SAA group (89.53 ± 45.68%) than the remission (56.39 ± 27.94%; P < 0.01) and control (48.63 ± 27.38%; P <0.01) groups. No significant differences were observed between the remission and control groups. FasL expression in CD8(+) T cells was significantly higher in newly diagnosed patients, and CD34(+), CD33(+), CD14(+), and GlycoA(+) cells all showed Fas antigen expression. The Fas/FasL pathway might play an important role in excessive hematopoietic cell apoptosis in SAA bone marrow. Furthermore, CD34(+) cells are likely the main targets of SAA immune injury.