Expression and influencing factors of hepcidin in classical paroxysmal nocturnal hemoglobinuria / 中华血液学杂志

Objective@#To investigate the serum expression and influencing factors of hepcidinin patients with classical paroxysmal nocturnal hemoglobinuria (PNH) .@*Methods@#Retrospective analysis of 36 classical PNH patients from 2016.3 to 2017.3. Serum hepcidin concentration was measured by ELISA method. The relationship between serum hepcidin concentration and erythropoiesis and iron homeostasis parameters was evaluated.@*Results@#The median serum hepcidin level of 36 classical PNH patients was 32.03 (23.11, 118.48) μg/L, it was significantly lower than of 181.42 (106.80, 250.53) μg/L in 292 normal control subjects (z=-5.107, P<0.001) . The median serum hepcidin of 56.41 (44.60, 95.06) μg/L in PNH patients with normal ferritin was significantly lower than that in normal controls. The median serum hepcidin concentration 23.75 (21.77, 30.35) μg/L in iron deficiency PNH patients was lower than that in the normal ferritin PNH patients. However, the median serum hepcidin level of classical PNH with elevated ferritin patients 336.19 (304.19, 375.08) μg/L was significantly higher not only than that of normal ferritin and iron deficiency PNH ones, but also than that of normal control subjects. Regression analysis showed that serum ferritin, transferrin saturation and serum albumin level were independent influencing factors of serum hepcidin level in patients with classical PNH.@*Conclusion@#The decreased serum hepcidin level in patients with classical PNH was mainly influenced by iron metabolism factors.

Significance of hemoglobin H detection in the diagnosis of α-thalassemia and MDS / 国际检验医学杂志

Objective To evaluate the significance of hemoglobin H in the diagnosis of alpha thalassemia and Myelodysplastic syndrome. Methods A retrospective analysis of the complete clinical data of hemoglobin H in our hospital from January 2007 to October 2017 was performed. The hemoglobin H content in 34 cases of alpha thalassemia and the corresponding blood routine, reticulocyte, Bilirubin, and plasma free hemoglobin levels were analyzed by Pearson correlation. Results 39 cases of hemoglobin H positive cases, of which 34 cases diagnosed as alpha thalassemia,5 cases diagnosed as myelodysplastic syndrome; for alpha thalassemia, hemoglobin H content and reticulocyte, plasma free hemoglobin and Indirect bilirubin test results are related(r =0. 453 ,0. 398,0. 412,P<0. 05). Conclusion Hemoglobin H is not only found in alpha thalassemia but detected in other types of hematologic disorders.

Flow cytometric test using eosin-5'-maleimide (EMA) labelling of red blood for diagnosis of hereditary spherocytosis / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the sensitivity and specificity of eosin-5'-maleimide (EMA)assay for the diagnosis of hereditary spherocytosis (HS), and to verify the stability of reagent and samples.</p><p><b>METHODS</b>EMA flow cytometry test, NaCl-osmotic fragility test and acidified glycerol lysis test were performed using peripheral blood samples from 80 patients with HS and 44 patients with other blood diseases, the sensitivity and specificity of the three methods were compared, and the feasibility of EMA binding test was estimated. The stability of EMA reagent and HS samples stored at different temperatures were tested.</p><p><b>RESULTS</b>Among the 124 tested samples, the sensitivity and specificity of EMA binding test was 0.925 and 0.954, that of NaCl-osmotic fragility test was 0.950 and 0.455, and that of acidified glycerol lysis test was 1.000 and 0.318, respectively. Although the sensitivity of NaCl-osmotic fragility test and acidified glycerol lysis test was a little higher than that of EMA binding test, the specificity of the former two methods was poor, they couldn't clearly distinguish whether spherocytosis is hereditary spherocytosis. The experiment results showed that EMA was sensitive to the temperature and should not be stored in a small aliquots at -80 ℃ over a period of 6 months. The stability of the HS sample was better, 6 days storage at 4 ℃ and 3 days storage at room temperature had no influence on the results.</p><p><b>CONCLUSION</b>EMA binding test by flow cytometry showed good sensitivity and specificity for HS diagnosis. EMA reagent should be stored at-80 ℃ and the HS samples should be tested within 6 days storage at 4 ℃ and 3 days at room temperature.</p>

Application of May-Grunwald-Giemsa staining followed by fluorescence in situ hybridization techniques in the diagnosis of acute leukemia / 中华检验医学杂志

Objective To evaluate the clinical application of May-Grunwald-Giemsa staining followed by fluorescence in situ hybridization (MGG-FISH) technique in the differentiation diagnosis of Ph-chromosome positive acute lymphoid leukemia (Ph + ALL) from chronic myeloid leukemia in lymphoid blast crisis(CML-LBC). Methods The bone marrow smears of 4 patients with Ph+ ALL, 4 patients with CML-LBC, 1 patient with CML in myelocytic blast crisis complicated with lymphoma and 1 patient with CML in mixed blast crisis were assayed with the MGG-FISH technique in which the spectrum green labeled BCR and spectrum orange labeled ABL dual color dual fusion probes were used. Based on the morphological classification, the percentages of BCR-ABL positive cells were subsequently determined respectively in the erythroid, myeloid and lymphoid hneages for the 10 specimens. Results According to the MGG-FISH analysis, the erythroid lineage was not involved in the 4 Ph+ ALL specimens without BCR/ABL positive cells. While the BCR/ABL positive percentage of myeloid cells was 11% (1/9), 8% (1/12), 0% (0/8) and 10% (1/10) respectively and that of lymphoid cells was 97% (76/78), 98% (87/89), 98% (97/99) and 97% (75/77) respectively. On the other hand, the BCR/ABL positive percentage was 100% (8/8), 91% (10/11), 82% (9/11), 88% (7/8) in the erythroid lineage, 89% (8/9), 96% (94/98), 100% (47/47), 98% (40/41)in the myeloid lineage and 96% (78/81), 93% (52/56), 96% (68/71), 95% (58/61) in the lymphoid lineage respectively for the 4 CML-LBC specimens. The BCR/ABL positive percentages of the other 2 specimens were all above 80% and through MGG-FISH analysis we also identified the source of the malignant clones and ascertained the diagnosis of the 2 patients. Conclusions The MGG-FISH technique has proved useful in providing rapid and precise differentiation between Ph + ALL and CML-LBC. The source of the malignant clones can also be analyzed by this technique.