Journal of Clinical and Experimental Hematopathology最新文献

Duodenal-type follicular lymphoma (DFL) is a rare subtype of follicular lymphoma (FL) characterized by a remarkably indolent clinical course. However, histological transformation to diffuse large B-cell lymphoma (DLBCL) has been sporadically reported, and its clinicopathological features remain poorly understood. We describe a rare case of DFL that transformed to non-germinal center DLBCL, including the specific biological characteristics based on immunohistochemical, cytogenetic, and molecular analyses. A 62-year-old woman was diagnosed with DFL and followed without treatment. Six years later, she presented with progressive anemia and an ulcerative lesion in the ileocecum. Biopsy confirmed the diagnosis of DLBCL with the non-GCB immunophenotype according to Hans' algorithm, which is an uncommon immunophenotype of transformed FL. Fluorescence in situ hybridization analysis revealed BCL6 translocation in both DFL and DLBCL samples. In addition, polymerase chain reaction and sequencing analyses of immunoglobulin heavy and light chain rearrangements clearly demonstrated that the DFL and DLBCL share a common origin. After surgical resection of the ileocecal lesion and six cycles of R-CHOP chemotherapy, the patient has remained in complete remission for 3 years. This case highlights the importance of long-term follow-up of DFL and provides insights into the pathophysiology underlying the transformation of DFL.

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.

We encountered a patient with composite mantle cell lymphoma (MCL) and T-cell prolymphocytic leukemia (T-PLL) who presented with inactive disease to active T-PLL over 8 years. A 71-year-old man was diagnosed with MCL with an atypical T-cell population showing CD2+, CD3-, CD4+, CD7+, CD8-, and CD25+; however, the cause of the T-cell population could not be determined at the first MCL diagnosis. When MCL relapsed approximately 8 years after the initial treatment, T-PLL was definitively diagnosed using the T-PLL International Study Group criteria. MCL and T-PLL were determined to coexist in the lymph nodes and bone marrow by histological or flowcytometry analysis. Retrospective flow cytometry and T-cell receptor-polymerase chain reaction analysis of the stored samples suggested that the T-cell population noted at the time of initial MCL diagnosis eight years earlier was the same clone of T-PLL and the progression from inactive disease to active disease of his T-PLL. To the best of our knowledge, this is the first report of a composite MCL and T-PLL.

A 65-year-old man presented with painful swelling and thickening of both lower limbs, initially responding to oral steroid treatment. However, the patient later developed similar symptoms, accompanied by general malaise. No abnormalities were detected upon fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography. Magnetic resonance imaging (MRI) showed extensive fascial edema in the thigh and gluteal regions, mimicking fasciitis. Concurrently, a bone marrow biopsy revealed intravascular large B-cell lymphoma (IVLBCL), a rare lymphoma with a generally poor prognosis. This case highlights the significance of fasciitis-like MRI findings in diagnosing IVLBCL, underscoring the need for integrating imaging and histological evaluations in atypical presentations.

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.

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.