Changes and clinical significance of erythrocyte lifespan in megaloblastic anemia / 中华内科杂志

Objective: To investigate the lifespan of erythrocytes in megaloblastic anemia (MA) patients. Methods: A prospective cohort study analysis. Clinical data from 42 MA patients who were newly diagnosed at the Department of Hematology, Lanzhou University Second Hospital from January 2021 to August 2021 were analyzed, as were control data from 24 healthy volunteers acquired during the same period. The carbon monoxide breath test was used to measure erythrocyte lifespan, and correlations between erythrocyte lifespan and laboratory test indexes before and after treatment were calculated. Statistical analysis included the t-test and Pearson correlation. Results: The mean erythrocyte lifespan in the 42 newly diagnosed MA patients was (49.05±41.60) d, which was significantly shorter than that in the healthy control group [(104.13±42.62) d; t=5.13,P=0.001]. In a vitamin B12-deficient subset of MA patients the mean erythrocyte lifespan was (30.09±15.14) d, and in a folic acid-deficient subgroup it was (72.00±51.44) d, and the difference between these two MA subsets was significant (t=3.73, P=0.001). The mean erythrocyte lifespan after MA treatment was (101.28±33.02) d, which differed significantly from that before MA treatment (t=4.72, P=0.001). In MA patients erythrocyte lifespan was positively correlated with hemoglobin concentration (r=0.373), and negatively correlated with total bilirubin level (r=-0.425), indirect bilirubin level (r=-0.431), and lactate dehydrogenase level (r=-0.504) (all P<0.05). Conclusions: Erythrocyte lifespan was shortened in MA patients, and there was a significant difference between a vitamin B12-deficient group and a folic acid-deficient group. After treatment the erythrocyte lifespan can return to normal. Erythrocyte lifespan is expected to become an informative index for the diagnosis and treatment of MA.

Harm Avoidance is Correlated with the Reward System in Adult Patients with Attention Deficit Hyperactivity Disorder: A Functional Magnetic Resonance Imaging Study

Objective@#Hypoactivity in the reward system among patients with attention deficit hyperactivity disorder (ADHD) is a well-known phenomenon. Whether the activity in the reward pathway is related to harm avoidance, such as in sensitivity to punishment, is unclear. Evidence regarding the potential difference between ADHD patients and controls in terms of this association is scarce. @*Methods@#Event-related functional magnetic resonance imaging was conducted on subjects performing the Iowa gambling test. Fourteen adults with ADHD and 14 controls were enrolled in the study. @*Results@#Harm avoidance was found to be positively correlated with the activities of the bilateral orbitofrontal cortex and right insula in individuals with ADHD. A group difference was also confirmed. @*Conclusion@#Understanding the roles of harm avoidance and brain activation during risk tasks is important.

Diagnostic value of spiral CT arterial enhancement fraction in colorectal cancer staging / 肿瘤研究与临床

Objective:To explore the diagnostic value of spiral CT arterial enhancement fraction (AEF) in clinicopathological staging of colorectal cancer.Methods:The clinical data of 151 newly diagnosed colorectal cancer patients (colorectal cancer group) and 86 patients who underwent physical examination with no history of colorectal disease (the control group) admitted to Shanxi Province Cancer Hospital from September 2019 to May 2021 were retrospectively analyzed. All research subjects received whole abdominal spiral CT plain scan and three-phase enhanced scan, and the obtained images were transferred to the Siemens Syngo.via post-processing workstation for post-processing to obtain the AEF color map and its quantitative parameter AEF value. The correlation of AEF value with colorectal cancer stage and Ki-67 was analyzed, and its diagnostic performance for clinicopathological staging of colorectal cancer was evaluated by using receiver operating characteristic curve.Results:The AEF values ??of patients at stage Ⅰ, Ⅱ, Ⅲ, and Ⅳ colorectal cancer were (72±9)%, (65±11)%, (60±12)%, and (52±10)%, respectively, and the difference was statistically significant ( F = 22.15, P < 0.001). The AEF values ??of the control group and the colorectal cancer group were (85±6)% and (62±12)%, respectively, and the difference in AEF value of both groups was statistically significant ( t = 18.66, P < 0.001). The clinicopathological stage of colorectal cancer was negatively correlated with AEF value ( r = -0.548, P < 0.05). Taking the control group as the standard, the area under the curve (AUC) of AEF value for the diagnosis of colorectal cancer stage Ⅰ, Ⅱ, Ⅲ and Ⅳ were 0.866 (the sensitivity was 62.50%, the specificity was 96.51%) and 0.928 (the sensitivity was 76.74%, the specificity was 98.84%), 0.981 (the sensitivity was 92.68%, the specificity was 91.86%) and 1.000 (the sensitivity was 100.00%, the specificity was 98.84%), and the difference in diagnostic efficiency was statistically significant ( P < 0.05). The AEF value of colorectal cancer was negatively correlated with Ki-67 ( r = -0.537, P < 0.05). The Ki-67 positive index of patients at stage Ⅰ, Ⅱ, Ⅲ, and Ⅳ colorectal cancer was (55±12)%, (67±10)%, (73±12)%, and (79±11)%, respectively, and the difference was statistically significant ( F = 11.94, P < 0.001); the Ki-67 positive index of patients at stage Ⅳ was higher than those at stage Ⅰ+Ⅱ ( t = 3.45, P = 0.001); the clinicopathological stage of colorectal cancer was positively correlated with Ki-67 ( r = 0.393, P < 0.001). Conclusions:AEF quantitative color map and AEF value can reflect the characteristics of arterial and venous blood supply in colorectal cancer lesions. AEF value analysis before surgery is helpful for the clinicopathological staging of colorectal cancer.

Altered Auditory P300 Performance in Parents with Attention Deficit Hyperactivity Disorder Offspring

OBJECTIVE: Altered event-related potential (ERP) performances have been noted in attention deficit hyperactivity disorder (ADHD) patients and reflect neurocognitive dysfunction. Whether these ERP alterations and correlated dysfunctions exist in healthy parents with ADHD offspring is worth exploring. METHODS: Thirteen healthy parents with ADHD offspring and thirteen healthy controls matched for age, sex and years of education were recruited. The auditory oddball paradigm was used to evaluate the P300 wave complex of the ERP, and the Wechsler Adult Intelligence Scale-Revised, Wisconsin Card Sorting Test, and continuous performance test were used to measure neurocognitive performance. RESULTS: Healthy parents with ADHD offspring had significantly longer auditory P300 latency at Fz than control group. However, no significant differences were found in cognitive performance. CONCLUSION: The presence of a subtle alteration in electro-neurophysiological activity without explicit neurocognitive dysfunction suggests potential candidate of biological marker for parents with ADHD offspring.

Effect of Shexiang Tongxin Dropping Pills () on the Immediate Blood Flow of Patients with Coronary Slow Flow / 中国结合医学杂志

OBJECTIVE@#To observe the immediate effect and safety of Shexiang Tongxin dropping pills (, STDP) on patients with coronary slow flow (CSF), and furthermore, to explore new evidence for the use of Chinese medicine in treating ischemic chest pain.@*METHODS@#Coronary angiography (CAG) with corrected thrombolysis in myocardial infarction (TIMI) frame count (CTFC) was applied (collected at 30 frames/s). The treatment group included 22 CSF patients, while the control group included 22 individuals with normal coronary flow. CSF patients were given 4 STDP through sublingual administration, and CAG was performed 5 min after the medication. The immediate blood flow frame count, blood pressure, and heart rate of patients before and after the use of STDP were compared. The liver and kidney functions of patients were examined before and after treatments.@*RESULTS@#There was a significant difference in CTFC between groups (P0.05).@*CONCLUSIONS@#The immediate effect of STDP in treating CSF patients was apparent. This medication could significantly improve coronary flow without affecting blood pressure or heart rate. Our findings support the potential of Chinese medicine to treat ischemic chest pain.

Visualization of proliferation activity of luminescent bacteria in different physiological media / 军事医学

Objective To investigate the relationship between the number of colonies and the luminous intensity of two kinds of platelets contamination bacteria,Escherichia coli(ECO)and Pseudomonas aeruginosa(PAE)in single platelets, plasma and phosphate buffered salines(PBS), and to explore their growth and proliferation in different biological fluid media.Methods The two luminescent bacteria were constructed before the correlations between the number of colonies and the luminous intensity in different media in vitro were observed with a small animal bioluminescence imaging instrument, and correlation analysis was carried out.By recording the luminous intensity at serial time points in different media, a growth curve was drown to reflect the proliferation of bacteria.Results There was a significant positive correlation between the number of colonies and luminous intensity of ECO and PAE in single platelets,plasma and PBS.The larger the number of colonies was,the stronger the luminous intensity was.The results indicated that single platelets significantly inhibited the proliferation of ECO and PAE,and that plasma also had some inhibitory effect,but not so strong.Conclusion For the two luminous bacteria,luminous intensity can represent the growth and proliferation of bacteria.Single platelets have obvious inhibitory effect on the growth of ECO and PAE, and the plasma also has some inhibitory effect, but the effect is not so strong as that of single platelets.

Relationship between prostatic hyperplasia and serum 25-hydroxy vitamin D level in elderly men / 中华老年医学杂志

Objective To explore the relationship between serum 25-hydroxy vitamin D level and prostatic hyperplasia in elderly men.Methods Totally 95 male patients aged over 60 years were included.Blood pressure,body weight,body height,body mass index (BMI) were measured and calculated.Venous blood samples were obtained to determine fasting serum levels of 25-hydroxy vitamin D3 and blood glucose (FBG),total cholesterol (TC),triglycerides (TG),low-density lipoprotein cholesterol (LDL-C),high-density lipoprotein cholesterol (HDL-C),calcium and prostate specific antigen (PSA),total prostate volume(PV) and annual prostate growth rate were measured and calculated by ultrasound.Results The serum 25 (OH) D3 levels were varied from 12.1 nmol/L to 83.9 nmol/L,with an average of (35.5±15.2) nmol/L in elderly male patients.PV growth rate were significantly lower in elderly men with 25 (OH) D3 ＞ 50 nmol/L than in elderly men with 25 (OH) D3≤50nmol/L[(31.5± 6.0) mlvs.(39.9 ± 14.5) ml,(0.4± 0.2) ml/yvs.(0.5 ± 0.4) ml/y,P＜0.001 or 0.01].PV was negatively correlated with serum 25-hydroxy vitamin D3 level (r=-0.207,P＜0.05),and positively correlated with BMI and PSA (r=0.297,0.958,P＜0.05 or and P＜0.001).While annual prostate growth rate was positively correlated with BMI and PSA (r=0.316,0.464,P＜0.01 or ＜0.001),and positively correlated with serum 25-hydroxy vitamin D3 level,but the difference was not statistically significant (P＞0.05).Conclusions Low serum 25-hydroxy vitamin D3 level may play a role in the pathogenesis of prostatic hyperplasia.

The association between insulin resistance and prostatic hyperplasia / 中华老年医学杂志

Objective To evaluate the relationship between insulin resistance and benign prostatic hyperplasia (BPH).Methods Totally 150 male patient from the Department of Geriatrics in Peking University Hospital were included in this study.Blood pressure,body weight,body height,body mass index (BMI) were measured and calculated.Biochemical analyses including serum fasting levels of insulin(FINS),glucose,total cholesterol,triglycerides,low-density lipoprotein cholesterol,high density lipoprotein cholesterol,and prostate-specific antigen (PSA) were performed.Total prostate volume (PV) were measured by ultrasound.Results PV and annual prostate growth rate were more increased in insulin resistance group(40 cases) compared with insulin sensitivity group(110 cases) (t=2.91,3.71 respectively,both P＜0.01).Along with the levels of FINS,HOMA-IR and PSA were increased,the prostate volume was enhanced (t=-3.02,-2.88,-2.84 respectively;all P ＜0.05).PV was positively correlated with insulin resistance,serum fasting insulin and PSA (r=0.16,0.16,0.35;all P＜0.05),while annual prostate growth rate was positively related with insulin resistance,serum fasting insulin,PSA and BMI (r =0.22,0.21,0.24,0.19 ; all P ＜ 0.05).Conclusions Insulin resistance and fasting insulin plays roles in the pathogenesis of prostatic hyperplasia.

Contribution of the Akt2 gene to type 2 diabetes in the Chinese Han population / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>The Akt2 protein kinase is thought to be a key mediator of the insulin signal transduction process. Akt2 is suggested to play a role in glucose metabolism and the development or maintenance of proper adipose tissue and islet mass. In order to determine whether the Akt2 gene plays a role in the pathogenesis of type 2 diabetes characterized by insulin resistance, and to further identify if variations in this gene have a relationship with type 2 diabetes, we sequenced the entire coding region and splice junctions of Akt2 and made a further case-control study to explore the association between single-nucleotide polymorphisms (SNPs) in this gene and type 2 diabetes in the Chinese Han population.</p><p><b>METHODS</b>We selected 23 probands with a type 2 diabetic pedigree whose family members' average onset age was within 25 to 45 years old. The body mass index of all the participants was lower than 28 kg/m(2) and all of them were insulin-resistant (the fasting insulin level > 100 pmol/L or 16 µIU/ml). The entire coding region and splice junctions of Akt2 were directly sequenced in these 23 probands. SNPs with a frequency of minor allele over 20 percent were selected to be further studied in a case-control study. We chose 743 non-diabetic subjects as the control group and 742 type 2 diabetic patients as the case group. All these subjects were genotyped. A Snapshot Technology Platform (Applied Biosystems) was used for genotyping.</p><p><b>RESULTS</b>The Akt2 genes from all 23 subjects were successfully sequenced. We did not identify any mutation in the type 2 diabetic pedigree. Two SNPs were identified, 13010323T > C and 13007939G > T. 13010323T > C was in intron 9, which was the location of rs2304188 reported in Genbank. Its minor allele frequency was 13.04%. 13007939G > T was in the 3'-untranslated region (UTR) of exon 14, which was the location of rs2304186 reported in Genbank. Its minor allele frequency was 34.78%. The allele frequency of rs2304188 and rs2304186 were consistent with the frequency reported in Genbank. In the case-control study with 742 patients and 743 controls, there was no significant difference between the two groups for the allele frequency of rs2304186 (odd ratio: 0.96, 95% confidence interval: 0.82 - 1.12, P = 0.597).</p><p><b>CONCLUSIONS</b>The Akt2 gene is not a major cause of diabetes in a non-obese Chinese Han population characterized by insulin resistance. There is no significant relationship between rs2304186 and type 2 diabetes in the Chinese Han population.</p>

Effects of concentration of cyclosporine A on the early response to immunosuppressive therapy in severe aplastic anemia / 中华血液学杂志

<p><b>OBJECTIVE</b>To evaluate the effects of cyclosporine A (CsA) whole-blood concentration on the early response to immunosuppressive therapy (IST) in severe and very severe aplastic anemia (SAA/VSAA).</p><p><b>METHODS</b>Ninety SAA/VSAA patients treated with rabbit antithymocyte globulin (ATG) plus CsA as first line therapy in our hospital were retrospectively analysed. CsA levels between the response group and non-response group, and response rates of patients with variant CsA levels were compared respectively.</p><p><b>RESULTS</b>(1) There was no significant difference in the beginning unmodified CsA blood concentration between IST responded and non-responded SAA/VSAA patients. The beginning unmodified C(0) 133.91 ug/L in IST 2-month responders was higher than that of 49.9 ug/L in non-responded SAA patients (P = 0.009); (2) The mean CsA C(0) and C(2) levels during the third month following IST were significantly different in responders and non-responders(197.52 µg/L vs 161.49 µg/L, P = 0.024, and 738.76 µg/L vs 615.46 µg/L, P = 0.009), and no significant difference in other periods of IST (P > 0.05); (3) The response rate (87.5%) was significantly higher in patients with CsA C(0) ≥ 200µg/L the third month following IST than those of 55.6% in patients with CsA C(0) 150 - 200 µg/L (P = 0.023) and 59.3% in patients with CsA C(0) < 150 µg/L (P = 0.046), respectively. The response rate was significantly higher of C(2) ≥ 700 µg/L group than that of C(2) < 700 µg/L group (80.5%vs 55.3%, P = 0.012).</p><p><b>CONCLUSIONS</b>The CsA concentration related to the early IST response. The third month CsA concentrations was the most important for the response and maintaining CsA levels with C(0) ≥ 200 µg/L and C(2) ≥ 700 µg/L may improve the response to IST in SAA/VSAA.</p>

Relationship between metabolic syndrome and benign prostatic hyperplasia / 中华老年医学杂志

ObjectiveTo evaluate the relationship between metabolic syndrome (MS) and benign prostatic hyperplasia (BPH).MethodsTotally 101 male patients with MS and 117 male patients without MS were enrolled from September 2008 to January 2010 in the department of geriatrics of Peking University People's Hospital.The MS diagnosis was made according to the criteria by Chinese Diabetes Society (2004).Blood pressure,body weight,body height,body mass index (BMI) were measured and calculated.Biochemical analyses including serum fasting blood glucose (FBG),total cholesterol ( TC),triglycerides (TG),low-density lipoprotein cholesterol (LDL-C),high-density lipoprotein cholesterol (HDL-C),and prostate-specific antigen (PSA) were performed.Total prostate volume (PV) were measured by ultrasound.ResultsPV in patients with MS[(39.7±21.1)m1] was significantly larger than in patients without MS[(30.7±9.3)ml] (P＜ 0.05).There was no difference in PSA level between groups (P＞ 0.05).PV were positively correlated with BMI(r=0.27,P=0.019),hypertension duration(r=0.37,P=0.01),PSA(r=0.51,P=0.001),FBG(r=0.24,P=0.013) and systolic blood pressure(SBP)(r=0.20,P=0.047),but negatively correlated with HDL-C ( r = - 0.24,P = 0.013 ).Conclusions BMI,hypertension duration,PSA,FBG,SBP,and lower HDL-C may play roles in the pathogenesis of prostatic hvperplasia.

Association between T245G polymorphisms in the osteoprotegerin gene and bone mineral density in elderly individuals / 中国组织工程研究

BACKGROUND: As a primary clinical predictor of fracture risk, bone mineral density (BMD) is partly genetically determined. Osteoprotegerin (OPG) is one important candidate gene in the pathogenesis of osteoporosis. OBJECTIVE: To investigate the association between T245G polymorphisms in the OPG gene and BMD. METHODS: A total of 281 elderly men and postmenopausal women, 182 males and 99 females, who received routine examinations at Peking University People's Hospital between September 2008 and April 2010 were included in this study. T245G polymorphisms in the OPG gene was detected by polymerase chain reaction-restriction fragment length polymorphism together with DNA sequencing. The BMD of the lumbar spine, Ward's triangle, and forearrm was determined by dual energy X-ray absorptiometry. Clinical variables and biochemical measurements were collected simultaneously. The association between T245G polymorphisms and each detection index was analyzed using analysis of variance. RESULTS AND CONCLUSION: The distribution of T245G genotype (alleles T, G) had no difference in elderly men or postmenopausal women (P > 0.05). The GG genotype and TG genotype had higher lumbar spine BMD and TT genotype had lower lumbar spine BMD (P < 0.05). There was no difference in BMD of the Ward's triangle or forearm among different genotypes (P > 0.05). Association between T245G polymorphism and BMD was not found in postmenopausal women. These findings indicate that OPG gene is related to lumbar spine BMD in elderly men.

Acupuncture and Tuina in Namibia / 中国针灸

The development of acupuncture-moxibustion and Tuina in Namibia was introduced in the article. The history of acupuncture in Namibia is only 15 years, and there are 3 acupuncturists and Tuina practitioners with permanent resident permit in Namibia. The University of Namibia has already established a medical college, which is now cooperating with the concerning Chinese medical university of China and carrying out education for the undergraduate students. The development of acupuncture-moxibustion and Tuina has a great potential in Namibia with an extensive indication involving diseases of internal medicine, surgery, gynecology, pediatrics, orthopedics, and neurology.

Combination of rabbit antithymocyte globulin and cyclosporine A as first-line therapy for adult severe aplastic anemia / 中华血液学杂志

<p><b>OBJECTIVE</b>To analyze the efficacy and side-effects of combination of rabbit antithymocyte globulin (ATG) and cyclosporine A (CsA) as the first-line immunosuppressive therapy (IST) for adult severe aplastic anemia (SAA) patients.</p><p><b>METHODS</b>Adult SAA or very severe aplastic anemia (VSAA) patients treated with rabbit ATG + CsA as first line therapy in our hospital from 2003 to 2008 were retrospectively analysed and the therapeutic response relevant factors were analysed.</p><p><b>RESULTS</b>Seventy-nine patients were enrolled. Of all these patients, 6 died within 3 months after IST. The overall response rate was 82.2% and the median time to transfusion independent was 60 days. The therapeutic response rate in 32 SAA patients (100%) was significantly higher than that in 41 VSAA cases (68.3%) (P = 0.001). Patients with neutrophil response to G-CSF treatment had a higher IST response rate than those without response to G-CSF (100% vs 67.5%, P = 0.001). Sixty-one patients (77.2%) occurred serum sickness reaction. Three patients relapsed and two developed clonal hematological abnormalities after IST. The 3-year overall survival for all the patients was 88.9%.</p><p><b>CONCLUSIONS</b>Rabbit ATG in combination with CsA as first-line IST for adult SAA can lead to excellent treatment outcomes with minor adverse effects.</p>

The significance of hematopoietic cell genetic instability in aplastic anemia / 中华血液学杂志

<p><b>OBJECTIVE</b>To 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.</p><p><b>METHODS</b>Genetic 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.</p><p><b>RESULTS</b>Comet 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.</p><p><b>CONCLUSIONS</b>Increased 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.</p>

The clinical and laboratory characteristics of T cell large granular lymphocyte leukemia / 中华血液学杂志

<p><b>OBJECTIVE</b>To analyze the characteristics of T-cell large granular lymphocyte leukemia (T-LGLL).</p><p><b>METHODS</b>Retrospectively analyze the clinical and laboratory data of 27 patients with T-LGLL diagnosed between 1999 and 2007 in our hospital.</p><p><b>RESULTS</b>The median age at diagnosis was 48 years. All patients were symptomatic, mainly complaining of fatigue. Of the 27 patients, 14 (51.9%) had splenomegaly, and 4(14.8%) hepatomegaly. Rheumatoid arthritis was not present in any patients. The most frequent hematological abnormality was anemia (24 patients, 88.9%) with a median Hb level of 57.5 g/L. Pure red cell aplasia was found in 18 patients (66.67%). The median WBC count was 4.24 x 10(9)/L and 19 cases were neutropenia (ANC < 1.5 x 10(9)/L). The median LGL count in peripheral blood was 1.45 x 10(9)/L and most of them (77.8%) were less than 2.0 x 10(9)/L. Twenty-two patients (81.5%) showed the CD3+ CD8+ CD57+ CD56(-) LGL phenotype. With immunosuppressive therapy, 91.3% of patients responded and complete hematological remission rate was 65.2%.</p><p><b>CONCLUSION</b>T-LGLL mainly presented with anemia and complete hematological remission rate was 65.2%. Pure red cell aplasia was commonly associated with the disease. The patients had a good response to immunosuppressive therapy.</p>

Clinical and hematological features of congenital dyserythropoietic anemia type I / 中华血液学杂志

<p><b>OBJECTIVE</b>To analyze the clinical and laboratory features of patients with congenital dyserythropoietic anemia type I (CDA-I), and improve the clinical diagnostic accuracy.</p><p><b>METHODS</b>The clinical and hematological features of 5 patients diagnosed as CDA-I in our hospital between July 2002 and July 2007 were analyzed retrospectively, and the related literatures was reviewed.</p><p><b>RESULTS</b>Five CDA-I patients, 1 male and 4 females, all had a long history of varied degree of chronic anemia. One patient had congenital malformations, 3 jaundice and 4 hepatosplenomegaly. Bone marrow specimens invariably showed hypercellularity due to erythroid hyperplasia with megaloblastic changes, irregularly shaped nuclear, and chromatin bridges in 0.2% to 0.6% of all erythroblasts. All the 5 patients' bone marrow erythroblasts showed spongy heterochromatin appearances (swiss-cheese) with electron microscopy examination. There was no morphologic abnormality in the granulocytes and megakaryocytes. Serum ferritin levels were increased in 3/4 patients. One patient had been misdiagnosed as hereditary spherocytosis and performed splenectomy in the local hospital with no improvement in Hb level.</p><p><b>CONCLUSIONS</b>CDA-I is a rare congenital anemia characterized by ineffective erythropoiesis, jaundice, hepatosplenomegaly and iron overload, and may be misdiagnosed. Keeping these manifestations in mind should avoid misdiagnosis.</p>

Combination of rabbit antithymocyte globulin plus cyclosporin A as first-line immunosuppressive therapy for the childhood with severe aplastic anemia / 中华血液学杂志

<p><b>OBJECTIVE</b>To analyse the efficacy and side-effects of rabbit antithymocyte globulin (ATG) and cyclosporin A (CsA) as the first-line therapy for childhood severe aplastic anemia (SAA).</p><p><b>METHODS</b>Seventy-one childhood SAA patients treated with rabbit ATG + CsA as first line therapy were retrospectively analysed.</p><p><b>RESULTS</b>Seventy-one SAA patients, including 38 SAA and 33 very severe aplastic anemia (VSAA), were enrolled. The median age was 12 years. Of these patients, 3 died within 3 months after the immunosuppressive therapy (IST). The overall response rate was 67.6% (46/68) and the median time to transfusion independent was 53 days. Thirty-three patients (48.5%) obtained remission in 3 months after the IST and 45 (67.2%) in 6 months. The response rates were 57.7% (15/26), 56.5% (13/23) and 94.7% (18/19) for patients less than 10 years old, 10 - 15 year-old and 15 - 18 year-old, respectively. Sixty patients suffered from serum sickness on the IST. Three patients relapsed and another 3 unrespond patients received retreatment of IST, and one patient progressed to myelodysplastic syndromes (MDS).</p><p><b>CONCLUSION</b>Rabbit ATG in combination with CsA as first line therapy for childhood SAA/VSAA can lead to overall response rate of 67.6% with minor adverse effects.</p>

The clinical characteristics of T cell large granular lymphocyte leukemia associated with pure red cell aplasia / 中华血液学杂志

<p><b>OBJECTIVE</b>To analyze the characteristics of acquired pure red cell aplasia (PRCA) secondary to T cell large granular lymphocyte leukemia (T-LGLL).</p><p><b>METHODS</b>Fourteen patients with T-LGLL associated with PRCA between 2000 and 2006 in our hospital were retrospectively analyzed.</p><p><b>RESULTS</b>The median age at diagnosis was 61 years with equal gender distribution. The PRCA had indolent process, mainly presenting with anemia. Of the 14 patients, 9 had mild to moderate splenomegaly, one hepatomegaly and one lymphadenopathy. The median Hb level was 61.5 g/L and the median WBC count 4.3 x 10(9)/L. The median percentage and count of LGL in peripheral blood were 0.36 and 1.9 x 10(9)/L respectively. The median percentage of LGL in BM was 0.165 (0.085 - 0.410). Some patients had serologic abnormalities. All the 12 cases with available bone marrow cell cytogenetics showed normal karyotypes. With cyclosporine A or glucocorticoid immunosuppressive therapy, the overall response was 91%.</p><p><b>CONCLUSION</b>T-LGLL was one of the major causes of acquired PRCA. This type of PRCA has the similar clinical and laboratory feature to that of other type of PRCA and has a good response to immunosuppressive therapy.</p>

The impact of immunosuppressive therapy on genetic instabilities of bone marrow hematopoietic cells in patients with aplastic anemia / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the impact of immunosuppressive therapy (IST) on genetic instabilities of bone marrow hematopoietic cells (BMHCs) in patients with aplastic anemia (AA).</p><p><b>METHODS</b>Comet assay as used to detect genetic instabilities of hematopoietic cells from patients, and the percent of DNA in comet tail (TDNA), tail length (TL), tail moment (TM), olive tail moment (OTM) and the rate of comet cells were measured. BMHCs from AA patients were examined with comet assay before and after IST, and the results were compared with those from controls.</p><p><b>RESULTS</b>Comet parameters from 91 AA patients including TDNA, TL, TM, OTM comet cell percentage were (5.0 +/- 4.0)%, 11.3 +/- 7.2, 1.7 +/- 2.0, 1.5 +/- 1.4, (16.8 +/- 13.7)%, respectively, which were significantly higher than those from control group (P < 0.05). There were statistical differences between the comet parameters of severe AA (SAA)/non-SAA (NSAA) and those of control group (P < 0.05), but no difference in the comet parameters between SAA and NSAA patients (P > 0.05). The TDNA, TL, TM, OTM and comet cells percentage were (4.4 +/- 3.6)%, 10.4 +/- 7.5, 1.4 +/- 1.6, 1.3 +/- 1.4 and (20.2 +/- 21.2)%, respectively at 3 months after IST in 53 SAA patients and were (3.7 +/- 3.3)%, 10.0 +/- 7.2, 1.2 +/- 1.8, 1.1 +/- 1.3 and (18.5 +/- 19.0)% respectively at 6 months after IST in 30 SAA patients, being no statistical difference from those of 58 SAA patients before IST (P values were all > 0.05).</p><p><b>CONCLUSION</b>BMHCs of AA had inherent genetic instabilities which were not increased by recent IST. It indicated that there was no correlation between IST and the development of clonal hematologic disorders in AA.</p>