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1.
Hematology ; 29(1): 2340149, 2024 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38626148

RESUMO

OBJECTIVES: Patients with myeloproliferative neoplasms (MPNs) are at higher risk of developing secondary malignancies. In this study, we focused on patients with MPNs that complicated lymphoid neoplasms. To analyze the real-world status of lymphoid neoplasm treatment in patients with pre-existing MPNs in Japan, we conducted a multicenter retrospective study. METHODS: Questionnaires were sent to collect the data on patients who were first diagnosed with either polycythemia vera, essential thrombocythemia or myelofibrosis and who later were complicated with lymphoid neoplasms defined as malignant lymphoma, multiple myeloma, or chronic lymphocytic leukemia/small cell lymphoma. RESULTS: Twenty-four patients with MPNs complicated by lymphoid neoplasms were enrolled (polycythemia vera, n = 8; essential thrombocythemia, n = 14; and primary myelofibrosis, n = 2). Among these, diffuse large B-cell lymphoma (DLBCL) was the most frequently observed (n = 13, 54.1%). Twelve (92.3%) of the patients with DLBCL received conventional chemotherapy. Among these 12 patients, regarding cytoreductive therapy for MPNs, 8 patients stopped treatment, one continued treatment, and two received a reduced dose. Consequently, most patients were able to receive conventional chemotherapy for DLBCL with a slightly higher dose of granulocyte colony-stimulating factor support than usual without worse outcomes. All 3 patients with multiple myeloma received a standard dose of chemotherapy. CONCLUSION: Our data indicate that if aggressive lymphoid neoplasms develop during the course of treatment in patients with MPNs, it is acceptable to prioritize chemotherapy for lymphoma.


Assuntos
Leucemia Linfocítica Crônica de Células B , Linfoma , Mieloma Múltiplo , Transtornos Mieloproliferativos , Policitemia Vera , Trombocitemia Essencial , Humanos , Trombocitemia Essencial/tratamento farmacológico , Trombocitemia Essencial/epidemiologia , Estudos Retrospectivos , Japão/epidemiologia , Transtornos Mieloproliferativos/tratamento farmacológico , Transtornos Mieloproliferativos/epidemiologia , Transtornos Mieloproliferativos/diagnóstico , Linfoma/epidemiologia , Linfoma/etiologia , Linfoma/terapia
2.
Cancer Med ; 13(6): e7050, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38506241

RESUMO

AIMS: T-follicular helper (TFH) cells are effector T-cells that are crucial for B-cell selection and differentiation. T-cell lymphomas derived from TFH cells have distinct characteristics. Additionally, in the World Health Organization (WHO) classification 5th edition, three lymphomas were introduced as independent disease entities with TFH cell origin. We aimed to investigate the clinicopathological features of adult T-cell leukemia/lymphoma (ATLL) with a TFH phenotype (TFHP). METHODS AND RESULTS: We performed TFH immunohistochemistry analysis of five biomarkers for the biopsy specimen, with TFHP being indicated by a positive result for more than two markers. Among 75 cases of ATLL, 37.3% of them showed TFHP. Compared with cases of ATLL without TFHP, cases of ATLL with TFHP showed higher C-reactive protein levels (p = 0.0219) and increased high endothelial venule proliferation (p = 0.024). However, there were no significant between-group differences in overall survival as well as other clinical and morphological findings. Furthermore, there was no significant between-group difference in TFH markers and FOXP3 expression. CONCLUSION: Some patients with ATLL may present a TFHP, which should not preclude the diagnosis of ATLL. Although presenting a TFHP does not affect prognosis, it is important to identify cases of ATLL with a TFHP since it may inform future treatment strategies.


Assuntos
Leucemia-Linfoma de Células T do Adulto , Linfoma , Adulto , Humanos , Leucemia-Linfoma de Células T do Adulto/diagnóstico , Leucemia-Linfoma de Células T do Adulto/genética , Linfoma/patologia , Prognóstico , Fenótipo , Linfócitos T Auxiliares-Indutores/metabolismo , Linfócitos T Auxiliares-Indutores/patologia
3.
J Chromatogr A ; 1721: 464845, 2024 Apr 26.
Artigo em Inglês | MEDLINE | ID: mdl-38552371

RESUMO

Non-Hodgkin lymphoma (NHL) is a heterogeneous group of malignant tumors occurring in B or T lymphocytes, and no small molecule-positive drugs to treat NHL have been marketed. Cluster of differentiation 20 (CD20) is an important molecule regulating signaling for the life and differentiation of B lymphocytes and possesses the characteristics of a drug target for treating NHL. 2-Methoxyestradiol induces apoptosis in lymphoma Raji cells and CD20 protein is highly expressed by Raji lymphoma cells. Therefore, in this study, a CD20-SNAP-tag/CMC model was developed to validate the interaction of 2-methoxyestradiol with CD20. 2-Methoxyestradiol was used as a small molecule control compound, and the system was validated for good applicability. The cell membrane chromatography model was combined with high-performance liquid chromatography ion trap time-of-flight mass spectroscopy (HPLC-IT-TOF-MS) in a two-dimensional system to successfully identify, analyze, and characterize the potential active compounds of Schisandra chinensis (Turcz.) Baill. extract and Lysionotus pauciflorus Maxim. extract, including Schisandrin A, Schizandrol A, Schizandrol B, Schisantherin B, and Nevadensin, which can act on CD20 receptors. The five potential active compounds were analyzed by non-linear chromatography. The thermodynamic and kinetic parameters of their interaction with CD20 were also analyzed, and the mode of interaction was simulated by molecular docking. Their inhibitory effects on lymphoma cell growth were assessed using a Cell Counting Kit-8 (CCK-8). Nevadensin and Schizandrin A were able to induce apoptosis in Raji cells within a certain concentration range. In conclusion, the present experiments provide some bases for improving NHL treatment and developing small molecule lead compounds targeting CD20 with low toxicity and high specificity.


Assuntos
Ciclo-Octanos , Medicamentos de Ervas Chinesas , Lignanas , Linfoma , Compostos Policíclicos , Schisandra , Humanos , Medicina Tradicional Chinesa , 2-Metoxiestradiol , Células Imobilizadas/química , Simulação de Acoplamento Molecular , Espectrometria de Massas em Tandem/métodos , Lignanas/análise , Medicamentos de Ervas Chinesas/farmacologia , Medicamentos de Ervas Chinesas/química , Cromatografia Gasosa-Espectrometria de Massas , Linfoma/tratamento farmacológico , Schisandra/química , Cromatografia Líquida de Alta Pressão/métodos
4.
Am J Case Rep ; 25: e940963, 2024 Mar 04.
Artigo em Inglês | MEDLINE | ID: mdl-38437184

RESUMO

BACKGROUND Non-IgM lymphoplasmacytic lymphoma (LPL) is a rare subtype of LPL, constituting less than 5% of the cases, and is often associated with IgG, IgA, or light chain paraproteins and is rarely a non-secretor. Non-IgM LPL remains poorly studied, and the differential diagnosis from other small B-cell lymphomas with plasmacytic differentiation and plasma cell neoplasm is challenging. CASE REPORT A 67-year-old woman presented with weight loss, persistent anemia, and borderline leukopenia. Serum protein electrophoresis and immunofixation demonstrated a faint IgG and kappa band against a dense polyclonal background. Bone marrow biopsy revealed hypercellular marrow with involvement by abnormal B cells with undetectable surface and cytoplasmic immunoglobulin light chains. Interestingly, these B cells showed no expression of light chains or production of IgG and IgM; however, they showed production of intracytoplasmic IgA. The concomitant neoplastic plasma cells also displayed no definitive light chain expression. Both IgH and IgK gene rearrangements were positive for clonal process. Molecular studies showed positive MYD88 L265P mutation and CXCR4 mutation (c.1013C>G). The overall findings confirmed marrow involvement by non-IgM LPL. The patient received 6 cycles of rituximab and bendamustine treatment, and no residual marrow involvement was found on the follow-up bone marrow biopsy. CONCLUSIONS We report a non-IgM LPL case featuring no light chain production and no heavy chain secretion, which we believe is the first reported case of this kind in the literature.


Assuntos
Linfoma de Células B , Linfoma , Macroglobulinemia de Waldenstrom , Feminino , Humanos , Idoso , Macroglobulinemia de Waldenstrom/diagnóstico , Imunoglobulina A , Imunoglobulina G
5.
JAMA Netw Open ; 7(2): e2355727, 2024 Feb 05.
Artigo em Inglês | MEDLINE | ID: mdl-38363571

RESUMO

Importance: COVID-19 in pediatric patients with acute lymphoblastic leukemia or lymphoma (ALL/LLy) has not been described in detail and may affect chemotherapy administration and long-term outcomes. Objective: To describe the clinical presentation of COVID-19 and chemotherapy modifications in pediatric patients with ALL/LLy. Design, Setting, and Participants: This is a retrospective case series of patients at St Jude Children's Research Hospital and its affiliate sites with newly diagnosed ALL/LLy who were treated on the Total XVII protocol (NCT03117751) between March 30, 2020, and June 20, 2022. Participants included patients aged 1 to 18 years who were receiving protocol chemotherapy. Acute symptoms and chemotherapy modifications were evaluated for 60 days after the COVID-19 diagnosis, and viral clearance, adverse events, and second SARS-CoV-2 infections were followed up during the 27-month study period. Exposures: SARS-CoV-2; all patients were screened at least weekly and at symptom onset and/or after known exposure to SARS-CoV-2. Main Outcomes and Measures: Description of the spectrum of COVID-19 illness and chemotherapy modifications. Results: Of 308 pediatric patients, 110 (36%) developed COVID-19 at a median age of 8.2 (IQR, 5.3-14.5) years. Sixty-eight patients (62%) were male. Most patients were in the continuation/maintenance phase of chemotherapy (101 [92%]). Severe disease was rare (7 [6%]) but was associated with older age, higher white blood cell counts at ALL/LLy diagnosis, lower absolute lymphocyte counts at COVID-19 diagnosis, abnormal chest imaging findings, and SARS-CoV-2 reinfection. Rare but serious thrombotic events included pulmonary embolism and cerebral venous sinus thrombosis (n = 1 for each). No multisystem inflammatory syndrome in children or death was seen. SARS-CoV-2 reinfection occurred in 11 patients (10%) and was associated with older age and with receiving standard or high-risk vs low-risk ALL/LLy therapy. Chemotherapy interruptions occurred in 96 patients (87%) and were longer for patients with severe disease, SARS-CoV-2 reinfection, and/or a COVID-19 diagnosis during the pre-Omicron variant period vs the post-Omicron period (after December 27, 2021). Conclusions and Relevance: In this case series of COVID-19 in pediatric patients with ALL/LLy, severe COVID-19 was rare, but chemotherapy administration was affected in most patients. Long-term studies are needed to establish the outcomes of COVID-19 in this population.


Assuntos
COVID-19 , Linfoma , Leucemia-Linfoma Linfoblástico de Células Precursoras , Humanos , Masculino , Criança , Pré-Escolar , Adolescente , Feminino , COVID-19/complicações , COVID-19/epidemiologia , SARS-CoV-2 , Estudos Retrospectivos , Teste para COVID-19 , Reinfecção , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/epidemiologia , Linfoma/complicações , Linfoma/epidemiologia
6.
Cancer Rep (Hoboken) ; 7(2): e1987, 2024 02.
Artigo em Inglês | MEDLINE | ID: mdl-38351548

RESUMO

BACKGROUND: Mercaptopurine is an important component of acute lymphoblastic leukemia (ALL) and lymphoma (LLy) maintenance therapy. The 6-thioguanine nucleosides (6-TGN) are believed to be the primary contributor to myelosuppression and immunosuppressive effects, while 6-methylmercaptopurine (6-MMPN) is believed to be responsible for several toxicities including hepatotoxicity, pancreatitis, and hypoglycemia. Previous reports suggest the addition of allopurinol may reduce these toxicities. AIMS: To assess the use of allopurinol to improve both safety and efficacy of mercaptopurine in pediatric patients with ALL and LLy during maintenance therapy. Secondary objectives included evaluating patient tolerability and skewed metabolism. In addition, we also analyzed mercaptopurine daily dose reduction upon allopurinol initiation. METHODS AND RESULTS: The primary endpoint was time within goal ANC prior to and after initiation of allopurinol. Secondary endpoints included; improvement in selective toxicities (hepatotoxicity, pancreatitis, and hypoglycemia) and 6-MMPN to 6-TGN ratio prior to and after allopurinol initiation. In addition, an exploratory endpoint assessing mercaptopurine daily dose reduction prior to and after allopurinol initiation was included. Sixteen patients met inclusion criteria and 15 (94%) of which were included in this study. Median percent of maintenance days within goal ANC prior to and after initiation of allopurinol was 27.8 (IQR 22.6-44.9) and 41.6 (IQR 20.2-58.2) respectively. All patients experienced selective toxicities; 15 (100%) hepatotoxicity, 1 (7%) pancreatitis, and 3 (20%) hypoglycemia. Improvement of toxicities was seen in 13/15 (87%), 1/1 (100%), and 2/3 (67%) respectively. Average 6-MMPN:6-TGN ratio prior to allopurinol initiation was 304:1 and after, allopurinol initiation improved to 15:1, resulting in a 95% reduction. Average mercaptopurine dose prior to and after allopurinol initiation decreased by about 56% (63 to 28 mg/m2 /day). CONCLUSION: Results suggest that the use of allopurinol in pediatric patients with ALL and LLy receiving mercaptopurine during maintenance therapy is both safe and effective.


Assuntos
Doença Hepática Induzida por Substâncias e Drogas , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos , Hipoglicemia , Linfoma , Pancreatite , Leucemia-Linfoma Linfoblástico de Células Precursoras , Humanos , Criança , Mercaptopurina/efeitos adversos , Alopurinol/efeitos adversos , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Hipoglicemia/tratamento farmacológico , Doença Hepática Induzida por Substâncias e Drogas/tratamento farmacológico , Linfoma/diagnóstico , Linfoma/tratamento farmacológico , Pancreatite/induzido quimicamente , Pancreatite/diagnóstico
7.
J Clin Exp Hematop ; 64(1): 37-44, 2024 Mar 28.
Artigo em Inglês | MEDLINE | ID: mdl-38281744

RESUMO

Peritoneal lymphomatosis (PL) is a rare lymphoma-associated condition defined as the dissemination of lymphoma cells in the peritoneum. An 82-year-old man presented with abdominal pain, heartburn, and high fever. Radiological findings, including positron emission tomography-computed tomography (PET-CT), and gastrointestinal fiberscopy, showed diffuse thickening of the peritoneum, omentum, and mesentery; however, no lymphadenopathy, hepatosplenomegaly, or gastrointestinal lesions were observed. Under suspicion of peritonitis carcinomatosa of unknown origin, exploratory laparoscopy was performed that revealed multiple white nodules and masses on the surfaces of the peritoneum, mesentery, and intestinal serosa. The histopathological and cytogenetic findings of the peritoneum revealed high-grade B-cell lymphoma, not otherwise specified, and a gain of MYC by fluorescence in-situ hybridization. The patient was treated with two cycles of R-CHOP therapy, followed by six cycles of dose-adjusted EPOCH-R therapy, and a complete metabolic response was confirmed by PET-CT. Since there are no specific radiological findings to confirm the diagnosis of PL, a histopathological diagnosis is usually required. Most PL exhibit an aggressive lymphoma phenotype and can be cured by appropriate chemotherapy. Therefore, early diagnosis and treatment are desirable.


Assuntos
Linfoma de Células B , Linfoma Difuso de Grandes Células B , Linfoma , Neoplasias Peritoneais , Masculino , Humanos , Idoso de 80 Anos ou mais , Peritônio/patologia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Neoplasias Peritoneais/diagnóstico , Neoplasias Peritoneais/tratamento farmacológico , Neoplasias Peritoneais/patologia , Linfoma de Células B/diagnóstico , Linfoma de Células B/tratamento farmacológico , Linfoma de Células B/patologia , Linfoma/patologia , Prednisona/uso terapêutico , Rituximab/uso terapêutico , Vincristina/uso terapêutico , Ciclofosfamida/uso terapêutico , Doxorrubicina/uso terapêutico , Etoposídeo/uso terapêutico , Linfoma Difuso de Grandes Células B/patologia
8.
Pathology ; 56(2): 239-258, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38216400

RESUMO

Recent advances in molecular diagnostics have markedly expanded our understanding of the genetic underpinnings of lymphomas and catalysed a transformation in not just how we classify lymphomas, but also how we treat, target, and monitor affected patients. Reflecting these advances, the World Health Organization Classification, International Consensus Classification, and National Comprehensive Cancer Network guidelines were recently updated to better integrate these molecular insights into clinical practice. We summarise here the molecular biomarkers of lymphomas with an emphasis on biomarkers that have well-supported prognostic and predictive utility, as well as emerging biomarkers that show promise for clinical practice. These biomarkers include: (1) diagnostic entity-defining genetic abnormalities [e.g., B-cell acute lymphoblastic leukaemia (B-ALL) with KMT2A rearrangement]; (2) molecular alterations that guide patients' prognoses (e.g., TP53 loss frequently conferring worse prognosis); (3) mutations that serve as the targets of, and often a source of acquired resistance to, small molecular inhibitors (e.g., ABL1 tyrosine kinase inhibitors for B-ALL BCR::ABL1, hindered by ABL1 kinase domain resistance mutations); (4) the growing incorporation of molecular measurable residual disease (MRD) in the management of lymphoma patients (e.g., molecular complete response and sequencing MRD-negative criteria in multiple myeloma). Altogether, our review spans the spectrum of lymphoma types, from the genetically defined subclasses of precursor B-cell lymphomas to the highly heterogeneous categories of small and large cell mature B-cell lymphomas, Hodgkin lymphomas, plasma cell neoplasms, and T/NK-cell lymphomas, and provides an expansive summary of our current understanding of their molecular pathology.


Assuntos
Linfoma de Células B , Linfoma , Humanos , Prognóstico , Linfoma/diagnóstico , Linfoma/genética , Linfoma/patologia , Linfoma de Células B/diagnóstico , Mutação
9.
Am J Surg Pathol ; 48(4): 395-405, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38287877

RESUMO

After allogeneic hematopoietic stem cell transplantation (HSCT), accurate differentiation between donor-derived post-transplant lymphoproliferative disorder (PTLD) and relapse of recipient-derived lymphoproliferative disorder (LPD) is crucial for determining treatment. Conventional diagnostic approaches for PTLD include histopathological examination, flow cytometry, and chimerism analysis of bulk tumor tissue. However, these methods are inconclusive in cases in which the primary disease is an Epstein-Barr virus (EBV)-positive LPD and is of the same lineage as that of the post-HSCT LPD tumor cells. Particularly, in cases where the number of tumor cells in the tissue is low, it is difficult to determine the origin of tumor cells. In this study, we developed a new method to simultaneously detect signals using sex chromosome fluorescence in situ hybridization, immunofluorescence staining, and EBV-encoded small RNA in situ hybridization on a single section of formalin-fixed paraffin-embedded histopathological specimen. The utility of the method was validated using specimens from 6 cases of EBV-positive LPD after sex-mismatched HSCT that were previously difficult to diagnose, including Hodgkin lymphoma-like PTLD that developed after HSCT for Hodgkin lymphoma and recurrence of chronic active EBV infection. This method successfully preserved the histologic structure after staining and allowed accurate determination of tumor cell origin and lineage at the single-cell level, providing a definitive diagnosis in all cases. This method provides a powerful tool for the diagnosis of LPDs after sex-mismatched HSCT.


Assuntos
Infecções por Vírus Epstein-Barr , Transplante de Células-Tronco Hematopoéticas , Doença de Hodgkin , Linfoma , Transtornos Linfoproliferativos , Humanos , Infecções por Vírus Epstein-Barr/diagnóstico , Herpesvirus Humano 4/genética , Hibridização in Situ Fluorescente , Recidiva Local de Neoplasia , Transtornos Linfoproliferativos/diagnóstico , Transtornos Linfoproliferativos/etiologia , Transtornos Linfoproliferativos/terapia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Doença Crônica
10.
J Vis Exp ; (203)2024 Jan 12.
Artigo em Inglês | MEDLINE | ID: mdl-38284545

RESUMO

Vitreoretinal lymphoma (VRL) represents an aggressive lymphoma, often categorized as primary central nervous system diffuse large B-cell lymphoma. To diagnose VRL, specimens such as vitreous humor and, more recently, aqueous humor are collected. Diagnostic testing for VRL on these specimens includes cytology, flow cytometry, and molecular testing. However, both cytopathology and flow cytometry, along with molecular testing using cellular DNA, necessitate intact whole cells. The challenge lies in the fact that vitreous and aqueous humor typically have low cellularity, and many cells get destroyed during collection, storage, and processing. Moreover, these specimens pose additional difficulties for molecular testing due to the high viscosity of vitreous humor and the low volume of both vitreous and aqueous humor. This study proposes a method for extracting cell-free DNA from vitreous and aqueous specimens. This approach complements the extraction of cellular DNA or allows the cellular component of these specimens to be utilized for other diagnostic methods, including cytology and flow cytometry.


Assuntos
Ácidos Nucleicos Livres , Neoplasias Oculares , Linfoma , Neoplasias da Retina , Humanos , Corpo Vítreo , Neoplasias da Retina/diagnóstico , Neoplasias da Retina/genética , Neoplasias da Retina/patologia , Humor Aquoso , Biomarcadores Tumorais/genética , Neoplasias Oculares/patologia , Linfoma/diagnóstico , Linfoma/genética , Linfoma/patologia , DNA
11.
Eur J Surg Oncol ; 50(1): 107268, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38043361

RESUMO

Intestinal lymphomas can rarely present as abdominal catastrophes with perforation or small bowel obstruction. There is little data regarding their optimal surgical management and associated outcomes. We aimed to systematically review relevant published literature to assess the presentation, diagnosis, optimal surgical approach and associated post-operative outcomes. A systematic on-line literature search of Embase and Medline identified 1485 articles of which 34 relevant studies were selected, including 7 retrospective studies, 1 case series and 26 case reports. Selected articles were assessed by two reviewers to extract data. 95 patients with abdominal catastrophes secondary to lymphoma (predominately Burkitt (28 %) and Diffuse Large B-cell lymphoma (29 %)) were identified with a median age of 52 years, 40 % were female. Of the small bowel resections 25% (n = 18) suffered post-operative complications with a 13.8 % (n = 10) 30-day mortality. Ileocolonic resections had a 27 % complication rate with 18 % mortality and primary repair had a 25 % complications rate and 25 % mortality. Median follow-up was 8 days (range 1-96). Notable points of differences in the presentations between these different lymphomas included the majority of Burkitt's lymphoma were younger, had a known diagnosis, were on chemotherapy and presented with perforation in contrast to those with B cell lymphoma who were predominately older, had new diagnoses and presented with a balanced proportion of obstruction and perforation. Abdominal catastrophes secondary to intestinal lymphomas most commonly present with perforation. Aggressive surgical management, including small bowel resection, may offer similar remission rates for lymphoma patients presenting with abdominal catastrophes as those without such emergency complications.


Assuntos
Linfoma de Burkitt , Neoplasias Intestinais , Obstrução Intestinal , Linfoma , Humanos , Feminino , Pessoa de Meia-Idade , Masculino , Laparotomia , Estudos Retrospectivos , Linfoma/complicações , Linfoma/cirurgia , Linfoma de Burkitt/complicações , Linfoma de Burkitt/diagnóstico , Linfoma de Burkitt/cirurgia , Obstrução Intestinal/etiologia , Obstrução Intestinal/cirurgia , Neoplasias Intestinais/complicações , Neoplasias Intestinais/cirurgia
12.
Ann Hematol ; 103(3): 905-915, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38057424

RESUMO

The study analysed the clinical characteristics, treatment approaches, and survival outcomes of 97 consecutive patients with orbital lymphoma (OL) over a 25-year period at. The median age of the patients was 57.6 years, and 59.8% (n = 58) were male. Marginal zone lymphoma constitutes the most prevalent subtype, accounting for 67% of cases, whereas other common subtypes include diffuse large B-cell lymphoma, follicular lymphoma, mantle cell lymphoma, Burkitt lymphoma, and T-cell lymphomas. Unilateral involvement was observed in the majority of cases (72.3%). Common clinical presentations included mass (30.9%), swelling (26.8%), and epiphora (11.3%). Of the patients, 7.2% received rituximab alone, 14.4% received radiotherapy alone, 48.5% received chemotherapy, 27.8% received radiotherapy plus rituximab, 22.7% received radiotherapy plus chemotherapy, and 5.2% underwent surgery as the first-line treatment. During a median follow-up of 4.3 years, 15.5% of patients experienced relapse or disease progression. The 5-year and 10-year progression-free survival rates were 84.1% and 79.1%, respectively. This study contributes to our understanding of OLs and provides a foundation for further investigations in this field. Male gender, presence of B symptoms, advanced stage, secondary orbital lymphoma, aggressive histological subtype, and elevated serum lactate dehydrogenase levels were associated with poorer (either inferior or worse) progression-free survival.


Assuntos
Linfoma de Zona Marginal Tipo Células B , Linfoma Folicular , Linfoma , Neoplasias Orbitárias , Humanos , Masculino , Adulto , Pessoa de Meia-Idade , Feminino , Rituximab , Prognóstico , Recidiva Local de Neoplasia , Neoplasias Orbitárias/epidemiologia , Neoplasias Orbitárias/terapia , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/terapia , Linfoma de Zona Marginal Tipo Células B/patologia , Estudos Retrospectivos
13.
Ann Hematol ; 103(2): 553-563, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37951851

RESUMO

We performed a molecular analysis of formalin-fixed paraffin embedded and decalcified bone marrow trephine biopsies of 41 patients with a B-cell disorder with lymphoplasmacytic differentiation to enable a more precise diagnosis and to describe potentially prognostic and therapeutic relevant mutations. Analysis was performed with a commercially available next-generation sequencing (NGS) lymphoma panel (Lymphoma Solution, SophiaGenetics). Results were correlated with clinical and pathological parameters. Our group covered a spectrum of B-cell disorders with plasmacytic differentiation ranging from Waldenstroem's macroglobulinemia (WM), to small-B-cell lymphomas with plasmacytic differentiation (SBCL-PC) to IgM myeloma (MM). The most helpful diagnostic criteria included morphology and immuno-phenotype as a prerequisite for the interpretation of molecular analysis. MYD88 mutation was present in nearly all WM, but also in 50% of SBCL-PCs, while MM were consistently negative. Driver mutations, such as TP53, were already detectable early in the course of the respective diseases indicating a higher risk of progression, transformation, and reduced progression-free survival. In addition, we report on a novel BIRC3 frameshift mutation in one case of a progressive WM. Our data indicate that patients with LPL/WM might benefit from thorough pathological work-up and detailed molecular analysis in terms of precise diagnosis and targeted treatment allocation.


Assuntos
Leucemia Linfocítica Crônica de Células B , Linfoma , Macroglobulinemia de Waldenstrom , Humanos , Linfoma/patologia , Macroglobulinemia de Waldenstrom/diagnóstico , Macroglobulinemia de Waldenstrom/genética , Macroglobulinemia de Waldenstrom/tratamento farmacológico , Leucemia Linfocítica Crônica de Células B/patologia , Medula Óssea/patologia , Mutação , Fator 88 de Diferenciação Mieloide/genética
15.
Arch Pathol Lab Med ; 148(3): 292-298, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-37270801

RESUMO

CONTEXT.­: In their 2014 article "New Immunohistochemistry for B-cell Lymphoma and Hodgkin Lymphoma," Zhang and Aguilera reviewed new immunohistochemical markers for B-cell lymphoma and Hodgkin lymphoma and described how to use these markers for correct lymphoma diagnoses, using the 2008 World Health Organization classifications. Recently, the World Health Organization's WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues published 2022 updates, and, in quick sequence, a second group published an alternative International Consensus Classification of myeloid neoplasms, acute leukemias, and mature lymphoid neoplasms. Regardless of the system a hematopathologist chooses to follow, updates in the immunohistochemical diagnosis of disease are described in both publications as well as in the primary literature. In addition to updated classifications, the increasing use of small biopsy samples for the evaluation of lymphadenopathy continues to challenge hematopathology diagnosis and increase the utilization of immunohistochemistry. OBJECTIVE.­: To review new immunohistochemical markers or new uses of previously known immunohistochemical markers in the evaluation of hematolymphoid neoplasia for the practicing hematopathologist. DATA SOURCES.­: Data were obtained from a literature review and personal practice experience. CONCLUSIONS.­: The practicing hematopathologist requires knowledge of the ever-expanding repertoire of immunohistochemistry for the diagnosis and treatment of hematolymphoid neoplasia. New markers presented in this article help to complete our understanding of disease, diagnosis, and management.


Assuntos
Doença de Hodgkin , Linfoma de Células B , Linfoma , Humanos , Doença de Hodgkin/diagnóstico , Imuno-Histoquímica , Organização Mundial da Saúde
18.
J Cell Mol Med ; 28(3): e18060, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38095234

RESUMO

The variant form of hairy cell leukaemia (HCL-V) is a rare disease very different from hairy cell leukaemia (HCL), which is a very well-defined entity. The 5th WHO edition (Leukemia, 36, 2022 and 1720) classification (WHO-HAEM5) introduced splenic lymphomas/leukaemias including four different entities: (1) HCL, (2) splenic marginal zone lymphoma (SMZL) with circulating villous cells in the peripheral blood, (3) splenic lymphoma with prominent nucleolus (SLPN), which replaced HCL-V and CD5 negative B-prolymphocytic leukaemia (B-PLL), and (4) splenic diffuse red pulp lymphoma (SDRPL). All these entities have to be distinguished because of a different clinical course and the need for a different treatment. The diagnosis can be challenging because of complex cases and overlap and/or grey zones between all the entities and needs integrating clinical, histologic, immunophenotypic, cytogenetic and molecular data. We review the diagnostic criteria including clinical, immunophenotypic and molecular characteristics of patients with HCL-V and other HCL-like disorders including HCL, SDRPL, SMZL, B-PLL and the Japanese form of HCL. We also discuss the different criteria allowing us to separate these different entities and we will update the recent therapeutic options that have emerged, in particular the advances with chemoimmunotherapy and/or targeted therapies.


Assuntos
Leucemia de Células Pilosas , Leucemia Linfocítica Crônica de Células B , Linfoma , Humanos , Leucemia de Células Pilosas/diagnóstico , Leucemia de Células Pilosas/patologia , Baço/patologia
20.
J Vet Med Sci ; 86(2): 160-167, 2024 Feb 08.
Artigo em Inglês | MEDLINE | ID: mdl-38104975

RESUMO

Differentiating intestinal T-cell lymphoma from chronic enteropathy (CE) in endoscopic samples is often challenging. In the present study, automated machine learning systems were developed to distinguish between the two diseases, predict clonality, and detect prognostic factors of intestinal lymphoma in cats. Four models were created for four experimental conditions: experiment 1 to distinguish between intestinal T-cell lymphoma and CE; experiment 2 to distinguish large cell lymphoma, small cell lymphoma, and CE; experiment 3 to distinguish granzyme B+ lymphoma, granzyme B- lymphoma, and CE; and experiment 4 to distinguish between T-cell receptor (TCR) clonal population and TCR polyclonal population. After each experiment, a pathologist reviewed the test images and scored for lymphocytic infiltration, epitheliotropism, and epithelial injury. The models of experiments 1-4 achieved area under the receiver operating characteristic curve scores of 0.943 (precision, 87.59%; recall, 87.59%), 0.962 (precision, 86.30%; recall, 86.30%), 0.904 (precision, 82.86%; recall, 80%), and 0.904 (precision, 81.25%; recall, 81.25%), respectively. The images predicted as intestinal T-cell lymphoma showed significant infiltration of lymphocytes and epitheliotropism than CE. These models can provide evaluation tools to assist pathologists with differentiating between intestinal T-cell lymphoma and CE.


Assuntos
Doenças do Gato , Doenças Inflamatórias Intestinais , Linfoma de Células T , Linfoma , Gatos , Animais , Granzimas , Doenças Inflamatórias Intestinais/veterinária , Linfoma/veterinária , Linfoma de Células T/veterinária , Receptores de Antígenos de Linfócitos T , Aprendizado de Máquina , Doenças do Gato/diagnóstico
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