OBJECTIVE:To investigate the prognostic value of quantitative chromosomal abnormality in myelodysplastic syndromes (MDS).METHODS:Chromosomal karyotypes in seventy-one MDS patients' were analyzed quantitatively. Based on the number of abnormal metaphase in 20 counted metaphases, the patients were divided into three groups: no abnormal karyotypes, abnormal metaphases less than or equal to five, and that more than five. The leukemia transformation rate, death rate and survival time between these three groups were compared.RESULTS:Forty-four cases (62.0%) had abnormal karyotypes. The incidences of abnormal karyotypes in RA, RCMD and RAEB were 76.9%, 55.8% and 75.0%, respectively, being no significant difference (P > 0.05). Among the abnormal karyotypes, complex abnormality with two or more abnormal karyotypes was most common and accounted for 47.7%. The frequencies of trisomy 8, monosomy 7 and del 20q were 18.2%, 4.5% and 4.5%, respectively. Other kinds of abnormal karyotypes totally accounted for 25%. There were 27 cases of group 1, 28 of group 2 and 16 of group 3. Eighteen cases (25.4%) transformed to acute leukemia. The incidences of leukemia transformation in group 1, 2 and 3 were 18.5%, 25% and 37.5%, and the death rates were 29.6%, 42.9% and 56.3%, respectively. The median survival times were 60, 47 and 24 months respectively.CONCLUSION:The quantitative chromosome abnormality has prognostic value in MDS.

OBJECTIVE:To study the response of hematopoietic cells (HSC) to granulocyte colony stimulating factor (G-CSF) in paroxysmal nocturnal hemoglobinuria (PNH) patients.METHODS:(1) Bone marrow mononuclear cells (BMMNC) from 17 PNH patients and 12 normal subjects were inoculated into semisolid culture media containing or not G-CSF (50 ng/ml). The cluster/colony forming unit-granulocyte/monocyte (CFU/cFU-GM) were counted and compared. (2) BMMNC of 20 PNH patients and 12 normal controls were triply stained for CD34, CD59 and G-CSF receptor CD114/stem cell factor receptor (C-KIT) CD117 and assessed by FCM. The CD34(+) cells were identified as CD34(+)/CD59(+) and CD34(+)/CD59(-). Percentage of CD114 and CD117 expression in each cell population was calculated.RESULTS:(1) PNH cFU-GM without G-CSF were (112.41 +/- 22.74)/10(5) BMMNC, while with G-CSF: (133.82 +/- 25.85)/10(5) BMMNC and normal cFU-GM were (190.33 +/- 36.05)/10(5) BMMNC, (309.42 +/- 92.94)/10(5) BMMNC, respectively. Whether with or without G-CSF, PNH BMMNC formed less cFU-GM than control did, both of the two kinds of BMMNC responded to G-CSF well (P < 0.05), but the increment of PNH cFU-GM yields was less than that of the normal control (P < 0.05). CFU-GM yields of PNH BMMNC without G-CSF were (24.29 +/- 9.05)/10(5) BMMNC, with G-CSF were (27.53 +/- 10.65)/10(5) BMMNC, while normal control were (77.42 +/- 36.01)/10(5) BMMNC and (98.00 +/- 43.14)/10(5) BMMNC, respectively. Whether with or without G-CSF, PNH BMMNC showed less CFU-GM yields than that of control (P < 0.05). (2) The percentage of CD114 positive cells in PNH CD34(+)CD59(+) BMMNC was (73.34 +/- 29.40)% and that in PNH CD34(+)CD59(-) BMMNC and in control CD34(+)CD59(+) BMMNC were (32.70 +/- 6.89)% and (58.52 +/- 29.99)%, respectively. The percentage of CD114 expression in PNH CD34(+) CD59(-) BMMNC was less than that in the other two groups (P < 0.05). The percentages of CD117 positivities on the PNH CD34(+)CD59(+) BMMNC were (76.90 +/- 22.08)%, PNH CD34(+) CD59(-) (36.03 +/- 7.69)% and control CD34(+) CD59(+) (80.28 +/- 13.36)%, respectively (P < 0.01).CONCLUSION:In vitro, BMMNC of normal control grow better, and respond better to G-CSF than PNH BMMNC do. PNH CD34(+)CD59(-) BMMNC express less G-CSF receptor and C-KIT than PNH CD34(+)CD59(+) and normal CD34(+)CD59(+) BMMNC do, which may be the reason that abnormal PNH clone grow worse than the normal clones do.

This study was aimed to evaluate expression levels of CD166, Fas and apoptosis-related proteins in bone marrow neutrophils of PNH patients and normal controls, and to analyze their correlation in order to explore whether exist apoptosis abnormality in BM neutrophils of PNH patients. The expression levels of CD16b, Fas and Bax, Bcl-2 in BM neutrophils of PNH patients and normal controls were assayed by flow cytometry; the difference of expression levels between patients and controls, and expression correlation between CD16b and apoptosis-related proteins were compared. The results showed that (1) the expression levels of CD16b on BM neutrophils of patients and controls were (20.36 +/- 9.05)% and (71.34 +/- 26.8)% respectively (P = 0.01); (2) the expression levels of CD95 on BM neutrophils of patients and controls were (62.83 +/- 32.11)% and (48.00 +/- 38.52)% respectively, there were no significant difference between CD95 expressions in BM neutrophils of PNH patients and controls and no significant correlation between expression of CD95 and CD16b on BM neutrophils of PNH patients (P > 0.05); (3) the expression levels of Bcl-2 in BM neutrophil cytoplasma of patients and controls were (8.64 +/- 5.40)% and (16.82 +/- 15.39)% respectively, there were no significant difference between Bcl-2 expression of patients and controls, and no significant correlation between the expression of Bcl-2 and CD16b in BM neutrophil cytoplasma of PNH patients (P > 0.05); (4) the expression levels of Bax in BM neutrophil cytoplasma of patients and control were (30.47 +/- 22.15)% and (48.47 +/- 15.99)% respectively, there were no significant difference between the Bax expressions of patients and controls, and no significant correlation between the Bax and CD16b expressions in BM neutrophil cytoplasma of PNH patients. In conclusion, BM neutrophils of PNH patients expressed apoptosis-related CD95, Bcl-2 and Bax without significant difference from the normal controls, and without significant correlation with the CD16b expression. It is suggested that the cell growth and decrease of PNH patients possibly are independent of abnormal apoptosis.

OBJECTIVE To investigate the abnormal hematopoietic clone burden of the patients with myelodysplastic syndromes (MDS) and its clinical implication. METHODS The ratio of the metaphase with abnormal karyotypes to the total was regarded as the index of MDS clonal burden. Thirteen parameters were assayed and the correlations between these parameters and MDS clone burden were analysed. RESULTS The clonal burden of MDS patients was (67.4 +/- 36.2)%. It correlated positively with bone marrow blasts (r = 0.483, P < 0.05), negatively with hemoglobin level (r = -0.445, P < 0.05). The number of blasts, hemoglobin and erythrocytes in high clonal burden (>50%) and low clonal burden (< or = 50%) groups were significantly different (P < 0.05). CD4+ T lymphocytes of MDS patients and normal controls were (274.18 +/-71.85) x 10(6)/L and (454.82 +/- 205.88) x 10(6)/L (P < 0.05) respectively. CD8+ T lymphocytes between MDS patients and normal controls had no difference. The serum level of IL-2 of MDS patients and normal control groups were (6.29 +/- 3.58) g/L and (3.11 +/- 1.40) microg/L (P < 0.05) respectively; but no difference in the serum level of TNF between MDS and control groups. The ratio of CD4+ to CD8+ in high clonal burden patients was 1.90 + 0.52, and in low clonal burden patients was 0.97 +/- 0.44 (P < 0.05). CONCLUSION The clonal burden and deficient T cell immunity are the indicators for predicting MDS patients clinical progression.

OBJECTIVE:To study the characteristics of cell cycle and proliferation of CD34+ hematopoietic stem cells in patients with myelodysplastic syndromes (MDS). METHODS:Propidium iodide staining was used to examine cell cycle parameters (G(0)/G(1), S and G(2)/M) of bone marrow mononuclear cells (BMMNCs) while immunofluorescent double staining and FACS techniques were used to measure Ki67 expression in BM CD34+ cells from normal control, patients with MDS, acute myeloid leukemia preceded by MDS (MDS-AML) and primary AML. RESULTS:There was a statistical up-tendency in G(0)/G(1) phase proportion of BMMNCs whereas a statistical down-tendency in S and G(2)/M phase proportions among normal control, MDS and primary AML. Compared to primary AML, MDS-AML had significantly higher ratios of S (P < 0.05), G(2)/M (P < 0.05) and S + G(2)/M (P < 0.05) phase cells while lower ratio of G(0)/G(1) phase cells (P < 0.05). The proportion of CD34+Ki67+ cells in MDS patients was significantly higher than that in normal control (P = 0.004). So were the percentages of CD34+Ki67+ cells in low-risk [(0.54 +/- 0.49)%, P < 0.05] and high-risk MDS patients [(1.69 +/- 1.66)%, P = 0.022]. Furthermore, there was statistical difference between low-risk and high-risk MDS (P < 0.05). Compared to normal control and primary AML, MDS-patients had the highest proportion of CD34+Ki67+ cells [(16.75 +/- 13.58)%, P < 0.05]. The proportion of CD34+Ki67+ cells in CD34+ cells in MDS patients [(48.50 +/- 20.49)%] was significantly higher than that in normal control [(27.71 +/- 16.04)%, P < 0.01]. So were the low-risk [(51.85 +/- 21.80)%, P = 0.002] and high-risk MDS [(43.93 +/- 18.57)%, P < 0.05]. The proportion of CD34+Ki67+ cells in CD34+ cells in MDS-AML patients [(60.92 +/- 30.12)%] was the highest, and was statistically higher than that in both normal control (P < 0.01) and primary AML patients [(17.01 +/- 15.93)%, P < 0.001]. The proportion of CD34+Ki67+ cells in Ki67+ cells in MDS patients [(4.91 +/- 4.68)%, P < 0.01] was significantly higher than that [(2.43 +/- 2.37)%] in normal controls. In the low-risk MDS group it was (4.11 +/- 3.94)%, (P > 0.05) and in high-risk MDS group it was (5.76 +/- 5.38)%, (P < 0.05). CONCLUSION:High proportion of G(0)/G(1) cells and G(1) phase arrest occurred in MDS. High proliferation capacity of MDS clone, especially that derived from CD34+ cells, might play an important role in the clonal expansion, diseases deterioration and worse prognosis of MDS.

OBJECTIVE To study the apoptosis and proliferation of CD(34) positive (CD(34)(+)) bone marrow cells (BMC) in patients with polycythemia vera (PV). METHODS The expression of Annexin V and Ki67 of the CD(34)(+) BMC in 20 PV patients and control cases [10 essential thrombocythemia (ET), 12 normal persons] were assessed by bicolor flow cytometry (FCM), and the correlation between apoptosis and clinical situation was analysed in PV patients. RESULTS The Annexin V expressions of CD(34)(+) BMC were (15.96 +/- 1.45)% in PV patients and (15.53 +/- 1.76)% in ET patients which were lower than that in normal subjects [(23.61 +/- 3.89)%, (P < 0.05)]. The Ki67 expression of CD(34)(+) BMC was (48.79 +/- 11.68)% in PV patients and (49.60 +/- 9.98)% in ET patients, which were significantly higher than that in normal controls (33.87 +/- 6.82)%. The ratio of apoptosis/proliferation in PV patients was 0.33 +/- 0.10 and in ET patients 0.32 +/- 0.02 which were significantly lower than that in normal controls 0.72 +/- 0.11 (P < 0.01). The apoptosis of CD(34)(+) BMC was negatively correlated with the hemoglobin (Hb) levels (r = -0.481, P = 0.037), white blood cells (WBC) (r = -0.538, P = 0.026) and the numbers of endogenous erythroid colony (EEC) (r = -0.632, P = 0.50), and the ratio of apoptosis/proliferation was negatively correlated with the Hb (r = -0.537, P = 0.018) and WBC (r = -0.667, P = 0.003) in PV patients. CONCLUSION There were lower apoptosis and higher proliferation in CD(34)(+) BMC of PV patients. Lower apoptosis was correlated with the severity of the disease.

目的了解联合化疗治疗高危组骨髓增生异常综合征(MDS)及其转化的急性髓系细胞白血病(post MDS-AML)的疗效和影响因素.方法 24例高危组MDS和23例post MDS-AML患者接受联合化疗方案化疗(DA方案31例,HA方案13,IA方案3例),联合化疗加用造血生长因子17例,单用联合化疗30例.采用2000年MDS国际工作组疗效评定新标准评价47例患者的联合化疗疗效,并评估MDS国际预后积分系统(IPSS)的临床意义.结果 14例(29.8%)患者获得完全缓解(CR),部分缓解(PR)10例(21.3%),稳定状态(SC)2例(4.3%),失败(failure)21例(44.7%),总有效率51.1%.高龄组(年龄超过50岁)的CR率(53.8%)明显高于年龄小于50岁的低龄组(20.6%,P=0.037).IPSS积分系统中染色体核型分组(Good组,Intermediate组和Poor组)的CR率分别为76.9%,23.5%,0%(P<0.01),3组间的总有效率(76.9%,64.7%和11.1%),失败率(23.1%,29.4%,77.8%)均有统计学差异.姐妹染色体分染(SCD)阴性组的CR率(26.3%)显著低于阳性组的CR率(71.4%,P=0.036),且阴性组的总有效率(52.6%)也低于阳性组(100%,P=0.024).DA方案的CR率(38.7%)和总有效率(61.3%)明显高于HA方案CR率(7.7%,P=0.04)和总有效率(23.1%,P=0.021),并且DA方案的失败率(32.3%)显著低于HA方案的失败率(76.9%,P=0.007).结论高危组MDS及post MDS-AML是一组难治性疾病,约51%的患者可从联合化疗中获益.染色体核型异常和姐妹染色体分染对化疗疗效有预后意义.

OBJECTIVETo observe the efficacy and side effect of DA/HA regimen chemotherapy for the treatment of refractory and relapsed paroxysmal nocturnal hemoglobinuria (PNH).METHODSEight patients with refractory and relapsed PNH were treated with DA/HA regimen chemotherapy. Three patients were treated with DA (DNR 40 mg/d, i.v.drip, the first and the second day; 20 mg/d, i.v.drip, the third day; Ara-C 100 mg/d, i.v.drip, for 5 days) and 5 patients with HA (HHT 2 - 3 mg/d, i.v.drip, for 5 days; Ara-C 100 mg/d, i.v.drip, for 5 days).RESULTSAll the 8 patients responded well: the PNH clone was diminished in five patients. Hemolysis was remitted in 6 cases. Five patients showed improvement in hematological parameters. The dosage of corticosteroid was decreased in all of them. No serious side effect was revealed.CONCLUSIONDA/HA regimen chemotherapy was safe and effective for refractory and relapsed PNH patients.

自身免疫性溶血性贫血(AIHA)和Evans综合征复发率高,长期缓解率只有13%～16%[1,2].复发后如何再治疗是临床上一大难题.我们对我院收治的AIHA/Evans综合征复发患者30例进行再治疗观察,了解再治疗疗效,并分析不同治疗方案对再治疗疗效的影响.现将结果报告如下.

OBJECTIVES:To explore the proliferative capacity of bone marrow hematopoietic stem cells and the function of T helper (Th) lymphocytes of patients with immuno-related pancytopenia (IRP).METHODS:Twenty-five untreated IRP patients, 15 IRP patients in complete remission (CR) and 10 normal controls were studied for in vitro yields of CFU-GM, CFU-E and BFU-E from bone marrow mononuclear cells (BMMNC). The mRNA expressions of IL-4, IL-10, IFN-gamma and IL-2 genes in unstimulated BMMNC from 25 untreated IRP patients,15 IRP patients in CR, 19 patients with other hematological diseases presenting pancytopenia and 10 normal controls were detected by reverse transcription polymerase chain reaction (RT-PCR).RESULTS:There was no significant difference of the yields of CFU-E, CFU-GM or BFU-E among the untreated, and in CR IRP patients and normal controls (P > 0.05). The mRNA expressions of IL-4 and IL-10 of Th2 cells were significantly higher in untreated IRP patients than in the other groups. The mRNA expressions of IFN-gamma and IL-2 of the Th1 cells in all IRP patients were not higher than those in the other groups.CONCLUSIONS:The cytopenia of IRP patients was not caused by the qualitative abnormality of the hematopoietic stem cells but by the destruction or suppression of hematopoietic stem cells from certain extrinsic insults. The imbalance of Th lymphocytes subtypes and overfunction of Th2 lymphocytes played important roles in the pathogenetic mechanism of IRP leading to increased and overfunctional B lymphocytes, which produced autoantibodies destructing or suppressing hematopoiesis in IRP.

目的检测免疫相关性全血细胞减少症(IRP)患者骨髓B淋巴细胞的数量、功能及凋亡相关蛋白水平,以探讨B淋巴细胞在IRP发病中的作用.方法采用流式细胞术测定25例初诊患者,15例治疗后血象恢复正常的患者和10例正常人骨髓总B淋巴细胞、CD5+B淋巴细胞细胞的数量,B淋巴细胞胞浆内免疫球蛋白IgM的表达以及B淋巴细胞膜表面凋亡相关蛋白Fas抗原(CD95)、胞浆内抗凋亡蛋白bcl-2的表达.结果IRP初诊患者总B淋巴细胞、CD5+B淋巴细胞比例显著高于治疗后血象恢复组和正常人(P均＜0.05),后两组相比无显著性差异(P＞0.05);初诊患者骨髓B淋巴细胞免疫球蛋白IgM的表达也明显高于其他两组.三组患者B淋巴细胞Fas抗原的表达率无明显区别(P均＞0.05);初诊患者B淋巴细胞胞浆内bcl-2的表达率显著高于其它两组(P均＜0.05),治疗后血象恢复组也显著高于正常对照组(P＜0.01);各组间B淋巴细胞凋亡相关指数(CD19+CD95+/CD19+Bcl-2+)比较,初诊组显著低于治疗后血象恢复组和对照组(P均＜0.01),治疗后血象恢复组也显著低于对照组(P＜0.05);B淋巴细胞的比例与治疗起效时间呈正相关(r=0.69,P＜0.01).结论IRP患者自身抗体的产生可能与B淋巴细胞及其亚群数量异常、功能亢进及凋亡受抑有关.

OBJECTIVE:To study the clinical features of severe aplastic anemia (SAA) patients with complication of infection.METHODS:A retrospective analysis of prevalence of infection occurring in 229 SAA patients, their bacterial spectrum, and the effect of GM-CSF or G-CSF on the infection were done.RESULT:The prevalence of infection in SAA patients was 86.0%, among which 54.2% was infected with gram-positive organisms, 40.0% with gram-negative bacilli and 5.8% with fungal infections. Septicemia occurred mostly with E. coli and Pseudomonas infection. Patient's neutropenia was significantly related to the infection. The patients with neutrophil count less than 0.2 x 10(9)/L had more frequent and severe infection. Age, hemoglobin level, subtype of T lymphocytes and antithymocyte globulin therapy were not related to infection. Prophylaxis usage of floxacin could not reduce patient' gastrointestinal infection. The total mortality of SAA patients with infection was 23.1%. Pulmonary infection and septicemia increased mortality, and GM-CSF/G-CSF therapy reduce mortality.CONCLUSION:SAA patients were at high risk of infection which was significantly associated with severe neutropenia. GM-CSF or G-CSF therapy exerts an assistant role to antibiotics in controlling the infections.

OBJECTIVE To investigate the growth of endogenous erythroid colony (EEC) in polycythemia vera (PV) patients and its clinical significance. METHODS Bone marrow mononuclear cells of 26 PV patients, 2 secondary erythrocytosis (SE) and 19 normal controls were cultured by Marsh's method for EEC. RESULTS 1. EEC was present in 25/26 (96.2%) PV patients and was not found in 2 SE patients and 19 normal controls. 2. The number of EEC and the ratio of EEC/Epo-dependent CFU-E (EEC ratio) were positively correlated with the hemoglobin (Hb) levels (r = 0.608, P = 0.01) in PV patients, but did not correlate with white blood cell (WBC) counts, platelet counts and neutrophil alkaline phosphatase scores. 3. EEC did not correlate with PV patients' serum Epo levels (r = 0.518, P = 0.125). 4. Fifteen PV patients were treated with hydroxyurea and/or interferon-alpha. Their EEC ratio before treatment was correlated positively with the time required for complete remission (CR) (r = 0.651, P = 0.009) and negatively with the time before relapsing (r = -0.529, P < 0.02). 5. EECs of 7 PV patients treated with HU/IFN were decreased after their blood cell counts normalization. 6. There was a positive correlation between the EEC ratio and the attacks of vascular thrombosis (r = 0.524, P = 0.01). (7) The apoptosis of bone marrow mononuclear cells of PV patients was less than that of normal controls. PV patients' EEC was negatively correlated with the apoptosis of their bone marrow mononuclear cells (r = -0.192, P < 0.045). CONCLUSION EEC is peculiarly present in PV patients, and is a sensitive parameter in reflecting the abnormal hematopoietic clone burden and in diagnosing and monitoring the disease.

OBJECTIVE:To analyse the relapse rate and risk factors of autoimmune hemolytic anemia (AIHA) and Evans syndrome. METHODS:Fifty two cases of AIHA and Evans syndrome in remission being followed up for 1 - 14 years (median time 3.8 years) were analysed for relapse rate. The risk factors of relapse were analysed by case-control study. RESULTS:The total relapse rate of these AIHA and Evans syndrome patients was 57.7%, and the median remission duration to the first relapse was 9 months. The relapse rates in patients with negative Coombs test, warm autoantibodies and both of warm and cold autoantibodies were 30.8% (4/13), 54.0% (13/24) and 86.7% (13/15), respectively. The relapse rate in patients with cold antibody was the highest (P < 0.05). The relapse rate in patients with antibody titer >or= 100 was 92.9% (13/14) and was higher than that in patients with antibody titer < 100 [59.5% (13/22)] (P < 0.05). Patients treated with prednisone and cyclosporin relapsed less than those treated with prednisone alone, and the relapse was related to the therapy course of prednisone and CsA. CONCLUSION:Because of the high relapse rate, AIHA and Evans syndrome should be treated according to the class of autoantibodies, and with longer course of prednisone and cyclosporin and prophylaxis of infection.

单纯放血治疗真性红细胞增多症(PV),有较高的血栓栓塞及骨髓纤维化(MF)发生率.32P放疗、烷化剂化疗又可增加PV患者急性白血病、其他系统恶性肿瘤的转化率[1,2].因此选择安全、有效的治疗策略对PV患者至关重要.我们对比干扰素加羟基脲(IFN+HU) 、羟基脲(HU)治疗PV的有效性及安全性,现报告如下.

OBJECTIVES:To explore the category, quantity and clinical significance of autoantibodies on bone marrow hematopoietic cells in patients with immunorelated cytopenia and evaluate the sensitivity of direct antiglobulin reaction (Coombs test ) of bone marrow mononuclear cells (BMMNC).METHODS:The category and the positive rate of autoantibodies on bone marrow hematopoietic stem cells, nucleated erythrocytes, granulocytes in 32 patients with uncertain immunorelated cytopenia were investigated by using BMMNC-Coombs test and double immunofluorescence flow cytometry.RESULTS:The positive rate of autoantibodies on bone marrow hematopoietic cells tested by flow cytometry was 90.63% which was higher than that by BMMNC-Coombs test (50.0%) (p < 0.05). In 29 positive cases, IgG autoantibody accounted for 6.90%, IgM13.8%, IgG+IgA 3.4%, IgG+IgM 31.0%, and IgG+IgM+IgA 44.8%. Of the 29 Patients, 25 (86.2%) with IgG autoantibody, 26 (89.7%) with IgM and 14 (48.3%) with IgA. The patients with IgG autoantibody alone had the lowest hemoglobin levels, and those with IgM autoantibody might have intravascular hemolytic findings. The response time of patients with IgG and IgG+IgM was shorter than that of the other patients. 91.3% of the patients had autoantibodies on bone marrow hematopoietic stem cells and showed pancytopenia, and 50% of the patients had autoantibodies on nucleated erythrocytes and granulocytes. Eleven of 13 patients with negative BMMNC-Coombs tests had autoantibodies on bone marrow hematopoietic stem cells detected by FACS. There was no significant difference of the quantities of the three categories of autoantibodies of nucleated erythrocytes and stem cells. The quantities of IgA on granulocytes were lower than that of IgG and IgM. There was no significant difference between IgG and IgM on granulocytes. The quantity of IgA on hematopoietic stem cells was significantly higher than that on nucleated erythrocytes or granulocytes.CONCLUSIONS:The sensitivity of double immunofluorescence flow cytometry assay was higher than that of BMMNC-Coombs test for detecting autoantibodies. In immunorelated cytopenia patients, the predominant autoantibody was IgM which could cause intravascular hemolysis, and the second one was IgG which could cause severe anemia. Most immunorelated cytopenia patients had autoantibodies on hematopoietic stem cells and showed pancytopenia. IgA was more easily seen on the hematopoietic stem cells.

OBJECTIVE:To study the in vitro effects of low-molecular weight heparin (LMWH) and dexamethasone on the hemolysis of red blood cells from paroxysmal nocturnal hemoglobinuria (PNH) patients.METHODS:By Ham's test and micro-complement lysis sensitive test (mCLST), the changes of hemolysis of red blood cells from 6 PNH patients were tested by adding different doses of LMWH and dexamethasone into the test mixture. The effects of LMWH and dexamethasone on the coagulation of the tested blood samples were also studied by activated partial thromboplastin time (APTT).RESULTS:(1) Either LMWH or dexamethasone could dose-dependently inhibit the hemolysis of PNH red blood cells, and the effects were synergistic when added together. The same dose of LMWH induced a less than 100% prolongation of APTT. (2) Dexamethasone could inhibit the hemolysis in Ham's test and had different effects on the hemolysis by different adding methods in mCLST. LMWH could inhibit the hemolysis in both Ham's test and mCLST.CONCLUSION:Both LMWH and dexamethasone could inhibit the hemolysis of PNH red cells and showed a synergistic effect. The mechanisms of the inhibition of hemolysis were different. Furthermore, a tolerable dose of LMWH induced only a limited prolongation of APTT, which might be useful for controlling acute hemolysis and reducing the dose of dexamethasone.

OBJECTIVETo evaluate the long-term outcome of immunosuppressive therapy (IST) in patients with severe aplastic anemia (SAA).METHODSHematopoietic recovery (peripheral blood cell counts, bone marrow aspirates, bone marrow biopsy, in vitro culture of hematopoietic progenitors), immunity of T lymphocyte, quality of life and side-effects of the therapy were assessed in 50 SAA patients who have survived more than 3 years after IST.RESULTSAt 3 years, 4 years and 5 years follow-up, 81.5% (13 cases), 86.7% (13 cases) and 89.5% (17 cases) of the SAA patients reached and maintained normal peripheral blood cell counts, 93.4% (15 cases), 93.3% (14 cases) and 94.7% (18 cases) showed normal bone marrow pictures, and 37.5% (6 cases), 40.0% (6 cases) and 73.7% (14 cases) had normal yields of bone marrow cell culture, respectively. Overall, 86.0% (43 cases), 94.0% (47 cases) and 52.0% (26 cases) of the total SAA patients were normalized in peripheral blood counts, bone marrow picture and culture of hematopoietic progenitor yields, respectively. During the follow-up, 88.0% (44 cases) of the patients achieved 100 of Karnofsky scores; 26 of the 31 patients (83.9%) who received bone marrow biopsy showed normal histological pictures, and 29 of 37 patients (78.4%) tested had normal subsets of T lymphocytes. No clonal disease was found. The late side-effects of IST were mild. All of the parameters tested were normal in 24 patients.CONCLUSIONAfter IST, the hematopoietic function of bone marrow, the immunity of the T lymphocyte and the life quality were normalized with few side-effects in patients with SAA. These patients would probably be cured.

慢性粒细胞白血病(CML)是人造血干细胞发生的一种恶性肿瘤,临床上慢粒是以异常的、不成熟的恶性造血祖细胞的大量增生为特征.病理特征是因第九与第22位染色体易位{t(9:22)}形成的pH1染色体,该染色体易位产生的BCR/ABL融合基因表达的具有高酪氨酸激酶活性的p210BCR/ABL融合蛋白是慢粒发生的分子基础,它能结合或激活细胞内多种信号相关的分子,为造血细胞的恶性转化必要而充分的条件.该病的p210BCR/ABL是通过什么分子机制引起这些变化仍不清楚,目前临床上除干细胞移植外尚无治愈的方法.

目的研究再生障碍性贫血-阵发性睡眠性血红蛋白尿综合征(AA-PNH综合征)与典型阵发性睡眠性血红蛋白尿症(PNH)临床特征的异同,加深对AA-PNH综合征的认识.方法回顾分析了28例AA-PNH综合征和51例典型PNH的临床表现、实验室检查及治疗反应,并进行了对照研究.结果 AA-PNH综合征与典型PNH相比:①血栓形成、黄疸、肝脾肿大等临床表现均较轻.②网织红细胞虽较低,但仍高于正常;骨髓涂片及活检多表现为增生减低,但红系比例不低;③各溶血指标检查阳性率均较低,但CD55、CD59表达异常的检出率为100%,且其在红细胞、粒细胞、淋巴细胞中表达的百分率在两组患者中无明显差异.④免疫球蛋白、T细胞亚群的检测,两组患者均无异常.⑤两组患者对肾上腺糖皮质激素为主的治疗均反应良好.结论 AA-PNH综合征虽临床表现有别于典型PNH,但与典型PNH无本质区别;CD55、CD59的检测有助于提高AA-PNH综合征的检出率.吵