Inhibiting effects of low-molecular weight heparin and adrenocortical hormone on hemolysis of red cells in patients with paroxysmal nocturnal hemoglobinuria in vitro / 中华医学杂志(英文版)

<p><b>OBJECTIVE</b>To study the effects of low-molecular weight heparin (LMWH) and adrenocortical hormone (dexamethasone) on the hemolysis of red cells of patients with paroxysmal nocturnal hemoglobinuria (PNH) in vitro.</p><p><b>METHODS</b>Using Ham's test and micro-complement lysis sensitive test (mCLST), the changes in hemolysis of red cells from 6 typical PNH cases were examined after adding LMWH and dexamethasone in different concentrations into the test solution in vitro. The effects of LMWH and dexamethasone on the coagulation of the tested blood samples were also studied using the activated partial thromboplastin time (APTT) test.</p><p><b>RESULTS</b>Both LMWH and dexamethasone inhibited the hemolysis of PNH red cells, and they also showed a synergistic effect. The inhibiting effects were dose-dependent. Moreover, a tolerable dose of LMWH induced a limited prolongation of APTT. Dexamethasone showed two possible mechanisms in the inhibition of PNH red cells hemolysis through Ham's test and mCLST, respectively: (1) inhibiting both antibodies binding to red cells and (2) the initiation of the activation of complement 3 (C3). LMWH could inhibit hemolysis as determined by both Ham's test and mCLST, which indicated that LMWH could block the activation of complement cascade.</p><p><b>CONCLUSIONS</b>Both LMWH and dexamethasone could inhibit hemolysis in PNH, and they showed a synergistic effect. Their mechanisms of inhibiting hemolysis differed from each other. Furthermore, a tolerable dose of LMWH induced a limited prolongation of APTT. LMWH might be useful for controlling acute hemolysis in patients with PNH and reducing the dose of adrenocortical hormone.</p>

In vitro effects of low-molecular weight heparin and dexamethasone on the hemolysis of red blood cells from paroxysmal nocturnal hemoglobinuria patients / 中华血液学杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHODS</b>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).</p><p><b>RESULTS</b>(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.</p><p><b>CONCLUSION</b>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.</p>

Clinical analysis of 78 cases of paroxysmal nocturnal hemoglobinuria diagnosed in the past ten years / 中华医学杂志(英文版)

<p><b>OBJECTIVE</b>To learn more about the clinical and laboratory features of patients with paroxysmal nocturnal hemoglobinuria (PNH) diagnosed in the past ten years.</p><p><b>METHODS</b>Clinical and laboratory data for 78 cases of PNH diagnosed from January 1990 to November 1999 in our hospital were analyzed retrospectively.</p><p><b>RESULTS</b>In comparison with PNH cases reported in the 1980s, the newly diagnosed PNH cases showed the following features: (1) older age of disease onset (from 27 to 34 years); more female cases (from 18.5% to 38.5%); more cases without hemoglobinuria (from 24.2% to 38.5%). (2) No positive family hereditary history. (3) Bone marrow dysplasia, abnormal karyotype and negative sister chromatid differentiation were found in 19.2%, 12.2% and 8.9% of the PNH patients, respectively. 12.3% of the patients had bone marrow hypoplasia, and most of them had no hemoglobinuria. Ham's tests were negative in about 34.2% of the cases. CD55 and CD59 on peripheral blood cells were deficient in 100.0% of the cases, suggesting that CD55 and CD59 tests can improve the diagnosis of PNH. (4) Adrenocortical hormone was effective in 83.8% of the patients, 54.2% of whom relapsed within one year. Eight refractory and relapsed patients were treated with low dose chemotherapy (MP therapy: Melphalan 2 - 6 mg x d(-1); Prednisone 0.5 mg x kg(-1) x d(-1)). Five (62.5%) of them showed positive responses. Bone marrow failure and other side effects were not serious in this group of patients.</p><p><b>CONCLUSIONS</b>PNH, an acquired blood disease seen more often among adult males, can be diagnosed more sensitively by hemocyte member CD55 and CD59 tests and treated more effectively with adrenocortical hormone or low dose chemotherapy.</p>

Clinical analysis of 185 patients with polycythemia vera / 中华血液学杂志

<p><b>OBJECTIVE</b>To understand the clinical feature and natural course of polycythemia vera (PV).</p><p><b>METHODS</b>The clinical symptoms, signs, laboratory examination and prognosis of 185 patients with PV were analysed.</p><p><b>RESULTS</b>There are 122 males and 63 females. The mean age was (52.7 +/- 14.1) years. The mean hemoglobin level was (208.3 +/- 21.2) g/L. Pancytosis was displayed in 74 (40%) cases, excess of red blood cells in 33 (17.8%), excess of red blood cells and granulocytes in 67 (36.2%) and excess of red blood cell and platelets in 11 (5.9%). Splenomegaly was found in 123 (66.5%) patients and hepatomegaly in 30 (16.2%). Quantitative assess of serum Epo was done in 25 patients. The level was low in 16 (64.2%) and normal in 9 (36.0%). Hematopoietic progenitor culture yields was elevated in 11 patients, endogenous erythroid colonies (EEC) formation was found in 10 cases (90.9%). Eighty two patients (44.3%) had 101 attacks of vascular thrombotic incidents, 7 patients developed myelofibrosis (MF). Secondary cancer occurred in 1 patient. Two patients died of thrombosis.</p><p><b>CONCLUSION</b>PV is an elderly adult myeloproliferative disease with a high frequency of thrombosis. EEC can be found out in PV patients. The serum Epo level is not increased in PV patients. The main sequelae of PV is MF.</p>

Role of Th1 cell in the pathogenesis of aplastic anemia / 中华血液学杂志

<p><b>OBJECTIVE</b>To detect the quantity, proportion and function of producing cytokines of Th1 and Th2 cells in aplastic anemia (AA) patients and their contribution to the hematopoietic failure.</p><p><b>METHODS</b>(1) Eleven patients with severe aplastic anemia (SAA) at diagnosis were observed by Marsh's method for the CFU-E, BFU-E and CFU-GM before and after depletion of CD(4)(+) T lymphocytes from bone marrow mononuclear cells (BMMNC); (2) Th1 (CD(4)(+) IFN-gamma(+)) and Th2 (CD(4)(+) IL-4(+)) cells in peripheral blood mononuclear cells (PBMNC) of 21 SAA patients and 17 normal controls were counted by FACS. (3) mRNA expression of IFN-gamma and IL-4 gene in unstimulated BMMNC from 16 SAA patients, 11 chronic aplastic anemia (CAA) patients, 26 other hematological diseases patients and 11 normal controls were measured by reverse transcriptase polymerase chain reaction (RT-PCR).</p><p><b>RESULT</b>(1) CFU-E, CFU-GM and BFU-E increased significantly after depletion of CD(4)(+) T lymphocytes from BMMNC of SAA patients. (2) The percentage of IFN-gamma producing CD(4)(+) T cell (Th1) of SAA patients was significantly higher than that of controls, the percentages of IL-4 producing CD(4)(+) T cells (Th2) had no difference between SAA patients and normal controls. (3) IFN-gamma mRNA was detected in unstimulated BMMNC in 13 of 16 SAA patients, 6 of 11 CAA patients and one of 6 paroxysmal nocturnal hemoglobinuria (PNH) patients. The IFN-gamma mRNA was not detected in unstimulated BMMNC of 11 normal controls and other hematological diseases patients.</p><p><b>CONCLUSIONS</b>Disbalance of CD(4)(+) T lymphocytes subsets and increases in quantity and IFN-gamma producing function of Th1 cells might be important for the development of bone marrow failure in AA and in distinguishing AA from other kinds of pancytopenic diseases.</p>

Quantity and apoptosis-related protein level of B lymphocyte in patients with immunorelated pancytopenia / 中华血液学杂志

<p><b>OBJECTIVE</b>To examine the quantity and apoptosis-related protein level of B lymphocyte in the patients with immunorelated pancytopenia (IRP) and explore the role of B lymphocyte in the pathogenetic mechanism of IRP.</p><p><b>METHODS</b>Quantities of all B lymphocytes and CD(5)(+) B lymphocytes and the expressions of Fas and bcl-2 on B lymphocytes in 25 patients with untreated IRP, 15 IRP patients in complete remission (CR) and 10 normal controls were assayed by FACS.</p><p><b>RESULTS</b>The percentages of B lymphocyte and CD(5)(+) B lymphocytes were significantly higher in untreated IRP patients than in CR IRP patients and normal controls (P < 0.05); there was no significant difference between the latter two groups (P > 0.05). There was no significant difference of Fas expression in B lymphocytes among the three groups (P > 0.05). The expression of bcl-2 on B lymphocytes was significantly higher in untreated patients than in CR patients or normal controls (P < 0.05), and so did in CR patients than in normal controls (P < 0.01). The apoptosis-related index was significantly lower in untreated patients than in CR patients or normal controls (P < 0.01), and was lower in CR patients than in normal controls (P < 0.05). The percentage of B lymphocyte was positively correlated with the duration from the beginning of treatment to response.</p><p><b>CONCLUSION</b>The production of auto-antibodies in IRP patients probably has some relationships with the abnormal quantities of B lymphocyte and its subsets, and with the inhibition of B lymphocyte apoptosis.</p>

A long-term follow-up study of 50 patients with severe aplastic anemia who have survived more than 3 years after immunosuppressive therapy / 中华血液学杂志

<p><b>OBJECTIVE</b>To evaluate the long-term outcome of immunosuppressive therapy (IST) in patients with severe aplastic anemia (SAA).</p><p><b>METHODS</b>Hematopoietic 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.</p><p><b>RESULTS</b>At 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.</p><p><b>CONCLUSION</b>After 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.</p>

Giant Tumor Cells in the Bone Marrow and Their Significance / 中国实验血液学杂志

Giant tumor cells and their varieties in the bone marrow were found in 7 patients with abnomal hematopoiesis phenomena. These cells were artificially devided into 5 kinds according to the difference of their morphology. Most of these cells were corresponding to lymphoid-monocytoid-macrophagocytoid cells with Wright's staining, cytochemical stainings, immunocytochemical stainings, flow cytometry examination, electron microscopy and pathologic study. The bone marrows were hypercellular and marked dysplastic hematopoiesis phenomena. Two of the 7 cases were diagnosed as malignant lymphoma with bone marrow biopsy. All cases characteristically showed no lymph node enlargement or hepatosplenomegaly or any local tumor mass. As to the prognosis of these cases, two patients died with survival time of 8 and 17 months, respectively, one was on critical condition at course of 10 months, and the other 4 cases were in comparatively stable condition with courses of 2.5 to 24 months. These patients seem to be a group of rare malignant lymphoid-monocytoid-macrophagocytoid proliferative diseases.