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1.
Journal of Experimental Hematology ; (6): 889-895, 2023.
Article in Chinese | WPRIM | ID: wpr-982146

ABSTRACT

OBJECTIVE@#To investigate the expression and clinical significance of soluble Fas (sFas) and sFasL in patients with secondary hemophagocytic lymphohistiocytosis (sHLH).@*METHODS@#From September 2015 to December 2020, 86 sHLH patients who met the HLH2004 diagnostic criteria were collected. They were divided into 55 cases in the MAHLH group and 31 cases in the NonMAHLH group according to the etiology. Thirty healthy persons were chosen as the normal control group, and 20 patients with systemic lupus erythematosus (SLE) were chosen as the disease control group. The expression levels of sFas and sFasL in the serum of patients with each group were detected by ELISA, and the clinical data were collected for statistical analysis. The significance of sFas and sFasL in sHLH was analyzed by ROC curve.@*RESULTS@#Serum levels of sFas and sFasL in patients with newly diagnosed sHLH were significantly higher than those in disease control group and normal control group (P<0.01). The levels of sFas and sFasL in MAHLH group were significantly higher than those in nonMAHLH (infection related HLH and autoimmune disease related HLH) group (P<0.01). The serum levels of sFas and sFasL in 17 newly treated patients with sHLH (17/86) after treatment were significantly lower than those before treatment (P<0.01). The serum sFas level in newly diagnosed sHLH patients was positively correlated with SF(r=0.35), sCD25(r=0.79) and sFasL(r=0.73). The serum sFasL level was positively correlated with SF(r=0.39), sCD25(r=0.64) and sFas(r=0.73). Compared with the NonMAHLH group, the area under the ROC curve was 0.707 (95% CI: 0.593-0.821) (P=0.0015). The optimal critical value for diagnosing MAHLH by sFas level was 12 743 pg/ml, and the sensitivity and specificity were 70.9% and 71% respectively. Compared with the NonMAHLH group, the area under the ROC curve was 0.765(95% CI: 0.659-0.87)(P<0.01). The median OS time of sFas high expression group (≥16798.5 pg/ml) and sFasL high expression group (≥4 785 pg/ml) was significantly shorter than that of the low expression group (P<0.001).@*CONCLUSION@#Serum levels of sFas and sFasL can be used for the early diagnosis and differential diagnosis of sHLH disease, and are the factor related to the poor prognosis of sHLH.


Subject(s)
Humans , Lymphohistiocytosis, Hemophagocytic , Clinical Relevance , ROC Curve , Sensitivity and Specificity , Lupus Erythematosus, Systemic
2.
Journal of Experimental Hematology ; (6): 1006-1011, 2020.
Article in Chinese | WPRIM | ID: wpr-827170

ABSTRACT

OBJECTIVE@#To investigate the expression and clinical significance of soluble B7-H3 (sB7-H3) in patients with secondary hemophagocytic lymphohistiocytosis (sHLH).@*METHODS@#The plasma samples of 85 newly diagnosed sHLH patients from December 2012 to April 2018 were collected. The patients were divided into lymphoma-related HLH(LHLH)group and infection-related HLH(IHLH)group. The expression of sB7-H3 in plasma was detected by ELISA, and the clinical data were collected for analysis. Fifteen healthy people were chosen as control group.@*RESULTS@#The expression level of sB7-H3 in lymphoma-related HLH and infection-related HLH group significant increased as compared with the control group, (P<0.05), and the expression level of sB7-H3 in lymphoma-related HLH group was significant higher than that in infection-related HLH group [(35.75± 9.90) vs (28.70±8.95) ng/ml)] (P<0.001). There were no significant statistical difference in the expression of some clinical factors (including age, fever, splenomegaly, ANC, Plt, FIB, calcium ion, serum albumin, LDH, serum ferritin, sCD25) in lymphoma-related HLH and infection-related HLH group (P>0.05). The evaluation of expression and significance of sB7-H3 in sHLH by using ROC curve, showed that the area under ROC curve comparison of patients in lymphoma-related HLH group and infection-related HLH group was 0.718 (95% CI 0.610-0.810) (P=0.0002), and predicting the sensitivity and specificity of the lymphoma-related HLH patients were 77.36% and 59.38%, respectively. The best cut-off value of patients in sB7-H3 was 29.81 ng/ml, the overall survival time of sB7-H3 high-expression group (≥29.81 ng/ml) was significant shorter than that in low-expression group (<29.81 ng/ml) (24 vs 440 d) (P<0.001). The clinical factors affecting the survival status of sHLH patients were neutrophils, albumin, serum ferritin, serum calcium ions and sB7-H3 levels.@*CONCLUSION@#sB7-H3 associates with poor prognosis of sHLH patients, and may be involved in disease progression.


Subject(s)
Humans , Enzyme-Linked Immunosorbent Assay , Lymphohistiocytosis, Hemophagocytic , Lymphoma , ROC Curve
3.
Chinese Journal of Hematology ; (12): 502-506, 2019.
Article in Chinese | WPRIM | ID: wpr-1012021

ABSTRACT

Objective: To investigate the clinical characteristics of secondary hemophagocytic lymphohistiocytosis (sHLH) complicated with capillary leak syndrome (CLS) . Methods: The clinical and laboratory data of 87 sHLH patients, who were treated in our hospital between January 2015 and December 2017, were retrospectively analyzed. Depending on whether they were complicated with CLS, 21 sHLH patients were classified as the CLS-sHLH group, while 66 were classified as the non-CLS-sHLH group. The differences of clinical manifestations, laboratory tests, treatment and prognosis between the two groups were compared. Results: There was no significant difference in the etiology of sHLH between the CLS-sHLH group and the non-CLS-sHLH group (P>0.05) . The neutrophil, fibrinogen and albumin levels in the CLS-sHLH group were lower than those in the non-CLS-sHLH group, while the triacylglycerol levels were higher than those in the non-CLS-sHLH group (P<0.05) . Varying degrees of edema, weight gain, hypotension, hypoproteinemia, oliguria and multiple serous effusions were observed in the CLS-sHLH group. Among them, there were 15 patients that CLS get improved, and the medial time of improvement was 7 (5-14) days. The other 6 patients did not get remission, while they died within 6-30 days. The median overall survival of the CLS-sHLH group was lower than that of the non-CLS-sHLH group (75 days vs not reached, P=0.031) . Conclusions: There may be no correlation between the cause of sHLH and the occurrence of CLS. Severity of neutropenia, fibrinogen and albumin levels, and triglyceride levels may be accompanied for sHLH patients complicated with CLS. Patients with sHLH who complicated with CLS have a poor prognosis. Active treatment of HLH and its primary disease, reasonable fluid replacement and oxygen supply are crucial, which can effectively control disease progression.


Subject(s)
Humans , Capillary Leak Syndrome , Fibrinogen , Lymphohistiocytosis, Hemophagocytic , Prognosis , Retrospective Studies
4.
Journal of Experimental Hematology ; (6): 878-883, 2016.
Article in Chinese | WPRIM | ID: wpr-246851

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the expression levels and clinical significance of serum high mobility group box 1 (HMGB-1) in patients with secondary hemophagocytic lymphohistiocytosis (sHLH).</p><p><b>METHODS</b>Serum HMGB-1 levels were determined by using enzyme linked immunosorbent assays (ELISA) in 51 sHLH patients and 15 healthy contrlols. Other laboratory data, including soluble interleukin-2 receptor (sCD25), white blood cells (WBC), hemoglobin (Hb), platelet (Plt), fibrinogen (FIB), lactate dehydrogenase (LDH), triglyceride (TG), alanine transaminase (ALT), aspartate aminotransferase (AST), serum ferritin (SF), C reactive protein (CRP), and blood sedimentation rate (ESR) were also collected.</p><p><b>RESULTS</b>Serum HMGB-1 levels in the newly diagnosed group were significantly higher than that in the control group (P<0.01). Serum HMGB-1 levels in the newly diagnosed lymphoma-associated HLH (LHLH) group were significantly higher than that in non-HLH group, including infection-associated HLH (IHLH) and autoimmune-associated HLH (AHLH) group (P<0.05); The serum HMGB-1 levels in the clinical remission group were significantly lower than that in the newly diagnosed group (P<0.05), however, serum HMGB-1 was not decreased significantly in the progression/relapsed group, compared with the newly diagnosed group (P>0.05). Serum HMGB-1 levels in newly diagnosed sHLH patients positively correlated with sCD25 (r=0.62, P<0.01) and ESR (r=0.55, P<0.05). The receiver operating characteristic curves (ROC) for serum HMGB-1 levels of sHLH patients and healthy controls produced a cutoff value at 15.3 µg/L, with its 90% sensitivity and 99% specificity, respectively. In addition, an optimal cutoff value for HMGB-1 was 27.4 µg/L in the patients LHLH and non-HLH (AHLH+IHLH) with 96% sensitivity and 81% specificity, separately.</p><p><b>CONCLUSION</b>Serum HMGB-1 levels possesses an important clinically significance for disease diagnosis, differential diagnosis, evaluation of nosographic activity and treatment efficacy in the patients with sHLH.</p>


Subject(s)
Humans , C-Reactive Protein , Case-Control Studies , Diagnosis, Differential , Enzyme-Linked Immunosorbent Assay , Fibrinogen , HMGB1 Protein , Blood , Interleukin-2 Receptor alpha Subunit , Blood , L-Lactate Dehydrogenase , Blood , Leukocytes , Lymphohistiocytosis, Hemophagocytic , Blood , Lymphoma , Sensitivity and Specificity , Treatment Outcome
5.
Journal of Experimental Hematology ; (6): 698-702, 2014.
Article in Chinese | WPRIM | ID: wpr-349645

ABSTRACT

This study was aimed to enhance clinical understanding the effect of nilotinib on CML patients with V299L mutation who were resistant to imatinib. Bone marrow specimens from 2 cases of CML with V299L mutation were collected before and after the treatment with nilotinib. ABL mutation was detected by nested reverse transcription polymerase chain reaction (PCR) followed by direct sequencing. The clinical characteristics of the two cases were analyzed. The results showed that both cases were resistant to imatinib presented V299L mutation. Out of them 1 case achieved complete haematological response (CHR) after treatment with nilotinib for 6 months and another case abstained obvious molecular response after using nilotinib for 7 month. V299L mutation of both cases was turned into negative after the treatment with nilotinib. It is concluded that the nilotinib can safely and effectively override tyrosine kinase inhibitor (TKI) resistance mediated by the V299L mutation. The safety and efficacy of nilotinib for treatment of CML patients with TKI resistance and V299L mutation are satisfactory.


Subject(s)
Adult , Aged , Humans , Male , Benzamides , Pharmacology , Drug Resistance, Neoplasm , Genetics , Imatinib Mesylate , Leukemia, Myelogenous, Chronic, BCR-ABL Positive , Drug Therapy , Genetics , Mutation , Piperazines , Pharmacology , Pyrimidines , Pharmacology , Therapeutic Uses
6.
Journal of Experimental Hematology ; (6): 465-468, 2013.
Article in Chinese | WPRIM | ID: wpr-332757

ABSTRACT

This study was aimed to detect the peripheral blood serum neopterin (Npt) level in the patients with hemophagocytic lymphohistiocytosis (HLH) and to explore its significance in HLH. The enzyme-linked immunosorbent assay (ELISA) was applied to detect the serum Npt level and sCD25 level in 20 HLH patients before and after treatment and 15 healthy controls. The results indicated that the serum Npt and sCD25 levels in HLH patients were significantly higher than those in healthy controls (P < 0.0001). The serum Npt and sCD25 levels in the HLH group decreased significantly after treatment, respectively (P < 0.0001). The correlation analysis of Npt with sCD25 before and after treatment showed that they had significant correlation (r = 0.81, P < 0.05 before treatment; r = 0.65, P < 0.05 after treatment). Meanwhile, the level of serum Npt and ferritin had a significant correlation in newly diagnosed HLH patients (r = 0.55, P < 0.05). It is concluded that the serum Npt may play an important role in the HLH pathogenesis, the enhancement of Npt levels has an important significance for diagnosis and evaluation for HLH.


Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Case-Control Studies , Enzyme-Linked Immunosorbent Assay , Lymphohistiocytosis, Hemophagocytic , Blood , Diagnosis , Neopterin , Blood
7.
Journal of Experimental Hematology ; (6): 1121-1125, 2013.
Article in Chinese | WPRIM | ID: wpr-283969

ABSTRACT

This study was purposed to investigate the molecular mechanism of 4-1BBL reverse signals in the human acute monocytic leukemia cell line of U937. The U937 cell line was used as target cells, and stimulated by the mouse anti-human 4-1BBL monoclonal antibody 1F1. The nuclear translocation of NF-κB and the co-location of 4-1BBL and CD28i molecules in U937 cells were observed with confocal laser scanning microscopy. The protein and m-RNA expression levels of 4-1BBL and CD28i were detected by flow cytometry and RT-PCR respectively. The results showed that the significant nuclear translocation of NF-κB and co-localization of 4-1BBL and CD28i on membrane of U937 cells appeared after being stimulated by mAb1F1. It is concluded that the 4-1BBL reverse signals transduction mediating the growth of U937 cells relates with the nuclear translocation of NF-κB. CD28i may be involved in intracellular 4-1BBL reverse signaling pathways.


Subject(s)
Humans , 4-1BB Ligand , Allergy and Immunology , Metabolism , Antibodies, Monoclonal , Pharmacology , CD28 Antigens , Metabolism , Coculture Techniques , NF-kappa B , Genetics , Signal Transduction , U937 Cells
8.
Chinese Journal of Hematology ; (12): 1024-1027, 2013.
Article in Chinese | WPRIM | ID: wpr-295748

ABSTRACT

<p><b>OBJECTIVE</b>To investigate SRSF2 mutations in patients with chronic myelomonocytic leukemia (CMML) and the clinical characteristics of patients with SRSF2 mutants.</p><p><b>METHODS</b>In this study, the frequency of SRSF2 mutation in a cohort of 20 patients with CMML was detected by polymerase chain reaction (PCR) followed by direct sequencing to couple with their clinical features.</p><p><b>RESULTS</b>Of 20 patients, 4 patients were found harboring SRSF2 mutations, including 2 P95L, 1 P95H and 1 P95R point mutations. There were no significantly statistical differences in terms of their clinical characteristics between mutant and wild type group.</p><p><b>CONCLUSION</b>SRSF2 mutation was not frequently occurred in CMML patients and might associated with poor prognosis. It might be a practically diagnostic maker and therapeutic target in CMML.</p>


Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , DNA Mutational Analysis , Genotype , Leukemia, Myelomonocytic, Chronic , Genetics , Mutation , Nuclear Proteins , Genetics , Prognosis , Ribonucleoproteins , Genetics , Serine-Arginine Splicing Factors
9.
Journal of Experimental Hematology ; (6): 401-404, 2012.
Article in Chinese | WPRIM | ID: wpr-263382

ABSTRACT

This study was aimed to detect the level of soluble interleukin-2 receptor (sCD25) and cytotoxic activity of NK lymphocytes in patients with hemophagocytic lymphohistiocytosis (HLH), and to explore their clinical significance in HLH. The enzyme-linked immunosorbent assay was used to detect the sCD25 level in serum of 20 patients with HLH, 15 healthy controls, 20 cases of acute myeloid leukemia and 20 cases of systemic lupus erythematosus. The NK cell cytotoxicity in peripheral blood of patients with HLH and controls were detected by flow cytometry with CD107a antibody labeling and LDH release assay. The results indicated that the level of sCD25 in HLH patients was significantly higher than that in healthy controls and disease groups (P < 0.001). The NK cell cytotoxicity in peripheral blood detected by both methods in patients with HLH were lower than that in healthy controls (P < 0.05), and the results detected by flow cytometry correlated significantly with those by LDH release assay (r = 0.73, P < 0.05). It is concluded that detection of sCD25 levels and NK cell activity in peripheral blood in HLH is of great value. Using flow cytometry following CD107a antibody labeling to measure NK activity is a simple, stability, reproducibility method and can be used for clinical diagnosis of HLH.


Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Case-Control Studies , Interleukin-2 Receptor alpha Subunit , Blood , Killer Cells, Natural , Metabolism , Lupus Erythematosus, Systemic , Blood , Lymphohistiocytosis, Hemophagocytic , Blood , Diagnosis
10.
Journal of Experimental Hematology ; (6): 1110-1116, 2012.
Article in Chinese | WPRIM | ID: wpr-278425

ABSTRACT

This study was purposed to investigate the incidence of mixed lineage leukemia (MLL) gene rearrangement and partner gene types as well as the clinical features and prognosis of acute leukemia (AL) with this rearrangement through detection in adult AL using combination of 3 techniques, and to evaluate the clinical value of this combination detection. The MLL gene rearrangement in 183 cases of adult AL was detected by combination of conventional cytogenetics, split signal FISH and multiplex nested PCR. The results showed that the incidence of MLL rearrangements in adult patients with AL was low (8.2%), and MLL-AF4 fusion gene was most common and predominant in acute lymphoblastic leukemia (ALL), while the MLL-AF6 and MLL-AF9 were most frequent in acute myeloid leukemia (AML). Extramedullary involvements were found in 40% of MLL-rearranged AL patients, and 33.3% of patients with MLL-rearranged AL reached to complete remission within 30 days during induction chemotherapy. In addition, in this cohort of MLL-rearranged adult AL patients, the 3-month relapse rate and 6-month overall survival rate were 50.0% and 50.0% respectively. It is concluded that the rate of missed diagnosis of CC technique for patients with MLL-rearranged AL reached to 60% in this study, while the combination of CC, FISH and multiplex nested PCR has been confirmed to have important significance for evaluating prognosis and conducting clinical therapy of patients with MLL-rearranged AL.


Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Gene Rearrangement , Leukemia, Myeloid, Acute , Genetics , Myeloid-Lymphoid Leukemia Protein , Genetics , Oncogene Proteins, Fusion , Genetics
11.
Chinese Journal of Hematology ; (12): 628-631, 2012.
Article in Chinese | WPRIM | ID: wpr-278353

ABSTRACT

<p><b>OBJECTIVE</b>To explore the effectivity and safety of single high-dose (HD) etoposide (Vp16) with granulocyte colony-stimulating factor (G-CSF) for mobilization of autologous peripheral blood stem cells (PBSC) in patients with hematologic malignancies.</p><p><b>METHODS</b>80 patients of hematologic malignancies including 20 patients with acute leukemia (AL), 23 with multiple myeloma (MM), 35 with non-Hodgkin's lymphoma (NHL) and 2 with Hodgkin's lymphoma (HL) received Vp16 (1.6 g/m(2)) continuous intravenous infusion for 10 hrs on day 1. G-CSF at 10 µg/kg once daily subcutaneous injection began to use on day of ANC lower than 1×10(9)/L and continued until PBSC collection was completed. Autologous PBSC (APBSC) was collected on day of WBC greater than 5×10(9)/L and continuing until the collection goal was met (target value: MNC ≥ 6.0×10(8)/kg and CD34(+) ≥ 2.0×10(6)/kg). The patients received APBSC after conditioning regimen. The number of the cells collection, time of hematopoietic reconstruction, adverse effect and so on were observed during the course of stem cell mobilization and collection.</p><p><b>RESULTS</b>PBSC was collected on day 11 (range: 7 - 25 days) of after Vp16 administration with a median collection time of 2 (range 1 - 5). 3/80 patients with AML got stem cell mobilization failure. 5 of 6 patients who failed to mobilize before got successful stem cell mobilization, 1/6 patient with AML-M(5) got a second failure after the mobilization of VP16 whose first time's mobilization using Ara-C did not succeed. The median number of CD34(+) cells collected in 77 patients who got successful mobilization was 4×10(6)/kg \[range (1.59 - 24.68)×10(6)/kg\]. The collection of 20 patients with AL and 23 with MM were got detection for minimal residual disease, no pollution of tumor cells were happened. All patients could tolerate the whole course of stem cell mobilization. 29/80 (36.25%) patients got a 4 grade leucopenia, 19/80 (23.75%) patients got infection.</p><p><b>CONCLUSION</b>Single high-dose etoposide with G-CSF for mobilization of APBSC has a higher achievement ratio, a controllable adverse effect, a promising hematopoiesis recovery, which is an effective and safe mobilizing regimen for patients with hematologic malignancies.</p>


Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Etoposide , Therapeutic Uses , Granulocyte Colony-Stimulating Factor , Therapeutic Uses , Hematologic Neoplasms , Hematopoietic Stem Cell Mobilization , Methods , Peripheral Blood Stem Cell Transplantation
12.
Journal of Experimental Hematology ; (6): 983-986, 2011.
Article in Chinese | WPRIM | ID: wpr-261945

ABSTRACT

The aim of this study was to explore cytogenetic characteristics of acute promyelocytic leukemia (APL) and compare the interphase fluorescence in situ hybridization (I-FISH) with conventional cytogenetic (CC) analysis. A total number of 157 APL patients were recruited in this study, and the I-FISH and CC were applied to analyze cytogenetic features. Chromosome samples of bone marrow cells were prepared by short-term culture. Out of all 157 cases, 136 were observed with CC assay, 66 with I-FISH, of which 45 samples were analyzed with both methods. The results showed that among all 136 CC samples, t(15;17)(q22;q21) was found in 120 cases, of which 107 cases was isolated t(15;17)(q22;q21) abnormality, 13 cases was complex abnormalities and 12 case without mitotic figure. Among all 66 cases of I-FISH group, PMI/RARα fusion gene was found in 64 cases (97.0%), suggesting that I-FISH group was more sensitive than CC group (p = 0.041). It is concluded that combination of I-FISH and CC techniques plays a pivotal role for diagnosis and detection of minimal residual disease in APL.


Subject(s)
Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Female , Humans , Infant , Male , Middle Aged , Young Adult , Cytogenetic Analysis , Methods , In Situ Hybridization, Fluorescence , Methods , Karyotyping , Leukemia, Promyelocytic, Acute , Diagnosis , Genetics
13.
Journal of Experimental Hematology ; (6): 1511-1514, 2010.
Article in Chinese | WPRIM | ID: wpr-332328

ABSTRACT

This study was aimed to investigate the therapeutic efficacy and adverse events of bortezomib-based chemotherapy for 40 patients with multiple myeloma. 16 newly diagnosed patients and 11 patients with refractory/relapse myeloma were treated with bortezomib, dexamethasone and thalidomide; 7 newly diagnosed patients and 4 patients with refractory/relapse myeloma were treated with bortezomib and dexamethasone; 2 newly diagnosed patients were treated with bortezomib, melphalan and thalidomide. Cycles were repeated every 28 or 35 days, all the patients were treated for 2 to 8 cycles. The therapeutic efficacy and adverse events were evaluated according to International Myeloma Working Group Uniform Response Criteria. The results indicated that the median follow-up duration was 13 months, the total response rate was 72.5%, among which 16 patients achieved complete response (CR), 13 achieved partial response (PR). The main side effects included gastrointestinal symptoms, peripheral neuropathy, thrombocytopenia, respiratory infection, herpes zoster and urinary retention and so on. The adverse events were ameliorated by treatment and decrease of the bortezomib dose. It is concluded that bortezomib-based chemotherapy is effective in the treatment of either newly diagnosed or refractory/relapse MM patients and the adverse events are tolerable and manageable for patients.


Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Antineoplastic Combined Chemotherapy Protocols , Therapeutic Uses , Boronic Acids , Bortezomib , Multiple Myeloma , Drug Therapy , Pyrazines , Treatment Outcome
14.
Journal of Experimental Hematology ; (6): 1525-1530, 2010.
Article in Chinese | WPRIM | ID: wpr-332325

ABSTRACT

This study was aimed to investigate the therapeutic efficacy of HLH-2004 chemotherapy in patients with secondary hemophagocytic lymphohistiocytosis (sHLH). 10 cases of sHLH treated with HLH-2004 regimen at our department were analyzed retrospectively. The results showed that 7 patients had clinical response to HLH-2004 regimen, while other 3 patients had no clinical response. 5 cases did not complete initial therapy of 8 weeks. Out of 5 cases, 4 died in the process of chemotherapy, 1 patient abandoned for serious side effects but finally acquired remission following 4 cycles of CHOP regimen. 5 cases underwent the whole courses of initial therapy. Out of 5 cases, 3 patients acquired remission, and other 2 were not well controlled. Out of the 3 patients who had achieved remission, one died of relapse, and other 2 patients kept remission. Out of the 2 patients who were not well controlled, one patient died, but another patient acquired remission after being discharged. It is concluded that patients with infection-associated hemophagocytic syndrome (IAHS) have high rates of remission after receiving HLH-2004 regimen combining with effective antibiotics. However, patients with HLH secondary to EBV (EBV-HLH) or lymphoma (LAHS) have low rates of remission or are easy to get relapse after remission.


Subject(s)
Adult , Female , Humans , Male , Middle Aged , Young Adult , Antineoplastic Combined Chemotherapy Protocols , Therapeutic Uses , Cyclophosphamide , Therapeutic Uses , Doxorubicin , Therapeutic Uses , Lymphohistiocytosis, Hemophagocytic , Drug Therapy , Prednisolone , Therapeutic Uses , Retrospective Studies , Treatment Outcome , Vincristine , Therapeutic Uses
15.
Journal of Experimental Hematology ; (6): 859-862, 2010.
Article in Chinese | WPRIM | ID: wpr-237636

ABSTRACT

In order to evaluate the incidence of CCAAT/enhancer binding protein alpha (cebpa) gene mutation in patients with acute myeloid leukemia (AML), 22 AML patients with normal karyotype (NK-AML) were enrolled in this study, including de novo AML and relapsed AML. The cebpa gene was amplified by 2 stages using genomic DNA as template, the cebpa gene mutation amplified product was detected by direct sequencing or clone sequencing. The results showed that the cebpa mutations including deletion and insertion were found in 4 out of 22 AML patients (18.2%) and all of these 4 patients were M(2). Two patients had N-terminal nonsense mutation and the other two had C-terminal in-frame mutation. It is concluded that PCR combined with direct sequencing and clone sequencing can be used to detect cebpa mutations. cebpa mutations are mainly identified in M(2) subtype of NK-AML patients, its significance for prognosis needs to further investigate.


Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , CCAAT-Enhancer-Binding Protein-alpha , Genetics , Karyotyping , Leukemia, Myeloid, Acute , Genetics , Pathology , Mutation , Neoplasm Staging
16.
Journal of Experimental Hematology ; (6): 469-472, 2010.
Article in Chinese | WPRIM | ID: wpr-243332

ABSTRACT

This study was aimed to investigate the relationship between cytogenetic evolution and disease progression in patient with MDS-RAEB. By a long term (6 years) follow-up of a patient with MDS-RAEB, peripheral blood cell count, bone marrow cell morphology and conventional cytogenetics were monitored regularly. In addition, fluorescence in situ hybridization (FISH) was applied to confirm the aberrant karyotype. The results indicated that this patient was failed with conventional chemotherapy of AML, but had response to ATRA and 6-MP in the 72 months follow-up. At initial diagnosis, the cytogenetics analysis showed normal karyotype, whereas 46, XY, 2q+[1]/46, XY[19] was found at 48 months, 46, XY, dup(1q)[3]/46, XY[7] at 56 months, and dup (1) as well as der (11) with complex karyotype at 68 months, which was accompanied by progressive decrease of platelet count. It is concluded that karyotype evolution is perhaps associated with progression of MDS.


Subject(s)
Adult , Humans , Male , Middle Aged , Chromosome Aberrations , Chromosomes, Human, Pair 1 , Chromosomes, Human, Pair 11 , Follow-Up Studies , Karyotyping , Myelodysplastic Syndromes , Genetics
17.
Chinese Journal of Hematology ; (12): 22-25, 2009.
Article in Chinese | WPRIM | ID: wpr-314517

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the efficacy and toxicity of standard-dose IA regimen (idarubicin and cytarabine) as induction therapy followed by FLAG regimen in patients with acute myeloid leukemia (AML), and its influence on peripheral stem cell mobilization.</p><p><b>METHODS</b>A total of 23 previously untreated de novo AML patients were enrolled. Thirteen patients were male, and 10 female, with ages ranging from 14 to 54 (median: 41) years. Cytogenetic analysis was performed for all patients. The IA regimen contained idarubicin (12 mg x m(-2) x d(-1), days 1 to 3) and cytarabine (100 mg x m(-2) x d(-1), days 1 to 7), and the FLAG regimen contained'fludarabine (50 mg/d, days 1 to 5), cytarabine (2 g x m(-2) x d(-1), days 1 to 5) and granulocyte colony-stimulating factor (G-CSF, 300 microg/d, days 0 to 5).</p><p><b>RESULTS</b>After one course of induction therapy, the CR rate was 91.3%. The CR rate for patients with favourable and intermediate prognostic karyotypes was 100% and 91.3%, respectively. Nineteen patients in CR were consolidated with FLAG regimen, of which 6/9 (66.7%) patients were able to mobilize a sufficient number of CD34+ cells and successfully performed autologous stem cell transplantation. Four patients relapsed. The median survival duration was 19.5 months and median disease-free survival was 14 months. Myelosuppression and infections due to neutropenia were the most frequent adverse effects, severe nonhematologic toxicity and the early death were not observed in all patients.</p><p><b>CONCLUSION</b>IA followed by FLAG regimen is effective and well tolerable in AML patients especially in those with favourable and intermediate prognostic karyotypes, and 1 to 2 courses of this therapy shows no influence on peripheral stem cell mobilization and subsequent autologous stem cell transplantation.</p>


Subject(s)
Adolescent , Adult , Female , Humans , Male , Middle Aged , Young Adult , Antineoplastic Combined Chemotherapy Protocols , Therapeutic Uses , Leukemia, Myeloid, Acute , Drug Therapy , Prognosis , Treatment Outcome
18.
Chinese Journal of Medical Genetics ; (6): 443-445, 2009.
Article in Chinese | WPRIM | ID: wpr-349057

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the incidence of trisomy 8 in chronic lymphocytic leukemia (CLL) and its significance in prognosis.</p><p><b>METHODS</b>A panel of probes and fluorescence in situ hybridization (FISH) were used to detect trisomy 8 in 151 CLL patients combined with chromosome karyotype analysis.</p><p><b>RESULTS</b>There were 2 patients (1.3%) with trisomy 8 in the 151 CLL patients, and the number of trisomy 8 cells was 8% and 10% respectively. The karyotypes were 47,XY,+8[2]/49,XY,+14,+20,+21[2]/ 46,XY[16], and 47,XX,+8[2]/46,XX[18], respectively.</p><p><b>CONCLUSION</b>Trisomy 8 was rare in CLL, and its significance in prognosis of CLL still remains unknown.</p>


Subject(s)
Adult , Aged , Aged, 80 and over , Humans , Male , Middle Aged , Chromosomes, Human, Pair 8 , Genetics , Karyotyping , Leukemia, Lymphocytic, Chronic, B-Cell , Diagnosis , Genetics , Prognosis , Trisomy
19.
Journal of Experimental Hematology ; (6): 1047-1051, 2009.
Article in Chinese | WPRIM | ID: wpr-343352

ABSTRACT

This study was aimed to explore the effect and adverse reaction of cyclosporine A (CsA) in treatment of pancytopenia without reticulocyte decrease but with elevated LDH. 10 patients were selected according to our standards and were treated by CsA. The curative effect and adverse reaction of patients were evaluated by following up for 6 - 116 months. The effect of CsA in the maintenance treatment of AA patient with an obvious rise of LDH was illustrated by means of typical case. The results indicated that 10 patients had different diagnosis, but had similar clinical and laboratory characteristics. Among them, 9 patients showed a relatively good curative reaction to CsA. Treatment of 1 patient was stopped because of pneumonia tuberculosis. It is concluded that the elevated LDH without reticuleocyte decrease may be a biomarker to predict the curative reaction to CsA for patients with pancytopenia. Selectively treating pancytopenia with CsA can obtain a higher curative reaction and maintenance treatment with CsA is an important factor for reducing recurrence of this disease.


Subject(s)
Adolescent , Adult , Female , Humans , Male , Middle Aged , Young Adult , Cyclosporine , Therapeutic Uses , Immunosuppressive Agents , Therapeutic Uses , Pancytopenia , Drug Therapy , Treatment Outcome
20.
Journal of Experimental Hematology ; (6): 1335-1338, 2009.
Article in Chinese | WPRIM | ID: wpr-343291

ABSTRACT

This study was aimed to investigate the effect of FLAG consolidatory therapy on peripheral blood stem cell (PBSC) mobilization in patients with acute myelogenous leukemia (AML) for autologous PBSC transplantation. A total of 15 AML patients were enrolled in this study. 10 patients were male, and 5 were female, with ages ranging from 14 to 51 (median 36) years. Out of 15 patients 13 were newly diagnosed, and 2 were refractory/relapsed AML. All patients were consolidated with FLAG regimen which including fludarabine 50 mg/d, days 1-5; Ara-C 2 g/(m2.d), days 1-5; G-CSF 300 microg/d, injection subcutaneously starting 24 hours before Ara-C and continuing until neutrophil count exceeding 1.0x10(9)/L. The harvest of the stem cells was performed after hematologic recovery from the second or third course of FLAG consolidation, or mobilized by high dose etoposide (1.6 g/m2). The results showed that among 15 patients scheduled for PBSC harvest, 11 (73.3%) harvested a median of 3.52x10(6)/kg CD34+ cells (range 2.2-4.6) and underwent autologous transplantation, while the minimal number of CD34+ cells could not be reached in the remaining 4 patients. It is concluded that the FLAG regimen is effective and well-tolerated treatment as consolidation regimen in AML, which does not influence PBSC mobilization and autologous transplantation after 2 courses of FLAG.


Subject(s)
Adolescent , Adult , Female , Humans , Male , Middle Aged , Young Adult , Antineoplastic Combined Chemotherapy Protocols , Therapeutic Uses , Cytarabine , Therapeutic Uses , Granulocyte Colony-Stimulating Factor , Therapeutic Uses , Hematopoietic Stem Cell Mobilization , Leukemia, Myeloid, Acute , Drug Therapy , Therapeutics , Peripheral Blood Stem Cell Transplantation , Methods , Transplantation, Autologous , Vidarabine , Therapeutic Uses
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