Association between P-Selectin Autoantibody Positive and Response to Steroid Treatment in Newly Diagnosed Immune Thrombocytopenia Patients.

OBJECTIVE: This study aimed to detect the association between P-selectin autoantibody positive and response to steroid treatment in newly diagnosed immune thrombocytopenia (ITP) patients. METHODS: The data from 105 newly diagnosed adult ITP patients administered with first-line of steroid treatment from October 2016 to May 2021 were retrospectively analyzed. Treatment responses were evaluated within 3 months after the onset of treatment. RESULTS: Among the 105 patients, 80.00% (84/105) of patients presented with platelet glycoprotein-specific antibody positive; 44.76% (47/105) patients were anti-P-selectin positive, while 35.24% (37/105) were anti-P-selectin negative. No significant difference in overall response was observed between patients who were anti-P-selectin positive and those who were anti-P-selectin negative (74.47% vs. 89.19, χ2 = 2.910, p = 0.088). But patients who were anti-P-selectin negative had significantly higher complete response rate, compared to those who were anti-P-selectin positive (72.97% vs. 48.94%, χ2 = 4,965, p = 0.026). Logistic regression analysis revealed that anti-GP IIb/IIIa positive (OR = 3.114, p = 0.010, 95% CI: 1.313-7.388) and anti-P-selectin positive (OR = 0.309, p = 0.036, 95% CI: 0.127-0.753) were two factors that could affect patients' response. CONCLUSIONS: Our study found that ITP patients with anti-GP IIb/IIIa may have a higher response to steroid treatment, but anti-P-selectin-mediated-ITP might be less responsive to steroid treatment. In adults with ITP, the presence of anti-P-selectin autoantibodies is a predictive factor for poor response to steroid treatment.

The association between platelet glycoprotein-specific antibodies and response to short-term high-dose dexamethasone with prednisone maintenance treatment in adult patients with primary immune thrombocytopenia.

OBJECTIVE: The aim of the present study was to detect the association between platelet glycoprotein-specific autoantibodies and the patient response to short-term high-dose dexamethasone (HD-DXM) + prednisone maintenance treatment. METHODS: The data from 112 adult patients newly diagnosed with ITP who were administered first-line HD-DXM + prednisone maintenance therapy between January 2016 and January 2021 were retrospectively analyzed. RESULTS: A total of 72 patients positive for platelet glycoprotein-specific antibodies were enrolled in the antibody-positive group, and 40 patients not positive for platelet glycoprotein-specific antibodies were enrolled in the antibody-negative group. In the antibody-positive group, six platelet glycoprotein-specific antibody types were found: 41.67% of the patients were anti-GP IIb/IIIa-positive only, 5.56% were anti-GP Ib/IX-positive only, 5.56% were anti-P-selectin-positive only, 19.44% were anti-GP IIb/IIIa- and anti-GP Ib/IX-positive, 16.67% were anti-GP Ib/IX- and P-selectin-positive and 11.11% were positive for all three antibodies. There was no significant difference in the overall response rate between the antibody-positive group and the antibody-negative group (94.44 versus 80.00%, p = .221). However, the CR rate was significantly higher in the antibody-positive group than in the antibody-negative group (69.44% versus 40.00%, p = .032). The logistic regression analysis revealed that platelet glycoprotein-specific antibody positivity and age were two factors that could affect patient response. CONCLUSIONS: The present study discovered that adult patients newly diagnosed with ITP who had positive platelet glycoprotein-specific antibody test results were likely to achieve a better response after treatment with HD-DXM + prednisone maintenance.

[Expression Level of S100A6 mRNA in MM and Its Clinical Significance].

OBJECTIVE: To investigate the expression level of S100A6 mRNA in MM and to its clinical significance, and to evaluate its significance. METHODS: The expression level of S100A6 mRNA in MM patients was determined by real time quantitative PCR(RQ-PCR), and its relationship with the clinical features and outcomes of patients was analyzed by statistic method. RESULTS: S100A6 mRNA was detected in 20 MM patients. Compared with normal persons, the S100A6 mRNA expression in MM patients was higher. In different groups, the S100A6 mRNA expression in MM patients of 3 stages was higer than that in patients of 1 and 2 stages. MM patients with higher S100A6 mRNA expression had poor prognosis and higer extramedullary metastasis rate. CONCLUSION: The high expression of S100A6 mRNA is associated with poor prognosis and may be a prognostic molecular marker of MM.

[Effect of free light chain ratio normalization after treatment on prognosis of patients with multiple myeloma].

This study was aimed to investigate the normalization of serum free light chain ratio (sPLCR) after treatment of multiple myeloma (MM) and its influence on the prognosis of MM patients. The clinical data of 42 patients with MM were analyzed retrospectively from January 2009 to November 2013 in out department. According to sPLCR consecutive normalization for more 4 weeks or not after treatment, the patients were classified in patients with mormalized sPLCR and patients with abnormalized sPLCR, then the influence of traditional prognostic factors of MM on sPLCR and effect of sPLCR on overall survival (OS) time of MM patients were analyzed. The results showed that the influence of age, ISS stage displayed statistical difference between sFLCR normalization group and abnormalization group, the age ≥ 65 years and ISS stage III negatively impacted on sFLCR normalization (P < 0.05). The response rates of patients with normalized sFLCR were as follows: CR - 60%, VGPR - 38.89%; PR - 28.57%; 17 patients (40.48%) with sFLCR normalization showed superior OS, as compared with patients with sPLCR abnormalization (P < 0.01). It is concluded that the sFLCR normalization is the independent prognostic factor for MM, suggesting that the MM patients with sPLCR normalization after treatment have superior prognosis.

[Myeloid/natural killer cell acute leukemia resembling acute promyelocytic leukemia].

In order to improve the recognition of myeloid/natural killer cell acute leukemia and to reduce misdiagnosis, one case of myeloid/natural killer cell acute leukemia resembling acute promyelocytic leukemia(APL) was reported and the related articles published were reviewed. A series of clinical tests, the morphologic and immunophenotypic analysis of leukemia cells, cytogenetic and molecular biological examinations were performed. The results indicated that the patient had anemia, thrombocytopenia and leucocytosis, but no evidence of lymphadenopathy and hepatosplenomegaly. The morphology of leukemia cells was similar to that of abnormal promyelocytic cells, especially the variant of M3 (M3v) leukemia cells. The leukemia cells expressed CD117, CD33, CD15, CD56 and cMPO, but did not express CD34, HLA-DR, CD13 and CD16. Abnormal cytogenetics with del (7) (q22q32) was found. Neither t(15;17) nor PML/RARα gene rearrangement was detected. The patient failed to show a differentiation-induction response to all-trans retinoic acid(ATRA). In conclusion, the myeloid/natural killer cell leukemia is extremely rare. It is very important to distinguish the disorder from APL/M3v. The patient with myeloid/natural kill cell acute leukemia should be treated with chemotherapy as acute myeloid leukemia.

[Apoptosis-inducing effect of annexin A2 on multiple myeloma cells and its related mechanisms].

This study was purposed to investigate the apoptosis-inducing effect of Annexin A2 gene (AnxA2) on multiple myeloma (MM) cells and its mechanisms. The human MM cell lines U266 and RPMI8226 were transfected by using siRNA targeting at AnxA2; the expressions of AnxA2 mRNA and protein in the siRNA-transfected cells were detected by real-time PCR and Western blot, respectively; the cell apoptosis was assayed by flow cytometry. The results showed that silencing AnxA2 gene by siRNA resulted in decreased expressions of AnxA2 gene and protein, increased apoptosis of U266 and RPMI8226 cell lines (P < 0.05), at the same time resulted in down-regulation of apoptosis-related gene expressions including p65NF-κB, IL-2, IL-6 (P < 0.05), and up-regulation of P53 gene expression (P < 0.05). It is concluded that the AnxA2 silence plays a promoting role in apoptosis of MM cell lines U266 and RPMI8226.

[Angioimmunoblastic T-cell lymphoma with large granular lymphocytosis].

To improve the recognition of angioimmunoblastic T-cell lymphoma (AITL) and to reduce misdiagnosis, a case diagnosed as AITL with large granular lymphocytosis was reported and the related articles were reviewed. A series of clinical tests, pathologic examination and immunohistochemical test, TCR gene rearrangement detection by multiple PCR and assay of lymphocyte immunophenotypes were carried out. The results indicated that the patient was characterized by fever, skin rash, generalized lymphadenopathy, splenomegaly, pleural effusion, ascites, anemia and thrombocytopenia, increase of circulating large granular lymphocytes with CD3(-) and CD16(+), CD56(+) were detected, T-cell receptor γ-chain gene was rearranged. More large granular lymphocytes with abnormal mitosis were found in ascites. The histological and immunohistochemical changes observed by the lymph node biopsy were compatible with AITL, some cells of which were CD56-positive. In conclusion, AITL is characterized by aggressive progress and generally occurs in elderly patients, its clinical prognosis is poor, the large granular lymphocytosis may be an autoimmune response to the tumor cells or originate from tumor stem/progenitor cells.

[Prognostic significance of telomere length in patients with chronic lymphocytic leukemia].

This study was aimed to explore the prognostic significance of telomere length in patients with chronic lymphocytic leukemia (CLL) and to analyze relation of telomere length with Binet stage, IgVH mutation status, CD38, ZAP-70 expression as well as other clinical features. 35 CLL patients who contained 80% or more tumor cells in the peripheral blood or bone marrow samples were selected as objects studied, while 13 healthy donors were served as normal controls. The telomere relative length was detected by using a real-time fluorescent quantitative polymerase chain reaction method (qPCR); the expression of CD38 and ZAP-70 protein were detected by flow cytometry, the IgVH mutation was detected by multiplex PCR. The results showed that the mean telomere relative length in CLL patients and normal controls were 0.384 and 0.443 respectively, but the difference between them was not significant (p > 0.05). The telomere length was significantly correlated with Binet stages and IgVH mutation status. Patients in Binet stage B and C showed significantly shorter telomeres than those in Binet stage A (p = 0.001). Mean telomere relative lengths in patients without IgVH mutation were shorter than those in patients with IgVH mutation (p = 0.015). No relation of telomere length with sex, age, ZAP-70 protein and CD38 were found (p > 0.05). It is concluded that telomere length may have a prognostic significance for CLL patients. Combining telomere length and IgVH mutation status may achieve a better prognostic subclassification for CLL patients.