ABSTRACT
Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of malignant lymphoma. The incidence of Epstein-Barr virus (EBV)-positive DLBCL in Asian and Latin American countries ranges from 8 to 10%. The prognosis of patients with EBV-positive DLBCL is controversial. To compare the clinical outcome of EBV-positive and EBV-negative patients with DLBCL in the rituximab era, we analyzed 239 patients with de novo DLBCL diagnosed between January 2007 and December 2011. The presence of EBV in lymphoma cells was detected using EBV-encoded RNA in situ hybridization, and it was found that 18 (6.9%) of 260 patients with diagnosed DLBCL tested positive. Among the 260 cases, 216 cases were treated with rituximab plus chemotherapy, as were 8 EBV-positive DLBCL patients. The median overall survival and progression-free survival times in patients with EBV-positive DLBCL were 8.7 months and 6.8 months, respectively. The median overall survival and progression-free survival could not be determined in EBV-negative DLBCL patients (P = 0.0002, P < 0.0001, respectively). The outcome of patients with EBV-positive DLBCL remains poor, even in the rituximab era.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Epstein-Barr Virus Infections/complications , Lymphoma, Large B-Cell, Diffuse/drug therapy , Aged , Antibodies, Monoclonal, Murine-Derived/administration & dosage , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Cyclophosphamide/therapeutic use , Doxorubicin/therapeutic use , Epstein-Barr Virus Infections/mortality , Female , Humans , Kaplan-Meier Estimate , Lymphoma, Large B-Cell, Diffuse/mortality , Lymphoma, Large B-Cell, Diffuse/virology , Male , Middle Aged , Prednisone/therapeutic use , Proportional Hazards Models , Rituximab , Treatment Outcome , Vincristine/therapeutic useABSTRACT
Lymphoproliferative disorders and Epstein-Barr virus reactivation (EBV-LPDs) have various forms of onset, ranging from infectious mononucleosis-like syndrome (IM-like) to lymphoma, although whether or not IM-like progresses to lymphoma remains unclear. A 61-year-old man was diagnosed with aplastic anemia (AA). Polyclonal atypical B-lymphocytes were observed in the peripheral blood, and IM-like was diagnosed. Atypical lymphocytes disappeared, but a gastrointestinal examination revealed diffuse large B-cell lymphoma (DLBCL). Rituximab was initiated but later discontinued because of severe acute respiratory syndrome coronavirus 2 infection. Pancytopenia due to AA exacerbation recurred. The patient ultimately died of multiple organ failure due to bacterial infection.
Subject(s)
Anemia, Aplastic , COVID-19 , Epstein-Barr Virus Infections , Lymphoma, Large B-Cell, Diffuse , Male , Humans , Middle Aged , Antilymphocyte Serum/adverse effects , Anemia, Aplastic/drug therapy , Anemia, Aplastic/complications , Herpesvirus 4, Human , Epstein-Barr Virus Infections/diagnosis , COVID-19/complications , Neoplasm Recurrence, Local/complications , Lymphoma, Large B-Cell, Diffuse/complicationsABSTRACT
The relationship between fetal hemoglobin (HbF) levels and disease prognosis in patients with myelodysplastic syndrome (MDS) is unclear. This study aimed to clarify the relationship between HbF level and the prognosis of MDS. To this end, data from 217 patients diagnosed with MDS between April 2006 and August 2020 at Ebina General Hospital were analyzed retrospectively. The primary endpoint was leukemia-free survival (LFS) for 5Ā years after diagnosis. HbF levels were significantly higher in patients with MDS than in control patients without MDS (n = 155), with a cut-off value of 0.4%. Higher-risk patients had a similar prognosis regardless of HbF level, but lower-risk patients had longer LFS at intermediate HbF levels. Although prognosis based on pre-treatment HbF levels did not differ significantly among azacitidine-treated patients, prognosis tended to be better in lower-risk patients with intermediate HbF levels. Multivariate analysis showed that the intermediate HbF category correlated with LFS, independently of MDS lower-risk prognostic scoring system (LR-PSS)-related factors. This study is the first to assess the association between HbF levels and the new World Health Organization 2016 criteria for MDS, demonstrating the significance of HbF levels in the prognosis of MDS.
Subject(s)
Fetal Hemoglobin , Myelodysplastic Syndromes , Humans , Retrospective Studies , Myelodysplastic Syndromes/diagnosis , Myelodysplastic Syndromes/drug therapy , Prognosis , AzacitidineABSTRACT
Haematopoietic insufficiency is the treatment target of lower-risk myelodysplastic syndrome (MDS). Although erythropoiesis-stimulating agents (ESAs) are generally effective for treating anaemia, resistance can develop. Hypoxia-inducible factor-prolyl hydroxylase (HIF-PH) improves renal anaemia by promoting endogenous erythropoietin production and normalizing iron metabolism. HIF-PH inhibitors could be used to treat MDS, but their efficacy and safety have not been studied. A 78-year-old female patient with essential thrombocythemia gradually developed anaemia and was diagnosed with therapy-related MDS 4 years later. The anaemia temporarily improved with ESAs, but the patient became transfusion dependent. At the same time, anaemia and chronic renal failure due to nephrosclerosis progressed, and the patient was diagnosed with MDS with renal anaemia. After switching from ESAs to roxadustat, an HIF-PH inhibitor, anaemia improved, and the patient was no longer transfusion dependent. No progression of the underlying disease or any adverse events was observed 4 months after initiating roxadustat.
Subject(s)
Antineoplastic Agents/adverse effects , Boronic Acids/adverse effects , Multiple Myeloma/complications , Peripheral Nervous System Diseases/chemically induced , Pyrazines/adverse effects , Aged , Aged, 80 and over , Antineoplastic Agents/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Arm , Boronic Acids/administration & dosage , Bortezomib , Humans , Incidence , Injections, Subcutaneous , Male , Middle Aged , Multiple Myeloma/drug therapy , Peripheral Nervous System Diseases/diagnosis , Pyrazines/administration & dosage , Severity of Illness IndexABSTRACT
A 69-year-old man was diagnosed with chronic myelogenous leukemia (CML) and treated with dasatinib. After two years on dasatinib, the patient achieved complete molecular response, but dasatinib treatment was discontinued due to exacerbation of pleural effusion. Nilotinib and imatinib were started but stopped due to an increase in pleural effusion. Thoracentesis was performed and he was diagnosed with human herpesvirus 8-unrelated primary effusion lymphoma (PEL)-like lymphoma. Complex chromosomal abnormality, including BCL6 rearrangement, was found on chromosome analysis. To the best of our knowledge, this is the first report of PEL-like lymphoma following tyrosine kinase inhibitor treatment for CML.
Subject(s)
Dasatinib/adverse effects , Herpesviridae Infections/chemically induced , Herpesvirus 8, Human , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/drug therapy , Lymphoma, Primary Effusion/chemically induced , Neoplasms, Second Primary/chemically induced , Pleural Effusion, Malignant/chemically induced , Protein Kinase Inhibitors/adverse effects , Aged , Dasatinib/administration & dosage , Humans , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/genetics , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/pathology , Lymphoma, Primary Effusion/genetics , Lymphoma, Primary Effusion/pathology , Male , Neoplasms, Second Primary/genetics , Neoplasms, Second Primary/pathology , Pleural Effusion, Malignant/genetics , Pleural Effusion, Malignant/pathology , Protein Kinase Inhibitors/administration & dosageABSTRACT
Autoimmune hemorrhaphilia due to anti-factor XIII (FXIII) antibodies (AH13) is a life-threatening disease associated with high risk of surgical bleeding. Since AH13 occurs mainly in the elderly, patients of AH13 tend to be complicated with other life-threatening diseases that may require surgical procedures. During our nation-wide survey on AH13, supported by the Japanese Ministry of Health, Labor, and Welfare, patients with unexplained bleeding were examined for FXIII-related parameters and anti-FXIII autoantibodies. A 64-year-old man had previously been tentatively diagnosed with AH13 and received immunosuppressive therapies, as FXIII inhibitor was detected by functional cross-mixing studies. About 2 years later, he was definitively diagnosed with AH13, because our immuno-chromatographic test and enzyme-linked immuno-sorbent assay detected FXIII-bound anti-FXIII-A subunit autoantibodies. Since routine endoscopic examination revealed suspected esophageal carcinoma, a preparatory FXIII pharmacokinetic (PK) analysis was performed by infusing FXIII concentrates prior to biopsy. Consequently, biopsy of this lesion was done without bleeding complications. One month later, a second PK study was carried out before surgery, and esophageal bypass surgery was completed successfully under FXIII replacement therapy. Our experience with this case suggests that operations can be performed safely and with confidence even in patients with such life-threatening hemorrhagic diseases.
Subject(s)
Autoantibodies/blood , Autoimmune Diseases/immunology , Carcinoma/surgery , Esophageal Neoplasms/surgery , Factor XIII/administration & dosage , Factor XIII/immunology , Hemophilia A/etiology , Hemophilia A/immunology , Preoperative Care , Digestive System Surgical Procedures/methods , Factor XIII/pharmacokinetics , Humans , Male , Middle Aged , Treatment OutcomeABSTRACT
A 40-year-old male developed refractory acute promyelocytic leukemia (APL) after various treatments including all-trans retinoic acid, tamibarotene, arsenic trioxide (As2O3), conventional chemotherapy, and autologous peripheral blood stem cell transplantation. We attempted to use both tamibarotene and As2O3 as a combination therapy, and he achieved molecular complete remission. Grade 2 prolongation of the QTc interval on the electrocardiogram was observed during the therapy. The combination therapy of As2O3 and tamibarotene may be effective and tolerable for treating refractory APL cases who have no treatment options, even when they have previously been treated with tamibarotene and As2O3 as a single agent.