Mucormycosis in patients with hematological diseases: seven cases reports and literature review / 中华血液学杂志

Objective@#To explore the clinical characteristics, diagnosis, treatment and prognosis of patients with hematological diseases that complicated by mucormycosis, and to improve the understanding and clinical diagnosis and treatment of the disease.@*Methods@#The clinical data of 7 patients suffering from mucormycosis during September 2012 and September 2018 were retrospectively analyzed, and their clinical characteristics, treatment process and prognosis were analyzed.@*Results@#Of 7 patients, there were 4 males and 3 females, with a median age of 36 (19-79) years old. Two patients were diagnosed as acute myeloid leukemia as the underlying disease, the other 5 patients suffered from acute B lymphoblastic leukemia, peripheral T cell lymphoma, chronic myelocytic leukemia in blastic phase, myeloproliferative neoplasm and severe aplastic anemia after transplantation, respectively. Among them, disease types of mucormycosis were pulmonary in 4 patients, rhino-orbital-cerebral in 1 patient, cutaneous in 1 patient and disseminated in 1 patient. All the cases were confirmed by biopsy histopathology. The treatment drugs were amphotericin B or liposomal amphotericin B, and posaconazole. Surgical treatment was performed in 4 patients, 3 out of 4 achieved radical debridement, and the other one had local debridement. Two patients were cured, 1 patient was improved and 4 patients died.@*Conclusions@#The clinical manifestation and image feature of mucormycosis in patients with hematological diseases were diverse, and the mortality rate is high, diagnosis mainly depends on histopathology. Early diagnosis, control of underlying disease, improvement of immunosuppressive status, timely effective antifungal therapy and radical surgical debridement are the key points for improving the survival rate of patients with hematological diseases complicated by mucormycosis.

The usage of comprehensive geriatric assessment in elderly patients with acute myeloid leukemia: a multicenter, prospective study / 中华血液学杂志

Objective@#To evaluate the feasibility and potential value of comprehensive geriatric assessment (CGA) in elderly (≥60 years) patients with newly diagnosed acute myeloid leukemia (AML) in China.@*Methods@#The CGA results of 83 newly diagnosed AML (non-APL) patients from 16 hospitals in Beijing and Tianjin between March 2016 and December 2017 were prospectively collected and analyzed. The clinical data, treatment and follow-up information were also collected.@*Results@#Of 83 newly diagnosed elderly AML patients, 81 patients (97.6%) completed all designated CGA assessment. The median number of impaired scales of the CGA assessment in the studied population was 2(0-6). Sixteen patients (19.3%) showed no impairments according to the geriatric assessment scales implem ented by this study. The distributions of impaired scales were as follows: impairment in ADL, 55.4%; IADL impairment, 42.2%; MNA-SF impairment, 48.2%; cognitive impairment, 15.7%; GDS impairment, 31.7%; HCT-CI impairment, 19.5%, respectively. In patients with "good" ECOG (n=46), the proportion of impairment for each CGA scale ranged from 6.5% to 37.0% and 32 patients (68.9%) had at least one impaired CGA scale. Survival analysis showed that the number of impaired scales of the CGA was significantly correlated with median overall survival (P=0.050).@*Conclusions@#CGA was a tool with feasibility for the comprehensive evaluation in elderly AML patients in China. Combined with age and ECOG, CGA may be more comprehensive in assessing patients’ physical condition.

Efficacy and safety of CD19 chimeric antigen receptor T cells for the treatment of 22 patients with B-cell lymphoma / 中华血液学杂志

Objective@#To investigate the efficacy and safety of CD19 chimeric antigen receptor T (CAR-T) lymphocytes for the treatment of B cell lymphoma.@*Methods@#A total of 22 patients with B-cell lymphoma from February 1, 2017 to July 1, 2018 were reviewed to evaluate the efficacy and adverse reactions of CD19 CAR-T.@*Results@#Of 22 patients with B-cell lymphoma received CD19 CAR-T cells, the median dose of CAR-T cells was 7.2 (2.0-12.0) ×106/kg. Nine of 12 cases of relapse refractory patients were overall response. Complete remission (CR) occurred in 2 of 12 patients, partial remission (PR) in 7 of 12 patients. The overall response in minor residual disease positive (MRD) group was 8 of 10 patients. CD19 CAR-T cells proliferated in vivo and were detectable in the blood of patients. The peak timepoints of CAR-T cells proliferated in the relapsed refractory and MRD positive groups were 12 (5-19) and 4.5 (1-12) days after treatment respectively, and among peripheral blood cells, CAR-T cells accounted for 10.10% (3.55%-24.74%) and 4.02% (2.23%-28.60%) of T lymphocytes respectively. The MRD positive patients achieved sustained remissions during a median follow-up of 8 months (rang 3-18 months) . None of all the patients relapsed during a median follow-up time of 10 months (3-18 months) . However, 7 PR responders of the relapsed refractory patients maintained a good condition for 1.5-6.0 months. One patient bridged to hematopoietic stem cell transplantation, another one sustained remission for 12 months. Cytokine-release syndrome (CRS) occurred in 14 patients with grade 1-2 CRS in MRD positive group and grade 3 CRS in relapsed refractory group.@*Conclusions@#CAR-T cell therapy not only played a role in the rescue treatment of relapsed and refractory patients, but also produced a surprising effect in the consolidation and maintenance of B-cell lymphoma. CD19 CAR-T cells might be more effective in the treatment of MRD positive B-cell lymphoma patients than in the refractory or relapsed cases. High response rate was observed with fewer adverse reactions.

Effect of PD-1 inhibitor Nivolumab on the proliferation and cytotoxicity of anti-CD19 chimeric antigen receptor T cells / 中华血液学杂志

Objective@#To Evaluation the effect of PD-1 inhibitor Nivolumab on the proliferation and cytotoxicity of anti-CD19 chimeric antigen receptor T cells (CD19-CAR-T) in vitro.@*Methods@#Five patients with high PD-1 expression in peripheral blood and five healthy volunteers were selected. These peripheral blood mononuclear cells were used as the source of T cells to prepare CD19-CAR-T cells. Different doses (72, 36, 18 μg/ml) of Nivolumab was added on day 8 to the culture medium. Patient T cells incubated with 72 μg/ml Nivolumab and CD19-CAR-T cells of healthy volunteers were used as controls. CCK-8, lactate dehydrogenase (LDH) cytotoxicity assay and ELASA were used to detect the proliferation capacity, the specific cytotoxicity and the inflammatory factor secretion.@*Results@#①T cells from patients with high expression of PD-1 as the source of CD19-CAR-T cells did not affect transfection rate compared with that of healthy volunteers [(32.80±7.22)% vs (35.10±5.84)%, t=-0.554, P=0.593]. ②Incubation of CD19-CAR-T cells with 72 μg/ml Nivolumab did not affect CD19-CAR-T cell proliferation, but its cytotoxicity was significantly higher than that of CD19-CAR-T cells alone or patients’ T cells +72 μg/ml Nivolumab (all P<0.001), there was no significant difference in the killing activity between the 72 μg/ml and 36 μg/ml Nivolumab treated CD19-CAR-T cells on Pfeiffer cells (P=0.281, 0.267, respectively), and they were all higher than those of 18 μg/ml Nivolumab treated CD19-CAR-T cells (all P<0.001). ③Different doses of PD-1 inhibitor Nivolumab combined with CD19-CAR-T cells does not affect the secretion of IFN-γ and IFN-α (all P>0.05).@*Conclusion@#Combination of 36 μg/ml PD-1 inhibitor and CD19-CAR-T cells could reduce the drug toxicity and enhance the cytotoxicity.

Long Term Effects Observation on Acupuncture of Liji Therapy for Primary Dysmenorrhea of Cold-damp Stagnation Type / 世界科学技术-中医药现代化

Objective: To evaluate the long term efficacy of treating the primary dysmenorrhea of cold-damp stagnation type by Acupuncture of Liji therapy. Methods: In this study, a total of 76 cases of primary dysmenorrhea of cold-damp coagulation type were randomly divided into the acupuncture of Liji therapy group, and the body acupuncture group, with38 cases in each group. Both groups were continuously treated 3 menstrual cycles, and followed up in the third and six menstrual cycles after the end of the treatment. Visual analogue scale for abdominal pain and Dysmenorrhea Symptoms scale were used as therapeutic indexes. Remove shedding cases, the long-term effects and scores of the 2 groups were compared. Besides, untoward and side effects needed to be recorded. Results: There were 2 cases lost in the acupuncture ofLiji therapy group and 1 cases in the body acupuncture group. After treatment, The clinical comprehensive efficacy of acupuncture ofLiji therapy group was better than that of body acupuncture group (P ＜ 0. 05) . Both groups of VAS scores and dysmenorrhea symptom scores were decreased to different degrees during treatment and follow-up period (P ＜ 0.05) .The follow-up data of the body acupuncture group after six menstrual cycles were higher than that of the third menstrual cycles after treatment. Compared with the two groups, the acupuncture of Liji therapy group was superior to the body acupuncture group during the third menstrual cycles follow-up (P ＜ 0.05) and six menstrual cycles follow-up (P ＜ 0.01) .The treatment satisfaction of acupuncture of Liji therapy and body acupuncture was 91.67％ and 72.97％. There were no adverse reactions in the two groups during the study period. Conclusion: Acupuncture of Liji therapy can effectively relieve dysmenorrhea symptoms and the general discomfort caused by dysmenorrhea, the long-term effect is stable and durable. primary dysmenorrhea of cold-damp stagnation type with acupuncture of Liji therapy has definitely long term curative effect. In addition, the treatment of patients with acupuncture of Liji therapy is more satisfactory, it is worthy of cilnlcal application.

Mechanism of ferroptosis and its relationship with tumors / 国际肿瘤学杂志

Ferroptosis is a new type of cell death discovered in recent years.This form of irondependent cell death is morphologically and biochemically distinct from other cell death modalities,including apoptosis and necrosis.This process is accompanied by the depletion of glutathione,a decrease in glutathione peroxidase activity and the accumulation of lipid peroxidation products.Studies have found that ferroptosis can be involved in the occurrence and progression of tumor by activating different regulatory sites in the signaling pathways,which can promote tumor cell death.Therefore,to clarify ferroptosis pathway and its related mechanisms regulating tumor development may provide new ideas for clinical treatment of tumors.

The specific cytotoxicities of chimeric antigen receptor-engineered T cells on different lymphomas / 中华血液学杂志

Objective@#To investigate the specific cytotoxicities of the second and third generations of chimeric antigen receptor (CAR) -engineered T cells (CAR-T) on different lymphomas.@*Methods@#CAR-Ts were prepared by lentivirus packaging and infection of T cells. CCK-8, ELISA and Lactate dehydrogenase cytotoxicity assay were applied to detect the proliferation capacity, the secretion level of inflammatory factor and the specific cytotoxicity. Flow cytometry assay showed the specific cytotoxicity and residual level of CAR-T in lymphomas of treated mice.@*Results@#The results showed that the third generation CAR-T had greater capacity of the specific cytotoxicity and proliferation capacity than of the second generation CAR-T. But there was no prominent change of the secretion level of inflammatory factor. The specific cytotoxicity of the second generation CAR-T on highly aggressive lymphomas Raji was more prominent than in inert EHEB, but also could achieve satisfactory effect. The tumor burden in the mice injected with Raji was lower than in the mice injected with EHEB from nude mice experiment. But the residual level of CAR-T in the EHEB-injected mice was higher than in the Raji-injected ones. So the second generation CAR-T was more suitable for the treatment of indolent lymphoma.@*Conclusion@#The second generation CD19 CAR-T could treat aggressive lymphoma in a relatively short period, while the second generation CD19 CAR-T need a longer time in vivo to achieve satisfactory curative effect on the noble lymphoma.

Current strategies in the diagnosis and management of chronic neutrophilic leukemia / 中华医学杂志(英文版)

<p><b>OBJECTIVE</b>To review the implications for diagnosis, pathogenesis and potential for new therapeutic option for chronic neutrophilic leukemia (CNL).</p><p><b>DATA SOURCES</b>Data cited in this review were obtained mainly from PubMed and Medline from 1993 to 2013 and highly regarded older publications were also included. The terms "chronic neutrophilic leukemia" and "diagnosis" were used for the literature search.</p><p><b>STUDY SELECTION</b>We identified, retrieved and reviewed the information on the clinical and laboratory features, the new genetic findings, prognosis and disease evolution and management of CNL.</p><p><b>RESULTS</b>The discovery of high-frequency granulocyte-colony stimulating factor receptor (CSF3R) mutations in CNL identifies a new major diagnostic criterion, and lends more specificity to the World Health Organization (WHO) diagnostic criteria for CNL, which are variably applied in routine clinical practice.</p><p><b>CONCLUSIONS</b>In patients for whom the cause of neutrophilia is not easily discerned, the incorporation of CSF3R mutation testing can be a useful point-of-care diagnostic to evaluate the presence of a clonal myeloid disorder, as well as providing the potential for genetically informed therapy. The oncogenic CSF3R mutations are molecular markers of sensitivity to inhibitors of the SRC family-TNK2 and JAK kinases and may provide a new avenue for therapy.</p>

Effects and mechanism of iron overload on hematopoiesis in mice with bone marrow injury / 中华血液学杂志

<p><b>OBJECTIVE</b>To explore effects of iron overload on hematopoiesis in mice with bone marrow injury and its possible mechanism (s).</p><p><b>METHODS</b>C57BL/6 mice were divided into control, iron, irradiation, irradiation+iron groups. The iron-overloaded model of bone marrow injury was set up after mice were exposed to the dose of 4 Gy total body irradiation and (or) were injected iron dextran intraperitoneally. Iron overload was confirmed by observing iron deposits in mice and bone marrow labile iron pool. Additionally, the number of peripheral blood and bone marrow mononuclear cells and the frequency of erythroid cells and myeloid cells were counted and hematopoietic function was assessed.</p><p><b>RESULTS</b>(1)Iron overload occurred by bone marrow biopsy and flow cytometry analysis. (2)Compared with control group, the number of platelets [(801.9±81.2)×10⁹/L vs (926.0±28.2)×10⁹/L] and BMMNC and the frequency of erythroid cells and myeloid cells decreased. Moreover, hematopoietic colony forming units and single-cell cloning counts decreased significantly in irradiation group (P<0.05). (3)Compared with irradiation group, the number of platelets [(619.0±60.9)×10⁹/L vs (801.9±81.2)×10⁹/L] and the frequency of erythroid cells and myeloid cells decreased; moreover, hematopoietic colony forming units and single-cell cloning counts decreased significantly in irradiation+iron group (P<0.05). (4)Compared with irradiation group, ROS level increased by 1.94 fold in BMMNC, 1.93 fold in erythroid cells and 2.70 fold in myeloid cells, respectively (P<0.05).</p><p><b>CONCLUSION</b>The dose of 4 Gy total body irradiation caused bone marrow damage and iron overload based on this injury model, which could damage bone marrow hematopoietic function aggravatingly. And further study found that iron overload was closely related to increased ROS level in BMMNC. The findings would be helpful to further study the injury mechanism of iron overload on the hematopoiesis of bone marrow.</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>

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>

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 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>

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>

Study on the clinical features of patients with aplastic anemia-paroxysmal nocturnal hemoglobinuria syndrome and typical paroxysmal nocturnal hemoglobinuria / 中国实用内科杂志

Objective To explore the differences of clinical features and relationship between aplastic anemia paroxysmal nocturnal hemoglobinuria syndrome(AA PNH syndrome)and typical paroxysmal nocturnal hemoglobinuria(t PNH).Methods A case control study on the discrepancies of clinical and laboratory features between patients with AA PNH syndrome and t PNH was carried out.Results Compared with t PNH,AA PNH syndrome showed following features:①Lower frequencies of venous thrombosis,jaundice and enlarged liver or spleen.②Higher percentages of pancytopenia and bone marrow hypoplasia.③Lower percentages of positive hemolysis tests.The percentages of CD55 and CD59 of peripheral blood cells were not significantly different in most cases of both groups.④Immunoglobulins and subgroups of T lymphocytes were normal in cases of both groups.⑤Adrenocortical hormone was effective in cases of both groups.Conclusion AA PNH syndrome shares a same pathophysiology with t PNH;CD55 and CD59 tests can improve the diagnosis of AA PNH syndrome.