In order to study the pathogenesis of plasma cell dyscrasias with associated clinical features of chronic neutrophilic leukaemia, the concentration of granulocyte-colony stimulating factor (G-CSF) was measured in a patient, a 73 year old man, who underwent steroid pulse therapy. High G-CSF concentrations and leucocyte counts prior to treatment declined rapidly on administration of dexamethazone, but rose subsequently. G-CSF was not detected in primary cultures of bone marrow cells, but large amounts of interleukin-6 were found in the culture supernatant. These observations suggest that the neutrophilia observed in the patient represented a reactive response to G-CSF secreted from abnormal plasma cells or stromal cells rather than the existence of a genuine myeloproliferative disorder.
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http://dx.doi.org/10.1136/jcp.49.10.858 | DOI Listing |
A 66-year-old woman was diagnosed with chronic lymphocytic leukemia (CLL) due to the finding of leukocytosis and started acalabrutinib and obinutuzumab (AO) therapy. After three cycles of AO therapy, she developed severe pancytopenia with hypoplastic bone marrow and was diagnosed with fulminant aplastic anemia (AA) due to neutropenia with no response to granulocyte colony-stimulating factor. One month after the onset of AA, she received HLA-haploidentical allogeneic hematopoietic stem cell transplantation (haplo-SCT) from a daughter using FluMelTBI (fludarabine 180 mg/m, melphalan 80 mg/m, total body irradiation 4 Gy) as the conditioning regimen and tacrolimus, mycophenolate mofetil, and post-transplant cyclophosphamide (PTCy) for graft-versus-host disease (GVHD) prophylaxis.
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January 2025
Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Neutrophils are peripheral blood-circulating leukocytes that play a pivotal role in host defense against bacterial pathogens which upon activation, they release web-like chromatin structures called neutrophil extracellular traps (NETs). Here, we analyzed and compared the importance of myeloid differentiation factor 88 (MYD88), peptidyl arginine deiminase 4 (PAD4), and gasdermin D (GSDMD) for NET formation in vivo following sepsis and neutrophilia challenge. Injection of lipopolysaccharide (LPS)/E.
View Article and Find Full Text PDFCNS Neurosci Ther
January 2025
Computational Biology Center, Tianjin Anding Hospital, Nankai University Affiliated Tianjin Anding Hospital, Tianjin Mental Health Center of Tianjin Medical University, Tianjin, China.
Background: Clozapine exhibits significant therapeutic efficacy in schizophrenia, especially treatment-resistant schizophrenia. However, clozapine can cause agranulocytosis, a fatal adverse effect, and the aim of this study is to explore this mechanism based on network pharmacology and molecular docking.
Method: Six and two databases were used to identify targets associated with clozapine and agranulocytosis, respectively.
JAGN1 (Jagunal-homolog1) is a ER-resident transmembrane protein which is part of the early secretory pathway and granulocyte colony-stimulating factor receptor mediated signaling. Autosomal recessively inherited variants in the JAGN1 gene lead to congenital neutropenia, early-onset bacterial infections, aphthosis and skin abscesses due to aberrant differentiation and maturation of neutrophils. In addition, bone metabolism disorders and a syndromic phenotype, including facial features, short stature and neurodevelopmental delay, have been reported in affected patients.
View Article and Find Full Text PDFAnn Hematol
January 2025
Department of Hematology, Cheeloo College of Medicine, Qilu Hospital, Shandong University, 107 West Culture Road, Lixia District, Jinan, 250012, P.R. China.
To compare the efficacy and safety of pegylated recombinant human granulocyte colony-stimulating factor (PEG-rhG-CSF) and rhG-CSF in the recovery of neutrophils after induction therapy in ALL patients, PEG-rhG-CSF was injected subcutaneously within 24 ~ 48 h after the end of intravenous infusion of daunorubicin/idarubicin during induction chemotherapy. In rhG-CSF group, patients were given rhG-CSF. The main outcome indexes were the incidence and duration of grade 4 chemotherapy-induced-neutropenia (CIN, ANC less than 0.
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