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A retrospective analysis was conducted based on the clinical data from 60 patients older than 16 years from January 2016 to January 2021. All the patients were newly diagnosed with severe aplastic anemia (SAA) with an absolute neutrophil count (ANC) of zero. We compared the hematological response and survival of haploidentical–allogeneic hematopoietic stem cell transplantation (HID-HSCT) (n = 25) and intensive immunosuppressive therapy (IST) (n = 35) treatments. At six months, the overall response rate and complete response were significantly higher in the HID-HSCT group than those in the IST group (84.0% vs. 40.0%, P = 0.001; 80.0% vs. 17.1%, P = 0.001). With a median follow-up of 18.5 months (4.3~30.8 months), patients in the HID-HSCT group had longer overall survival and event-free survival (80.0% vs. 47.9%, P = 0.0419; 79.2% vs. 33.5%, P = 0.0048). These data suggested that HID-HSCT might be an effective alternative treatment option for adult patients with SAA with an ANC of zero, which requires further validation in an additional prospective study. [ABSTRACT FROM AUTHOR]

In wild-type JAK2, the activation loop of JAK2-homology domain 1 (JH1) is pulled toward the JH1/JH2 interface through interactions with key residues of JH2, especially S591, F595, and V617, and stabilizes the inactivated form of JH1 [[9]]. Dear Editor, JAK2 is a member of the JAK family of protein tyrosine kinases (PTKs) that play important roles in regulating a spectrum of key signaling pathways. A total of 1811 patients were tested between 2010 and 2013; five patients had a p.G571S mutation, and one had a p.Y570Y silent mutation in exon 13 of JAK2 [[6]]. [Extracted from the article]

Objectives: To evaluate the prognosis of adult severe aplastic anemia (SAA) patients with absolute neutrophil count (ANC) values of zero prior to immunosuppressive therapy (IST). Methods: Patients with ANC values of zero prior to IST were separated from very SAA and analyzed in a prospective study. All patients received IST with rabbit anti-thymocyte globulin (ATG) and cyclosporine (CsA). Results: A significantly lower response rate (RR) was identified in patients with ANC = 0 prior to IST when compared to patients with SAA after both 3 and 6 month periods or compared to those with vSAA at 3 months only. The efficacy of IST was inversely related to ANC = 0. The overall survival rate of the 'zero' group was significantly lower than that of the vSAA or SAA groups. Overall survival was closely associated with response to IST, and was inversely related to ANC = 0. Discussion: In SAA patients, ANC is associated with prognosis, the elucidated overall survival improvement in patients without ANC = 0 occurred in conjunction with decreased infection-related mortality. Our study revealed that adult patients with ANC = 0 prior to IST responded poorly to IST, suggesting that having a very low number of neutrophils was a highly predictive factor for efficacy and survival of SAA patients treated with IST. Conclusion: Adult SAA patients with ANC = 0 had a very poor prognosis and new therapeutic regimens may result in better outcome for these patients. [ABSTRACT FROM AUTHOR]

The usefulness of flow cytometric variable β-chain repertoire (FC-Vβ) and T-cell receptor gene rearrangement (TCR-GR) analyses for differentiating T-cell large granular lymphocytic leukemia (T-LGLL) from reactive T-large granular lymphocyte (T-LGL) lymphocytosis has been insufficiently studied to date. In this study, we analyzed the diagnostic value of TCR-GR and FC-Vβ analysis in T-LGLL, and compared these results. In our study, FC-Vβ analysis was positive in all cases of T-LGLL, and clonality assessment of FC-Vβ had equal sensitivity and specificity to GeneScanning analysis but was more sensitive than heteroduplex analysis. Suspected T-cell clonality can best be addressed by evaluating two TCR targets (TCRβ and TCRγ), either in parallel or consecutively. Signal transducer and activator of transcription 3 (STAT3) mutation may provide a diagnostic tool for classifying some cases of T-LGL lymphocytosis as true T-LGLL. Our results further demonstrate a significant correlation of STAT3 mutation with pure red cell aplasia, neutropenia, hepatomegaly, β2-microglobulin and anemia. [ABSTRACT FROM AUTHOR]