Journal of Clinical and Experimental Hematopathology最新文献

The prognostic value of models such as the international prognostic index (IPI) in patients with malignant lymphomas treated with a combination of rituximab, doxorubicin, cyclophosphamide, vincristine, and prednisolone is well established. However, whether these prognostic models apply to patients treated with a combination of tetrahydropyranyl adriamycin, rituximab, cyclophosphamide, vincristine, and prednisolone (R-THP-COP) is unclear. This retrospective analysis included 101 patients with Diffuse large B-cell lymphoma (DLBCL) treated with R-THP-COP. The primary endpoint was overall survival (OS). The secondary endpoints were progression-free survival (PFS), complete response rate (CRR), and effectiveness of risk prediction in the IPI, revised international prognostic index (R-IPI), and National Comprehensive Cancer Network (NCCN)-IPI groups. OS and PFS at 5 years were 67% and 58.9%. CRR was 63.5%. The IPI, R-IPI, and NCCN-IPI predicted the outcomes of patients treated with R-THP-COP. According to the NCCN-IPI, OS and PFS could distinguish four risk groups. In conclusion, the NCCN-IPI is the most effective prognostic tool for identifying patients with poor prognosis, even those treated with R-THP-COP.

Primary central nervous system (CNS) lymphomas account for 1.9-3% of all brain tumors, with the majority being histologically classified as primary large B-cell lymphoma of the CNS (PCNS-LBCL). PCNS-LBCL is characterized by mature germinal center-exit B cells, and most cases of this phenotype are classified as activated B-cell-like phenotype according to gene expression profiling, or as non-germinal center B-cell-like phenotype (non-GCB type) according to Hans's algorithm. Genetically, PCNS-LBCL often shows mutations in MYD88^L265P and CD79B^Y196, and is similar to MCD or C5 in genetic subtypes. Therefore, we here investigated the clinicopathological and molecular characteristics of primary CNS B-cell lymphomas (PCNSBLs), focusing on the differences in the frequency of MYD88^L265P and CD79B^Y196 mutations, as well as the prognosis between GCB and non-GCB types. Forty-two patients with PCNSBLs were included in this study, with 12 (28.6%) classified as GCB type and 30 (71.4%) as non-GCB type. There were no significant differences between the two types in gender, tumor location, or frequency of MYD88^L265P and CD79B^Y196 mutations. Even after consideration of the confounding of age and the presence of R-MPV therapy, the GCB type PCNSBLs tended to exhibit better prognosis. Overall survival tended to be better in those with the GCB/MYD88^L265P mutation (-) group, followed by the GCB/MYD88^L265P mutation (+) group, and the non-GCB type. We speculate that Hans's algorithm and MYD88^L265P mutation may have potential prognostic value for PCNSBLs.

Kimura disease (KD) is a rare chronic inflammatory condition that primarily affects Asian males and typically presents in the head and neck region. We describe an exceptionally rare case of KD involving the lingual tonsil of Waldeyer's ring in a 39-year-old Japanese man, marking only the second reported instance of lingual involvement and the first specifically affecting the tongue base. The patient presented with a well-circumscribed, 3.5-cm mass extending from the lingual tonsil to the epiglottis. Laboratory findings revealed significant peripheral eosinophilia (13.5%) and elevated serum IgE levels (2,750 IU/mL). Because of the challenging location for conventional biopsy, fine-needle aspiration cytology was performed on associated cervical lymph nodes. Cytological examination identified Warthin-Finkeldey-type multinucleated cells, eosinophilic infiltration, and vascular proliferation, leading to a presumptive KD diagnosis based on cytomorphology. The diagnosis was confirmed through surgical excision and histopathological analysis. This case is noteworthy for two reasons: it documents an extremely rare presentation of KD in the tongue base and underscores the diagnostic value of cytological examination in anatomically difficult locations where surgical biopsy may be unfeasible. The presence of Warthin-Finkeldey-type multinucleated cells in cytological specimens provided a key diagnostic clue, particularly when integrated with clinical and laboratory findings. At six months post-surgery, the patient showed no recurrence. This case highlights the importance of considering KD in the differential diagnosis of head and neck masses, even in atypical locations, and demonstrates the potential utility of cytological examination in diagnosing KD.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy composed of immature cells that exhibit plasmacytoid dendritic cell (pDC) differentiation. The diagnosis of BPDCN is often challenging due to its rarity and morphologic and phenotypic overlap with other hematologic malignancies, such as acute myeloid leukemia (AML). The emergence of tagraxofusp, a CD123-directed cytotoxin, and other novel therapies has underscored the importance of accurately diagnosing BPDCN. This review initially outlined the clinical and histopathological features of BPDCN, including patients with immunoblastoid morphology. Various proposed diagnostic criteria based on flow cytometry and immunohistochemistry findings were presented, highlighting critical points of caution in the diagnostic process. Strategies for detecting minimal residual disease or microinvasion in BPDCN, a significant clinical issue, were also discussed. Additionally, we reviewed the recurrent 8q24 (MYC) and MYB rearrangements observed in BPDCN, which can aid in diagnosis. Furthermore, we explored mature plasmacytoid dendritic cell proliferation (MPDCP) associated with myeloid neoplasm, which is characterized by a clonal proliferation of pDCs in cases with a defined myeloid neoplasm and may also serve as a potential differential diagnosis for BPDCN. Lastly, we discussed pDC-AML, characterized by pDC proliferation in AML cases, which can also be part of MPDCP and is often associated with frequent RUNX1 mutations. Overall, this review provides insights into BPDCN diagnosis and highlights the current challenges in its detection and differential diagnosis.

Intravascular lymphoma (IVL) is a rare subtype of lymphoma, mostly of B-cell origin. A few cases of IVL have been reported as having NK/T cell origins (IVNKTL). These cases are known to be fatal, especially when systemic symptoms are present. We report the case of a patient of IVNKTL who was refractory to initial treatment and received autologous hematopoietic stem cell transplantation (auto-HSCT). She has maintained complete remission (CR) for over eight years. Our case might support the evidence of auto-HSCT for the treatment of IVNKTL with chemosensitivity.

Immune effector cell-associated neurotoxicity syndrome (ICANS) is a serious complication observed in patients receiving advanced immunotherapies such as bispecific antibodies and CAR-T cell therapies. Although the Immune Effector Cell-Associated Encephalopathy (ICE) score is commonly used to assess ICANS severity, its diagnostic accuracy can be compromised by factors such as concomitant medications, underlying comorbidities, and other external influences. This case report discusses a patient with diffuse large B-cell lymphoma who developed ICANS while receiving Epcoritamab. Notably, elevated interleukin-6 (IL-6) levels in the cerebrospinal fluid (CSF) correlated with the patient's clinical course of neurotoxicity. In contrast to conventional scoring systems, which can be affected by unrelated factors, CSF IL-6 levels appeared to more directly reflect the severity and progression of ICANS. These findings are consistent with similar reports from patients treated with CAR-T cells, suggesting that CSF IL-6 may serve as a reliable marker for ICANS progression. Further research that systematically measures CSF IL-6 in diverse clinical contexts could help validate its role as a biomarker, enhancing diagnostic precision and guiding optimal management strategies for ICANS.

Severe atopic dermatitis (AD) is known to be associated with a risk of lymphoma. We herein report a case of ALK-negative anaplastic large cell lymphoma (ALK-ALCL) complicated by severe AD during treatment with baricitinib, which is an oral, selective, and reversible Janus Kinase (JAK) 1 and 2 inhibitor used in the treatment of AD. Next-generation sequencing (NGS) demonstrated the TP53 p.G266E mutation, suggesting that this was the trigger of the disease and the cause of its refractory course. The JAK/signal transducer and activator of transcription (STAT) pathway is often activated in tumor cells of ALCLs, suggesting that it is a therapeutic target. The causal connection between baricitinib and lymphomagenesis remains unknown; however, this patient developed ALK-ALCL with TP53 mutations during baricitinib treatment.