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>

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>

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>

Clinical and laboratory features of preleukemia patients / 中华医学杂志(英文版)

<p><b>OBJECTIVE</b>To explore prospective diagnostic criteria for preleukemia.</p><p><b>METHODS</b>A case control study was done comparing the discrepancies on clinical and laboratory features between patients with preleukemia and those with chronic aplastic anemia (CAA) or atypical paroxysmal nocturnal hemoglubinuria (a-PNH).</p><p><b>RESULTS</b>There were eight variables of significance: (1) lymphocytoid micromegakaryocytes in the bone marrow; (2) immature granulocytes in the peripheral blood; (3) > or = 2.0% myeloblasts in the bone marrow; (4) positive periodic acid schiff (PAS) stained nucleated erythrocytes; (5) myeloid differentiation index > or = 1.8; (6) typical colonal karyotypic abnormalities; (7) negative sister chromatid differentiation; (8) cluster/colony ratio of granulocyte-macrophage colony-forming units (CFU-GM) > 4.0. The following criteria were assigned: A: to meet variable one and at least two of the other seven variables and B: to meet at least four of the eight variables. All of the patients with preleukemia met either A or B and none of the patients with CAA or a-PNH did.</p><p><b>CONCLUSIONS</b>Preleukemia is different from CAA or a-PNH. It has its own clinical and laboratory features, which may be useful for its prospective diagnosis.</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>