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1.
BMJ Case Rep ; 17(9)2024 Sep 20.
Artículo en Inglés | MEDLINE | ID: mdl-39306337

RESUMEN

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive haematological malignancy, typically characterised by cutaneous lesions and bone marrow involvement. We present a unique case of a woman in her 70s, initially seen for a spontaneous swelling on her left external ear resembling a haematoma, which recurred after initial treatment, triggering further evaluation.Diagnostic challenges arose as the patient displayed positive markers for Myeloperoxidase (MPO) (p-ANCA), suggesting vasculitis. Dermatology considered various differential diagnoses, but imaging and tests ruled out significant pathology. Steroid treatment led to improvement, but coincided with a surge in white cell count (WCC), prompting an urgent haematological review.Subsequent investigations, including a punch biopsy of the external ear and a bone marrow biopsy revealed BPDCN concurrent with chronic myelomonocytic leukaemia. This case highlights the challenging diagnostic journey, emphasising the need for multidisciplinary collaboration and the potential for unique BPDCN presentations, expanding our understanding of this malignancy.


Asunto(s)
Células Dendríticas , Leucemia Mielomonocítica Crónica , Humanos , Femenino , Células Dendríticas/patología , Anciano , Diagnóstico Diferencial , Leucemia Mielomonocítica Crónica/diagnóstico , Leucemia Mielomonocítica Crónica/complicaciones , Leucemia Mielomonocítica Crónica/patología , Oído Externo/patología , Neoplasias del Oído/patología , Neoplasias del Oído/diagnóstico , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/diagnóstico , Neoplasias Hematológicas/diagnóstico , Neoplasias Hematológicas/patología , Neoplasias Hematológicas/complicaciones
2.
BMJ Case Rep ; 17(9)2024 Sep 20.
Artículo en Inglés | MEDLINE | ID: mdl-39306333

RESUMEN

Gastric cancer primarily metastasizes to the peritoneum, liver and lungs, with bone marrow involvement being a rare occurrence, found in less than 1% of cases. Disseminated carcinomatosis of the bone marrow (DCBM) is characterised by widespread infiltration of cancer cells into the bone marrow, leading to haematological disorders such as disseminated intravascular coagulation and thrombocytopenia. We present a unique case of a man in his late 50s with acute thrombocytopenia as the initial symptom, subsequently diagnosed with gastric cancer on bone marrow examination. Despite receiving chemotherapy, the patient's condition deteriorated rapidly, emphasising the challenging management and poor prognosis associated with DCBM. This case underscores the need for improved diagnostic strategies and therapeutic approaches to enhance patient outcomes in DCBM associated with gastric cancer.


Asunto(s)
Adenocarcinoma , Neoplasias de la Médula Ósea , Neoplasias Gástricas , Humanos , Masculino , Neoplasias Gástricas/patología , Neoplasias Gástricas/secundario , Neoplasias de la Médula Ósea/secundario , Adenocarcinoma/secundario , Adenocarcinoma/patología , Persona de Mediana Edad , Resultado Fatal , Médula Ósea/patología , Trombocitopenia
3.
Parasitology ; : 1-9, 2024 Sep 23.
Artículo en Inglés | MEDLINE | ID: mdl-39311405

RESUMEN

Visceral leishmaniasis (VL) is a severe infectious disease caused by protozoan parasites of the Leishmania donovani complex. Blood cytokine concentrations in VL patients can inform us about underlying immunopathogenesis and may serve as a biomarker for treatment effectiveness. However, cytokine levels have not yet been studied in VL patients from Kenya, where case load is high. This study measured the serum cytokine profile, blood parasite load and clinical and haematological features of VL patients from West Pokot County, Kenya, over the course of treatment with sodium stibogluconate and paromomycin (SSG-PM). VL patients recruited at the hospital presented with splenomegaly and weight loss, and frequently had pancytopenia and anaemia. Median Leishmania parasite load in blood, determined with real-time polymerase chain reaction, was 2.6 × 104 parasite equivalents mL−1. Compared to endemic healthy controls, serum interferon gamma (IFN-γ), interleukin 5 (IL-5), IL-6, IL-10, IL-12p70, IL-17A and IL-27 were significantly elevated in untreated VL patients. Severe VL was associated with higher IL-10 and lower IFN-γ levels. After 17 daily injections with SSG-PM, disease symptoms disappeared, leukocyte and thrombocyte counts significantly increased, and blood parasite load decreased to undetectable levels in all VL patients. There was a significant decrease in IL-10 and IL-6, whereas IL-17A levels increased; the remaining cytokines showed no significant concentration change during treatment. In conclusion, the results suggest that SSG-PM treatment of VL patients from West Pokot was effective. Moreover, both inflammatory and regulatory immune responses appeared to decrease during treatment, although the increase in IL-17A could reflect a partial continuation of immune activation.

4.
Explor Target Antitumor Ther ; 5(4): 877-901, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39280243

RESUMEN

Splenic marginal zone lymphoma (SMZL) is a rare, predominantly indolent B-cell lymphoma constituting fewer than 2% of lymphoid neoplasms. However, around 30% of patients have a shorter survival despite currently available treatments and the prognosis is especially poor for the 5-15% of cases that transform to a large cell lymphoma. Mounting evidence suggests that the molecular pathogenesis of SMZL is critically shaped by microenvironmental triggering and cell-intrinsic aberrations. Immunogenetic investigations have revealed biases in the immunoglobulin gene repertoire, indicating a role of antigen selection. Furthermore, cytogenetic studies have identified recurrent chromosomal abnormalities such as deletion of the long arm of chromosome 7, though specific disease-associated genes remain elusive. Our knowledge of SMZL's mutational landscape, based on a limited number of cases, has identified recurring mutations in KLF2, NOTCH2, and TP53, as well as genes clustering within vital B-cell differentiation pathways. These mutations can be clustered within patient subgroups with different patterns of chromosomal lesions, immunogenetic features, transcriptional signatures, immune microenvironments, and clinical outcomes. Regarding SMZL epigenetics, initial DNA methylation profiling has unveiled epigenetically distinct patient subgroups, including one characterized by elevated expression of Polycomb repressor complex 2 (PRC2) components. Furthermore, it has also demonstrated that patients with evidence of high historical cell division, inferred from methylation data, exhibit inferior treatment-free survival. This review provides an overview of our current understanding of SMZL's molecular basis and its implications for patient outcomes. Additionally, it addresses existing knowledge gaps, proposes future research directions, and discusses how a comprehensive molecular understanding of the disease will lead to improved management and treatment choices for patients.

5.
Conserv Physiol ; 12(1): coae064, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39309467

RESUMEN

Reproduction is a physiologically demanding process for sea turtles. Health indicators, including morphometric indices and blood analytes, provide insight into overall health, physiology and organ function for breeding sea turtles as a way to assess population-level effects. The Archie Carr National Wildlife Refuge (ACNWR) on Florida's central eastern coast is critical nesting habitat for loggerhead sea turtles (Caretta caretta), but health variables from this location have not been documented. Objectives of the study were to (1) assess morphometrics and blood analyte data (including haematology, plasma biochemistry, protein electrophoresis, ß-hydroxybutyrate, trace nutrients, vitamins and fatty acid profiles) from loggerheads nesting on or near the beaches of the ACNWR, (2) investigate correlations of body condition index (BCI) with blood analytes and (3) analyse temporal trends in morphometric and blood analyte data throughout the nesting season. Morphometric and/or blood analyte data are reported for 57 nesting loggerheads encountered between 2016 and 2019. Plasma copper and iron positively correlated with BCI. Mass tended to decline across nesting season, whereas BCI did not. Many blood analytes significantly increased or decreased across nesting season, reflecting the catabolic state and haemodynamic variations of nesting turtles. Twenty-three of 34 fatty acids declined across nesting season, which demonstrates the physiological demands of nesting turtles for vitellogenesis and reproductive activities, thus suggesting potential utility of fatty acids for the assessment of foraging status and phases of reproduction. The findings herein are relevant for future spatiotemporal and interspecies comparisons, investigating stressor effects and understanding the physiological demands in nesting sea turtles. This information provides comparative data for individual animals in rescue or managed care settings and for assessment of conservation strategies.

6.
BMJ Neurol Open ; 6(2): e000800, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39296526

RESUMEN

Introduction: Immune effector cell-associated neurotoxicity syndrome (ICANS) is a common side-effect of chimeric antigen receptor T-cell (CAR-T) therapy, with symptoms ranging from mild to occasionally life-threatening. The neurological, cognitive, psychiatric and psychosocial sequelae of ICANS are diverse and not well defined, posing a challenge for diagnosis and management. The recovery trajectory of the syndrome is uncertain. Patients are rarely examined in this population pretherapy, adding a layer of complexity to specifying symptoms pertinent solely to CAR-T treatment. We present a protocol of a prospective longitudinal research study of adult patients in a single Australian haematology service undergoing CAR-T therapy. The study will describe neurocognitive features specific to ICANS, characterise the underlying syndrome, capture recovery, identify predictors of differential postinfusion outcomes and determine a set of cognitive instruments necessary to monitor patients acutely. Methods and analysis: This is a prospective longitudinal study that comprises neuropsychological and neurological examinations occurring prior to CAR-T, during the acute post-treatment period, 28 days, 6 months and 12 months post infusion. Data will be sourced from objective psychometric measures, clinical examinations, self-report questionnaires of psychopathology and accounts of subjective cognitive complaint. Ethics and dissemination: This study aims to guide diagnosis, management and monitoring of neurocognitive features of CAR-T cell therapy. Results of this study will be disseminated through publication in peer-reviewed journals and presentations at scientific conferences. All procedures involving human subjects/patients were approved by the Peter MacCallum Cancer Centre Human Research Ethics Committee (21/145).

7.
BMJ Case Rep ; 17(9)2024 Sep 24.
Artículo en Inglés | MEDLINE | ID: mdl-39317481

RESUMEN

Hodgkin's lymphoma (HL)-associated vanishing bile duct syndrome (VBDS) is a paraneoplastic phenomenon leading to cholestasis, end-stage liver failure and potentially death, due to cholestatic liver dysfunction typically precluding the commencement of curative intent chemotherapy. A female in her 20s presented with pruritus, jaundice and cholestatic hepatitis on laboratory tests, confirmed as VBDS on liver biopsy. CT of the chest demonstrated a mediastinal mass and widespread cervical lymphadenopathy. The patient received 30.6 Gy in 17 fractions to the involved sites of disease which led to a marked improvement in liver function, allowing curative intent chemotherapy to be initiated. The patient achieved complete metabolic response and at the most recent follow-up she had no signs of recurrent disease and near-normal liver function tests. This demonstrates that tumour-directed radiotherapy can be used as a potential bridge to curative chemotherapy in early stage HL-associated VBDS.


Asunto(s)
Enfermedad de Hodgkin , Humanos , Enfermedad de Hodgkin/radioterapia , Enfermedad de Hodgkin/complicaciones , Enfermedad de Hodgkin/tratamiento farmacológico , Femenino , Síndromes Paraneoplásicos/tratamiento farmacológico , Enfermedades de los Conductos Biliares/etiología , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Resultado del Tratamiento , Colestasis/etiología
8.
BMJ Case Rep ; 17(9)2024 Sep 12.
Artículo en Inglés | MEDLINE | ID: mdl-39266021

RESUMEN

The t(1;19) (q23;p13) TCF3::PBX1 is a well-described, recurring chromosomal abnormality in B-acute lymphoblastic leukaemia (B-ALL) that has historically been associated with a worse prognosis in paediatric patients. Gene expression profiling has demonstrated that TCF3::PBX1 results in a distinct subtype of B-ALL, leading to its recognition in the most recent WHO and ICC classifications. Though initially believed to be a poor prognostic sign in the adult population, emerging evidence suggests its presence may instead be intermediate or even favourable in B-ALL. However, adults with TCF3::PBX1 are typically younger and often qualify for treatment with paediatric-inspired regimens. Thus, the prognostic significance in this population remains unclear. This translocation appears to be very rare in older adults with B-ALL and its predictive and prognostic nature in this population is unknown. Herein, we explore a case of this translocation occurring in a patient in her 70s. She initially presented to the emergency department with abdominal pain and thrombocytopenia and was subsequently diagnosed with B-ALL. In addition to t(1;19) (q23;p13), a pathologic mutation in the CBL gene was identified. CBL mutations have been implicated in cancer progression and are mostly described in paediatric B-ALL. She was treated with modified Ph-negative EWALL induction (Vincristine, Idarubicin, dexamethasone) and achieved a complete remission. However, she subsequently experienced an early relapse and was refractory to targeted therapy with blinatumomab. After treatment with inotuzumab ozogamicin, she achieved a second complete remission. Unfortunately, she then suffered a central nervous system (CNS) relapse and passed away from complications of her disease. This case serves as an example of the heterogeneous nature of B-ALL. It demonstrates that patients with ostensibly favourable prognostic factors may experience poor response rates to traditional chemotherapy as well as targeted salvage agents. It also illustrates the challenges of treating B-ALL in the elderly population.


Asunto(s)
Proteínas de Fusión Oncogénica , Leucemia-Linfoma Linfoblástico de Células Precursoras B , Proteínas Proto-Oncogénicas c-cbl , Translocación Genética , Humanos , Femenino , Anciano , Proteínas de Fusión Oncogénica/genética , Proteínas Proto-Oncogénicas c-cbl/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras B/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras B/tratamiento farmacológico , Mutación , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Cromosomas Humanos Par 19/genética , Cromosomas Humanos Par 1/genética
9.
BMJ Open ; 14(9): e087723, 2024 Sep 10.
Artículo en Inglés | MEDLINE | ID: mdl-39260843

RESUMEN

INTRODUCTION: Sickle cell disease (SCD) poses a significant global health burden, particularly affecting individuals in developing countries with constrained healthcare resources. While research on self-management in the context of SCD is emerging, it has predominantly focused on primary studies. The aim of the scoping review was to identify and map self-management needs of individuals living with SCD, the strategies they employed to meet those needs, and the support interventions available to them. METHODS AND ANALYSIS: The review was conducted following the Askey and O'Malley's framework to examine the landscape of SCD self-management research. Searches were conducted in PubMed, Scopus, Embase and Dimensions AI, with additional searches in other databases from inception to June 2024 included. Evidence from 14 studies was synthesised to identify self-management needs, strategies and interventions for individuals with SCD. RESULTS: The review identified diverse self-management needs among individuals with SCD, including knowledge deficits, emotional challenges, physical limitations and barriers to healthcare access. Various self-management strategies were reported, such as nutritional management, psychological coping techniques and proactive healthcare management. Self-management interventions, predominantly delivered by healthcare professionals, focused on providing education, skills training and support to individuals with SCD. The outcomes of self-management interventions consistently demonstrated significant improvements across various dimensions, including self-efficacy, knowledge enhancement, self-care practices and psychological well-being among individuals with SCD. CONCLUSION: This scoping review underscores the importance of addressing the diverse self-management needs of individuals with SCD through tailored interventions and support systems to enhance overall well-being and disease management. Healthcare professionals should prioritise the implementation of multidisciplinary self-management interventions that encompass medical, emotional and social aspects of care to effectively support individuals with SCD in managing their condition. Future research should focus on longitudinal studies to assess the long-term effectiveness of self-management interventions in improving patient outcomes.


Asunto(s)
Anemia de Células Falciformes , Países en Desarrollo , Automanejo , Humanos , Anemia de Células Falciformes/terapia , Anemia de Células Falciformes/psicología , Automanejo/métodos , Adaptación Psicológica , Autocuidado
10.
Br J Haematol ; 2024 Sep 29.
Artículo en Inglés | MEDLINE | ID: mdl-39344046

RESUMEN

Large language models (LLMs) are a transformative technology poised to fundamentally change multiple fields including haematology. Here, we review the history of large language model development, describe their current capabilities and identify opportunities in haematology poised to benefit from their judicious application. We conclude by suggesting a framework for the safe and ethical development of LLM-derived tools in a way that minimizes risk and maximizes clinical benefit for haematology physicians and patients.

11.
BMJ Case Rep ; 17(9)2024 Sep 30.
Artículo en Inglés | MEDLINE | ID: mdl-39349299

RESUMEN

We present the case of a man in his 50s with multiple myeloma who developed foot drop after receiving bortezomib-dexamethasone combination chemotherapy. Diagnostic evaluations, including haematological parameters, nerve conduction studies and imaging, were performed to confirm the diagnosis and assess the extent of neuropathy. He was managed conservatively with analgesics and vitamin supplements, and bortezomib was temporarily withheld. The neuropathy gradually improved, and bortezomib was successfully reintroduced without recurrence of foot drop. Bortezomib-induced foot drop is a rare complication of bortezomib-based therapy in patients with multiple myeloma. Early recognition and intervention are crucial to minimise impact on quality of life. This case report emphasises the safe reintroduction of bortezomib post-neuropathy resolution, emphasising the importance of early recognition and multidisciplinary management.


Asunto(s)
Antineoplásicos , Bortezomib , Mieloma Múltiple , Neuropatías Peroneas , Humanos , Bortezomib/efectos adversos , Masculino , Mieloma Múltiple/tratamiento farmacológico , Mieloma Múltiple/complicaciones , Persona de Mediana Edad , Neuropatías Peroneas/inducido químicamente , Neuropatías Peroneas/etiología , Antineoplásicos/efectos adversos , Trastornos Neurológicos de la Marcha/inducido químicamente , Trastornos Neurológicos de la Marcha/etiología , Dexametasona/uso terapéutico , Dexametasona/administración & dosificación , Dexametasona/efectos adversos
12.
BMJ Case Rep ; 17(9)2024 Sep 25.
Artículo en Inglés | MEDLINE | ID: mdl-39322572

RESUMEN

Haemophagocytic lymphohistiocytosis (HLH) is a syndrome with an abnormal activation of the immune system and is associated with a high mortality even with treatment. We present a case of a woman in her mid-50s who developed HLH triggered by miliary tuberculosis (TB) while receiving a tumour necrosis factor alpha inhibitor.The patient was admitted with a high fever and respiratory pain. Her condition deteriorated despite empirical treatment. Diagnosis of HLH was established based on clinical presentation, H-score and HLH-04 criteria. Concurrently, miliary TB was identified as the trigger. She was treated with anti-tuberculous therapy and HLH-directed treatment with dexamethasone, etoposide and anakinra. Initial improvement was observed, leading to the withholding of HLH-orientated treatment. However, several relapses occurred, necessitating prolonged HLH treatment.A literature review corroborated the importance of combined anti-tuberculous and immunosuppressive therapy for managing HLH. This case underscores the necessity of timely and comprehensive management of HLH-oriented treatment.


Asunto(s)
Linfohistiocitosis Hemofagocítica , Tuberculosis Miliar , Humanos , Linfohistiocitosis Hemofagocítica/tratamiento farmacológico , Linfohistiocitosis Hemofagocítica/diagnóstico , Tuberculosis Miliar/tratamiento farmacológico , Tuberculosis Miliar/complicaciones , Tuberculosis Miliar/diagnóstico , Femenino , Persona de Mediana Edad , Antituberculosos/uso terapéutico , Dexametasona/uso terapéutico , Dexametasona/administración & dosificación , Inmunosupresores/uso terapéutico , Etopósido/uso terapéutico , Proteína Antagonista del Receptor de Interleucina 1/uso terapéutico , Terapia de Inmunosupresión/efectos adversos
13.
Bull Cancer ; 111(10): 980-986, 2024 Oct.
Artículo en Francés | MEDLINE | ID: mdl-39266427

RESUMEN

In response to the French hospital system crisis and the challenges faced by the heads of departments, we have undertaken an initiative to create a community of heads of haematology departments willing to assist each other. Our inaugural seminar, held in January 2023, established the foundational "core" group of heads of department. Throughout 2023, this emerging community has prospered, offering sustained support to peers. In January 2024, we broadened our community to include other heads of departments, following a second seminar gathering 36 participants. During this event, we took the time to exchange thoughts and reflect on our missions. Building on the experience of guest speakers and employing methods of co-development and co-construction in plenary sessions, small-group workshops, and social gathering, we were able to discover and experience the collective intelligence, creativity, strength, and support stemming from such a group. This peer community of heads of departments stands as a powerful tool for management support, whereby personal experiences nourish and enrich the experience of others. We hope that our initiative will inspire heads of departments from other specialties so that, together, we can better work towards our missions as heads of departments and collaborate on rebuilding the hospital "from the bottom up".


Asunto(s)
Hematología , Francia , Humanos , Departamentos de Hospitales/organización & administración , Grupo Paritario
14.
Pediatr Blood Cancer ; : e31325, 2024 09 12.
Artículo en Inglés | MEDLINE | ID: mdl-39267236

RESUMEN

The aim of this scoping review is to describe the role, education, policies/regulation, skills and competencies required for advanced practice in paediatric haematology-oncology nursing in Europe, highlighting the differences in development between the different European countries. A scoping review was conducted following the methodological framework of guidelines by Arksey and O'Malley and the recommendations for advancing the methodology by Levac et al. We searched MEDLINE/PubMed, EMBASE, CINAHL, Cochrane Library, Scopus, grey literature, webpages, reference lists and performed a manual search, without any restrictions on language or time. The intersection between databases, grey literature and evidence documents traced from the sites of the most authoritative European organisations in the field made it possible to identify the regulatory and training differences between the various countries that were examined. This scoping review highlights how advanced knowledge and competences are used in the care of paediatric haematology-oncology patients, which are strictly necessary for implementing quality care. At present these competences are not recognised in policies and regulation in most of the countries that were examined. It is desirable that all EU member states work to implement a radical change and allow these more competent figures to assist patients in the best possible way.

15.
BMJ Open ; 14(9): e084372, 2024 Sep 05.
Artículo en Inglés | MEDLINE | ID: mdl-39237275

RESUMEN

BACKGROUND: Assessing multidisciplinary prehabilitation strategies becomes crucial to pre-emptively counter the physical, psychological and social negative impacts experienced during an allogenic haematopoietic stem cell transplant (allo-HSCT) among acute myeloid leukaemia (AML) and myelodysplastic syndrome (MDS) patients. Current evidence is restricted to studies during induction chemotherapy, omitting rehabilitation interventions and predominantly using exercise-only approaches without a multidisciplinary framework. The aim of this study is to investigate the feasibility, safety and preliminary efficacy of multidisciplinary prehabilitation in adults offered allo-HSCT. METHODS AND ANALYSIS: This 8-week single-group pre-post feasibility study aims to pilot a multidisciplinary prehabilitation intervention for participants undergoing allo-HSCT, with a focus on feasibility and safety. Participants, aged 18 or older, diagnosed with AML or MDS, and offered allo-HSCT, will be recruited between June 2023 and July 2024. The multidisciplinary prehabilitation intervention, conducted by the cancer allied health team at the Royal Adelaide Hospital, includes exercise physiology, physiotherapy, dietetics, social work, occupational therapy and psychology interventions. Consistent with a multidisciplinary treatment approach, each component is tailored to address different aspects of patient care, and adherence calculations will assess patient engagement and compliance. In addition, participants will continue to receive usual care from cancer allied health staff. The primary outcome of the study is to assess the feasibility of a multidisciplinary prehabilitation intervention by evaluating intervention uptake, retention, adherence, acceptability and safety. Secondary outcomes are leg strength, upper-body strength, aerobic fitness, falls risk, anthropometry, nutritional status, quality of life, anxiety, depression, self-efficacy for coping with cancer and distress. ETHICS AND DISSEMINATION: Ethics approval for this study has been provided by the Central Adelaide Local Health Network (HREC 2022/HRE00284). Recruitment for the study commenced in June 2023 and will continue until July 2024. The methods have been designed and are reported according to the SPIRIT and CONSORT-pilot study checklist. TRIAL REGISTRATION NUMBER: The Australian New Zealand Clinical Trials Registry (ANZCTR): ACTRN12623000052639.


Asunto(s)
Estudios de Factibilidad , Trasplante de Células Madre Hematopoyéticas , Leucemia Mieloide Aguda , Síndromes Mielodisplásicos , Ejercicio Preoperatorio , Humanos , Trasplante de Células Madre Hematopoyéticas/métodos , Leucemia Mieloide Aguda/terapia , Leucemia Mieloide Aguda/rehabilitación , Síndromes Mielodisplásicos/terapia , Síndromes Mielodisplásicos/rehabilitación , Calidad de Vida , Proyectos Piloto , Adulto , Trasplante Homólogo
16.
Lab Anim ; : 236772241260909, 2024 Sep 30.
Artículo en Inglés | MEDLINE | ID: mdl-39344748

RESUMEN

Outbred stocks of mice are widely used in pre-clinical research as these animals possess a diversified genetic background when compared with inbred strains of mice. It is crucial to assess particular alterations in the physiological and functional profiles of laboratory animals using haematological and biochemical indicators. These values can also differ between laboratories because they are influenced by many different factors. We aimed to provide normal values and reference intervals for selected haematology and biochemistry analytes of 570 ICR mice at three different ages: 6-8 weeks, 10-14 weeks and 6-9 months. Reference values were calculated by non-parametric methods. For comparisons between sexes, the independent-sample t-test and Mann-Whitney test were employed, and analysis of variance was used for age differences. The findings of the study revealed age-related declines in haemoglobin concentration, haematocrit, mean corpuscular volume and mean corpuscular haemoglobin concentrations. Mice aged 6-9 months had statistically higher platelet counts in their blood than mice of other ages. The white blood cell count had a significant age effect and progressively decreased with age. As mice get older, the percentage of neutrophils, monocytes and basophils increases, but the percentage of lymphocytes decreases. For the biochemical values, age-related significant differences in glucose, aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and albumin concentrations were found. It was also found that creatinine concentrations were comparable across all age ranges. The values presented in the present work can be used as a reference to interpret clinical pathology data for other studies and to evaluate health status.

17.
BMJ Case Rep ; 17(9)2024 Sep 11.
Artículo en Inglés | MEDLINE | ID: mdl-39266023

RESUMEN

Multidrug therapy has significantly reduced the global burden of Hansen's disease; however, complications from long-term treatment persist. A male resident of southern Kentucky, in his 30s and of Micronesian descent, presented with worsening abdominal pain associated with anorexia, fatigue, functional decline and occasional haemoptysis. He was compliant with multidrug therapy for leprosy. Laboratory investigations revealed pancytopenia. He was initially treated under a sepsis protocol and later switched to high-dose steroids due to a suspected immune reaction from missed corticosteroid doses. Despite aggressive treatment for refractory pancytopenia, the patient's condition deteriorated, and he passed away from cardiac arrest. Posthumous bone marrow biopsy revealed haemophagocytic lymphohistiocytosis secondary to disseminated histoplasmosis with bone marrow infiltration. This case highlights the importance of proactive fungal screening in immunocompromised leprosy patients, particularly in endemic regions, as early detection and timely intervention can prevent severe complications.


Asunto(s)
Histoplasmosis , Linfohistiocitosis Hemofagocítica , Humanos , Linfohistiocitosis Hemofagocítica/diagnóstico , Linfohistiocitosis Hemofagocítica/tratamiento farmacológico , Linfohistiocitosis Hemofagocítica/etiología , Histoplasmosis/diagnóstico , Histoplasmosis/complicaciones , Histoplasmosis/tratamiento farmacológico , Masculino , Adulto , Resultado Fatal , Lepra/complicaciones , Lepra/diagnóstico , Lepra/tratamiento farmacológico , Huésped Inmunocomprometido
18.
Br J Haematol ; 2024 Sep 14.
Artículo en Inglés | MEDLINE | ID: mdl-39275864

RESUMEN

Given the significant limitations of available literature, central nervous system (CNS) prophylaxis in large B-cell lymphomas remains debatable. Wilson and colleagues provide cautious recommendations, on a case-by-case basis, useful to guide discussion with individual patient. In daily practice, CNS relapse risk, prophylaxis safety and prognosis of CNS recurrence must be considered. Commentary on: Wilson et al. Central nervous system prophylaxis in large B-cell lymphoma: A British Society for Haematology Good Practice Paper. Br J Haematol 2024 (Online ahead of print). doi: 10.1111/bjh.19686.

19.
Br J Haematol ; 2024 Sep 03.
Artículo en Inglés | MEDLINE | ID: mdl-39226157

RESUMEN

Large language models (LLMs) have significantly impacted various fields with their ability to understand and generate human-like text. This study explores the potential benefits and limitations of integrating LLMs, such as ChatGPT, into haematology practices. Utilizing systematic review methodologies, we analysed studies published after 1 December 2022, from databases like PubMed, Web of Science and Scopus, and assessing each for bias with the QUADAS-2 tool. We reviewed 10 studies that applied LLMs in various haematology contexts. These models demonstrated proficiency in specific tasks, such as achieving 76% diagnostic accuracy for haemoglobinopathies. However, the research highlighted inconsistencies in performance and reference accuracy, indicating variability in reliability across different uses. Additionally, the limited scope of these studies and constraints on datasets could potentially limit the generalizability of our findings. The findings suggest that, while LLMs provide notable advantages in enhancing diagnostic processes and educational resources within haematology, their integration into clinical practice requires careful consideration. Before implementing them in haematology, rigorous testing and specific adaptation are essential. This involves validating their accuracy and reliability across different scenarios. Given the field's complexity, it is also critical to continuously monitor these models and adapt them responsively.

20.
BMJ Case Rep ; 17(9)2024 Sep 03.
Artículo en Inglés | MEDLINE | ID: mdl-39231562

RESUMEN

We present a case of a woman in her 20s with inadequately treated systemic lupus erythematosus (SLE). She presented with heavy menstrual bleeding, along with nasal and gum bleeding worsening over 3 months. There was no bleeding history in her family, childhood, dental procedures or childbirth. Evaluation ruled out structural causes, revealing prolonged activated partial thromboplastin time (incomplete correction on mixing studies), normal prothrombin time, moderate thrombocytopenia, and lupus anticoagulant and anti-phosphatidylserine/prothrombin antibody positivity twice, 12 weeks apart. Further evaluation showed very low von Willebrand factor (vWF) levels (<5%). She was treated with pulse methylprednisolone for 3 days, resulting in complete symptom resolution and improvement in vWF levels to 130%. The absence of bleeding history, family history, presence of very low vWF and its response to corticosteroids led to a diagnosis of acquired vWF syndrome as the cause of mucosal bleeding in an SLE patient with concomitant positive antiphospholipid antibody. She was discharged on hydroxychloroquine, mycophenolate mofetil and tapering oral corticosteroids.


Asunto(s)
Anticuerpos Antifosfolípidos , Lupus Eritematoso Sistémico , Enfermedades de von Willebrand , Humanos , Femenino , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/inmunología , Lupus Eritematoso Sistémico/diagnóstico , Anticuerpos Antifosfolípidos/sangre , Enfermedades de von Willebrand/complicaciones , Enfermedades de von Willebrand/diagnóstico , Enfermedades de von Willebrand/tratamiento farmacológico , Enfermedades de von Willebrand/etiología , Adulto , Menorragia/etiología , Menorragia/tratamiento farmacológico , Metilprednisolona/uso terapéutico
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