Interferon regulatory factor 4 (IRF4) is one of the transcription factors in the interferon regulatory factor family. In the normal physiological process, IRF4 protein is a key factor regulating B cell development, such as early B cell development, pre-B cell switch recombination, mature B cell somatic hypermutation, and also a key factor regulating plasma cell differentiation. In addition, in recent years, it has been reported that Irf4 gene abnormalities or abnormal protein expression is closely involved in the occurrence and development of a variety of B cell or plasma cell tumors. This article reviews the physiological role of IRF4 in the differentiation and maturation of B cell or plasma cells, how IRF4 participates in the occurrence and development of B cell or plasma cell tumors, and its potential therapeutic target for B cell or plasma cell tumors.
With the continuous development of new drugs and immunotherapy, the survival period of patient with multiple myeloma (MM) is continuously prolonged, and the disease is becoming chronic. Due to the involvement of multiple systems and numerous complications, the daily nursing for MM faces significant challenges. The doctor-nurse-patient integration model and the whole life cycle health management model for daily nursing of MM are expected to reduce the social burden related to diseases, improve patients’ quality of life, and reduce medical costs. This article provides a review on three aspects of MM doctor-nurse-patient integration, whole life cycle health management, and daily health management involving multiple systems.
Soft tissue plasmacytomas are classified into primary or secondary. Primary soft-tissue plasmacytomas predominantly occur in the head and neck region. When there is no bone marrow involvement, the prognosis is favorable. Genetically, they are primarily characterized by 1q amplification and del(13q). The preferred treatment is local radiotherapy or surgical excision; for extensive lymph node involvement, bortezomib is administered. Secondary soft-tissue plasmacytomas are typically seen in relapsed multiple myeloma, with a shorter survival time. They frequently harbor high-risk mutations such as del(17p) and t(4;14), requiring multi-agent intensive therapy and/or autologous hematopoietic stem cell transplantation. The prognosis of soft-tissue plasmacytomas is influenced by genetic alterations and affected anatomical sites. Future research should focus on targeting drug-resistance mechanisms and establishing diagnostic-therapeutic protocols.
ObjectiveTo evaluate the effect of intravenous immunoglobulin (IVIG) on prognosis of patients with idiopathic systemic capillary leakage syndrome (ISCLS). MethodsCase reports and case series related to IVIG on prognosis of ISCLS were electronically searched from the PubMed, CNKI and WanFang Data databases from inception to December 31, 2021. Two researchers screened literature and extracted the data independently, then, prognostic data were analyzed. ResultsA total of 143 case reports (175 patients) and 5 case series (169 patients) were included. About 75% of patients had monoclonal gamma globulin, most of those were IgG κ type. A total of 40 patients received prophylaxis with IVIG, most of whom received a high dose (2 g/kg) of IVIG per month. The 5-year and 10-year survival rates of ISCLS patients receiving IVIG secondary prevention treatment were 96% and 72%, respectively, significantly better than the rates of 66% and 43% in the group without IVIG. The median number of acute episodes per year was 0 (0-20) in the group receiving secondary prevention with IVIG and 2 (1-16) in the group not receiving IVIG. ConclusionHigh-dose (2g/kg) IVIG can improve the long-term survival of ISCLS patients, but efficacy of IVIG in acute episodes is unclear.
ObjectiveTo report and analyze one case of Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia (ALL) initially presented with skeletal destruction treated with imatinib-based personal therapy. MethodsWe described the therapeutic advancements for ALL cases initially presented as skeletal destruction and Ph+ ALL through case report and literature review. ResultsDefinite diagnosis of Ph+ ALL was made for the patient who subsequently obtained inductive remission and 17-month molecular remission with the aid of imatinib-based regimen. ConclusionWe should take potential diagnosis of ALL into consideration for patients with skeletal destruction. Imatinib-based standard chemotherapeutic regimen may improve therapeutic model and prognosis of Ph+ ALL.