Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a rare Epstein-Barr virus (EBV)-associated T- or natural killer (NK)-cell lymphoproliferative disease that classically affects children. Adult-onset disease is uncommon and may follow an aggressive systemic course. We report a case of systemic adult-onset HVLPD in a 63-year-old woman who initially presented symptoms concerning for an autoimmune process. Initial biopsies demonstrated thrombotic vasculopathy and granulomatous dermatitis without malignant features. Despite transient improvement with immunosuppressive therapy, she later developed rapidly progressive disease and repeat biopsy confirmed and EBV-associated clonal T-cell lymphoproliferative process consistent with systemic HVLPD. Staging studies demonstrated widespread nodal and extranodal disease. The patient received CHOEP chemotherapy followed by nivolumab after early progression; however, the disease remained refractory, and she ultimately elected for hospice care. This case highlights the diagnostic difficulty of adult-onset HVLPD when early manifestations mimic inflammatory or vasculopathic disease.
Intravascular large B-cell lymphoma (IVLBCL) is a rare and aggressive subtype of extranodal non-Hodgkin lymphoma (NHL) that typically involves the central nervous system (CNS) and skin in its classic (Western) variant and frequently presents with systemic B-symptoms. We present a rare case of IVLBCL in a 59-year-old female with cutaneous-only involvement, no B-symptoms, and negative PET-CT. The patient’s initial presentation mimicked inflammatory conditions, contributing to diagnostic delay. Deep skin biopsy was crucial and confirmed the diagnosis of IVLBCL. PET-CT was non-revealing, showing limitations in cutaneous-dominant disease. The patient underwent R-CHOP treatment along with CNS prophylaxis, achieving complete remission with no relapse at follow-up. This case emphasizes the importance of high suspicion and early biopsy in atypical cutaneous presentations and the need for personalized strategies.
T-lymphoblastic lymphoma (T-LBL) is an uncommon hematological malignancy that rarely presents as a secondary cancer following chemotherapy. While IDH2 mutations are commonly identified in acute myeloid leukemia (AML), they were very rarely found in T-LBL. We present a case of an adult male diagnosed with therapy-related T-LBL with an IDH2 mutation after 3 years of complete remission following chemotherapy for AML with the same IDH2 mutation, which suggests a dedifferentiated multipotent cancer stem and progenitor cell that may give rise to both the myeloid and lymphoid malignancies.
Introduction Myeloid neoplasms harboring t(8;22)(p11;q11)/BCR::FGFR1 rearrangement are exceptionally rare and are typically associated with aggressive clinical behavior. The coexistence of trisomy 8 with concurrent RUNX1 and STAG2 mutations in this cytogenetic context has not been systematically described. Case Presentation We report a 71-year-old female who presented with fever, dysuria, cough, shortness of breath, and melaena. Initial laboratory evaluation revealed anemia, leukocytosis, and severe thrombocytopenia, with approximately 60% peripheral circulating blasts. Bone marrow examination demonstrated 78% blasts with monocytic morphology. Flow cytometry identified an abnormal blast population (∼87% of viable events) expressing CD33 and CD64 with variable CD13, CD38, HLA-DR, CD11c, and CD15, and a small subset positive for cytoplasmic myeloperoxidase, consistent with acute monocytic leukemia. Conventional cytogenetics revealed 47,XX,+8,t(8;22)(p11;q11). Targeted next-generation sequencing detected a BCR::FGFR1 fusion along with a likely pathogenic RUNX1 missense mutation affecting the Runt homology domain and a loss-of-function STAG2 frameshift mutation. The patient was treated with azacitidine and venetoclax, achieving early hematologic response with clearance of circulating blasts. Conclusion This case highlights a rare presentation of acute monocytic leukemia with trisomy 8 and t(8;22)(p11;q11)/BCR::FGFR1 rearrangement harboring concurrent RUNX1 and STAG2 mutations. These findings expand the molecular spectrum of FGFR1-rearranged neoplasms and underscore the value of integrated cytogenetic and molecular diagnostics in defining uncommon AML presentations.
Background Monoclonal M-protein is frequently detected in marginal zone lymphoma (MZL) and has been historically associated with adverse clinical features. Its prognostic value in the era of modern immunotherapy remains unclear. Patients and methods We retrospectively analyzed 43 patients with MZL, comparing clinical characteristics, treatments, and outcomes between patients with and without M-protein. Progression-free survival (PFS) and overall survival (OS) were assessed using Kaplan–Meier analysis, and prognostic factors were evaluated by Cox regression. Results M-protein was detected in 39.5% of patients and was associated with higher tumor burden, worse performance status, and elevated inflammatory and biochemical markers. However, no significant differences in PFS or OS were observed between groups, and M-protein was not an independent prognostic factor. Most patients received contemporary CD20 antibody–based combination therapies. Conclusion In the setting of modern treatments, M-protein positivity may not confer an adverse prognostic impact in MZL.
