[Allogeneic transplantation as a therapeutic option for atypical chronic myeloid leukemia].

Atypical chronic myeloid leukemia (aCML) is a rare disease classified as a myelodysplastic/myeloproliferative neoplasm (MDS/MPN). Recent advances in gene mutational profiling have clarified the characteristics of aCML as a disease entity relative to other MDS/MPNs. Although some studies suggest the efficacy of DNA demethylating agents and tyrosine kinase inhibitors, data about these agents are limited due to the small number of patients. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is only therapeutic option that can provide durable remission for aCML and other MDS/MPNs. Retrospective studies from Europe and Japan revealed the clinical results of allo-HSCT for aCML. This review summarizes the pathogenesis of aCML and the development of allo-HSCT and other therapeutic options.

Bilateral atypical femur fracture in a patient with breast cancer taking zoledronic acid and denosumab: a case report.

A 54-year-old woman developed stage IV breast cancer 8 years prior. Chemotherapy was administered, and she was started on zoledronic acid treatment for her bone metastases. Her chemotherapy regimen was then switched, owing to disease progression. Fifty-seven months after starting treatment with zoledronic acid, the patient suffered an atypical femoral fracture of her right femur, for which she underwent surgery. Twenty months later, she developed another atypical femoral fracture in her left femur and underwent intramedullary nail fixation. Zoledronic acid and denosumab use in patients with metastatic bone tumors caused by breast cancer should be done cautiously, considering atypical femoral fracture risk.

Uterine-Conserving Treatment Options for Atypical Endometrial Hyperplasia and Early Endometrial Cancer.

PURPOSE OF REVIEW: This review aims to synthesize available literature on uterine-conserving treatment options for atypical endometrial hyperplasia and grade 1 endometrial carcinoma while highlighting remaining unanswered questions. RECENT FINDINGS: The need for uterine-conserving treatment options for atypical endometrial hyperplasia and grade 1 endometrial carcinoma is growing with the increasing number of cases in younger patients or those who cannot undergo surgery. We reviewed the oncological and reproductive outcomes associated with endocrine therapies used for atypical endometrial hyperplasia and grade 1 endometrial carcinoma. The rising prevalence of delayed childbearing, obesity, and diabetes in reproductive-age individuals and of medical comorbidities associated with high surgical risk continues to amplify the demand for uterine-conserving therapies. Appropriate patient selection for such therapies is imperative to maximize likelihood of treatment response. The ideal candidates are patients with atypical endometrial hyperplasia or early-stage, low-grade endometrial cancer with no evidence of myometrial invasion or extrauterine disease. The most accepted conservative therapeutic approach is hormonal therapy with close surveillance, with or without eventual hysterectomy following childbearing or failure of treatment. Further prospective and randomized trials are needed to address optimal patient and treatment selection, as well as the use of molecular profiling for treatment individualization and prognostication.

Posterior pituitary tumors and other rare entities involving the pituitary gland.

Non-neuroendocrine tumors account for around 10% of all primary neoplasms of the sella. If meningiomas, craniopharyngiomas, and germ cell tumors are excluded, the remaining lesions include a broad spectrum of uncommon, benign, and aggressive, often diagnostically challenging lesions. This review aims to summarize the essential clinicopathological features of tumors of the posterior pituitary gland, infundibulum spectrum expressing thyroid transcription factor 1, and primary sellar atypical rhabdoid teratoid tumor, and provide the criteria for their diagnosis and management.

Evaluation of Antipsychotic-Induced Neuroleptic Malignant Syndrome Using a Self-Organizing Map.

Introduction In neuropsychiatric pharmacotherapy, neuroleptic malignant syndrome (NMS) is a potentially serious side effect of antipsychotics characterized primarily by fever, disorientation, extrapyramidal disorders, and autonomic nervous system imbalance, which can lead to death if left untreated. We visualized the NMS profile of antipsychotics using a self-organizing map (SOM). We combined it with decision tree analysis to discriminate between 31 antipsychotics in more detail than typical antipsychotic (TAP) and atypical antipsychotic (AAP) classifications. Method A total of 20 TAPs and 11 AAPs were analyzed. We analyzed NMS reports extracted from the Japanese Adverse Drug Event Report (JADER) database based on standardized Medical Dictionary for Regulatory Activities (MedDRA) queries (Standardized MedDRA Queries (SMQ) code: 20000044, including 68 preferred terms). The SOM was applied using the SOM package in R version 4.1.2 (R Foundation for Statistical Computing, Vienna, Austria). Results The Japanese Adverse Drug Event Report (JADER) database contained 887,704 reports published between April 2004 and March 2024. The numbers of cases of NMS (SMQ code: 20000044) reported for risperidone, aripiprazole, haloperidol, olanzapine, and quetiapine were 1691, 1294, 1132, 1056, and 986, respectively. After the antipsychotics were classified into six units using SOM, they were adapted for decision tree analysis. First, 31 antipsychotics branched off into groups with loss of consciousness, with one group (10 TAPs) consisting entirely of TAPs, and the other consisting of antipsychotics that were further separated into two groups with coma induced by TAPs and AAPs. Conclusion The results of this study provide a reference for healthcare providers when predicting the NMS characteristics induced by each drug in patients, thereby facilitating the effective treatment of schizophrenia.

Incidental finding of aberrant left common carotid artery during intracerebral hemorrhage examination.

We report a case involving an aortic arch anomaly that has not been previously documented. The patients were a 76-year-old female who was urgently transported to the hospital because of a sudden disturbance of consciousness. Neurological symptoms indicated impending brain herniation, and the patient was diagnosed with hypertensive intracerebral hemorrhage (left putamen-thalamus) and acute hydrocephalus, thereafter she died approximately 6 hours after arrival. Head and neck-to-chest computed tomography angiography revealed an atypical aortic arch branching pattern. From the proximal side, the left common carotid artery, right common carotid artery, right subclavian artery, and left subclavian artery originated sequentially, suggesting an aberrant left common carotid artery. In the context of acute stroke management, understanding such aortic arch variations is crucial for planning access routes and treatment strategies for neuroendovascular procedures.

Atypical Vancouver B1 periprosthetic fracture of the proximal femur in the United Kingdom: a case report challenged by myeloma, osteoporosis, infection, and recurrent implant failures.

The indications for total hip replacement are increasing and not limited to osteoarthritis. Total hip replacement may also be done for trauma and pathological fractures in patients otherwise physiologically fit and active. This trend has led to an inevitable rise in complications such as periprosthetic femoral fracture. Periprosthetic femoral fracture can be challenging due to poor bone quality, osteoporosis, and stress fractures. We present a case of periprosthetic femoral fracture in a 71-year-old woman with some components of an atypical femoral fracture. The fracture was internally fixed but was subsequently complicated by infection, implant failure needing revision, and later stress fracture. She was on a bisphosphonate after her index total hip replacement surgery for an impending pathological left proximal femur fracture, and this may have caused the later stress fracture. Unfortunately, she then experienced implant breakage (nonunion), which was treated with a biplanar locking plate and bone grafting. The patient finally regained her premorbid mobility 13 months after the last surgery and progressed satisfactorily towards bony union.

Rare Presentation of Atypical Fractures in a Patient of Autoimmune Polyglandular Syndrome - A Case Report.

Introduction: An atypical bone fracture is a complete or partial discontinuity of bone which occurs without sufficient trauma. Autoimmune polyglandular syndromes are complex conditions characterized by the simultaneous presence of at least two autoimmune-mediated endocrinopathies. These syndromes exhibit considerable heterogeneity in their manifestations and typically unfold sequentially, with a significant time gap between the onset of the initial and subsequent glandular autoimmune diseases. In this article, we report a case of bilateral subtrochanteric atypical fractures in a patient with autoimmune polyglandular syndrome. This case scenario has not been reported in the literature so far to the best of our knowledge. Case Report: A 37-year-old female patient presented at a tertiary care center in a metropolitan city with complaints of pain in both hips and the upper thigh. The patient faced difficulty walking and was unable to do so due to severe pain. Notably, there was no history of a fall or trauma. A radiographic examination revealed bilateral subtrochanteric atypical fractures. Upon further investigation, the patient was also found to be having hypothyroidism, hypoparathyroidism, pancytopenia, and an autoimmune disorder. The patient received a diagnosis of Autoimmune polyglandular syndrome with bilateral subtrochanteric atypical fractures. Treatment involved a comprehensive approach, including thyroxine for hypothyroidism, low-dose steroids, immunosuppressive agents, calcium supplementation, parathyroid hormone, and transfusion of blood and blood products. Due to the fractures being extra-articular, incomplete, and undisplaced, a conservative management approach was adopted. Conclusion: The occurrence of atypical and pathological fractures, particularly when bilateral or recurrent, necessitates a comprehensive evaluation to determine the root cause. It is imperative to address the underlying etiology thoroughly in every patient. Neglecting to do so may lead to a recurrence or insufficient resolution of the pathology. In instances where two or more endocrine organs are involved, consideration should be given to autoimmune polyglandular syndrome as a potential diagnosis.

Supraglottic Neuroendocrine Carcinoma: A Case Report and Literature Review.

Neuroendocrine neoplasms (NENs) are rare in the head and neck region, with the larynx being the most common site. To date, nearly 700 cases of laryngeal neuroendocrine carcinoma (NEC) have been reported in the literature, with an estimated incidence as low as 0.23%. This type of cancer is more prevalent among men aged 50-83 who are heavy smokers. NENs encompass paragangliomas and epithelial neoplasms. The latter categories include neuroendocrine tumors, or typical carcinoids, and NECs, or atypical carcinoids. Due to their nonspecific and often misleading presentation, and given the rarity of this condition, optimal management lacks standardization. Treatment typically involves a combination of surgery, chemotherapy, and radiotherapy. We present a case of supraglottic laryngeal NEC in a 61-year-old female nonsmoker. The patient underwent endoscopic excision followed by adjuvant radiotherapy.

Precision Imaging in Neurodegeneration: The Superiority of Diffusion Tensor Imaging Over Conventional MRI in Differentiating Parkinson's Disease From Atypical Parkinsonian Syndromes.

Background Parkinson's disease (PD) is a progressive neurodegenerative disorder characterized by the degeneration of dopaminergic neurons in the substantia nigra, leading to motor and non-motor symptoms. Atypical parkinsonian syndromes (APS), including progressive supranuclear palsy (PSP) and essential tremor (ET), present with overlapping clinical features, making differential diagnosis challenging. Conventional MRI has limitations in distinguishing PD from APS, necessitating advanced imaging techniques like diffusion tensor imaging (DTI) for more accurate diagnosis. Objectives This retrospective study aimed to evaluate the diagnostic accuracy of DTI in diagnosing PD and APS, particularly assessing its ability to differentiate these conditions from each other compared to conventional MRI. Additionally, the study sought to determine if DTI could diagnose PD in cases where conventional MRI results were normal, thereby highlighting the potential role of DTI in enhancing diagnostic precision in neurodegenerative disorders. Methodology The study included 30 patients with clinically diagnosed PD or APS who underwent both conventional MRI and DTI. Data were collected retrospectively. Imaging was performed using a Philips Multiva 1.5-Tesla MRI scanner (Philips, Amsterdam, Netherlands). DTI sequences were analyzed for fractional anisotropy (FA) values in the substantia nigra, superior cerebellar peduncle, middle cerebellar peduncle, transverse pontine fibers, and dentate nucleus. The FA values were compared with established normal values, and the findings from DTI were correlated with clinical diagnoses and conventional MRI results. Results Among the 30 patients, 53.3% were clinically diagnosed with PD and 46.7% with APS, including PSP and ET. Conventional MRI findings were normal in 46.7% of cases, indicating its limitations in detecting early or subtle changes in neurodegenerative disorders. In contrast, DTI identified abnormalities in 83.3% of cases, demonstrating its superior diagnostic sensitivity. DTI detected significant FA value reductions in the substantia nigra in PD patients (mean FA: 0.440), which is consistent with the degeneration of dopaminergic neurons characteristic of PD. In PSP patients, the superior cerebellar peduncle showed marked FA reductions (mean FA: 0.523), correlating with the clinical features of PSP, such as bradykinesia and postural instability. ET was identified by reduced FA values in the superior cerebellar peduncle and dentate nucleus, distinguishing it from other forms of parkinsonism. DTI was particularly effective in cases where conventional MRI results were inconclusive or normal, identifying early-stage PD and differentiating it from APS with greater accuracy. The study demonstrated a sensitivity of 95.8% and specificity of 93.8% for DTI in differentiating PD from APS compared to conventional MRI. Conclusion This study highlights DTI as a superior imaging modality for the early diagnosis and differentiation of parkinsonian disorders, particularly when conventional MRI results are inconclusive. DTI's ability to detect significant microstructural changes in specific brain regions, evidenced by FA value reductions, enhances diagnostic accuracy. Incorporating DTI into routine clinical practice is essential for accurate differentiation between PD and APS, facilitating better patient management.

Exploring cariprazine as a treatment option for varied depression symptom clusters.

Major depressive disorder (MDD) is among the most prevalent psychiatric conditions and a leading cause of disability worldwide. MDD presents a diverse range of symptoms that significantly impact personal, societal, and economic dimensions. Despite the availability of numerous antidepressant treatments (ADTs) targeting different molecular mechanisms, a substantial proportion of patients experience inadequate response, presenting a considerable challenge in MDD management. As a result, adjunctive strategies, particularly involving atypical antipsychotics, are often employed to enhance treatment efficacy. Cariprazine, a D2/D3 partial agonist, is distinguished from other atypical antipsychotics by its selective action on the D3 receptor and its modulation of 5-HT1A, 5-HT2A, and alpha 1B receptors. This distinctive pharmacological profile warrants investigation into its potential effectiveness and tolerability across various symptom domains of MDD, including pleasure, interest, and motivation; mood and suicidality; sleep and appetite; fatigue; psychomotor activity and anxiety; and cognitive function. Preliminary evidence from animal studies and clinical trials suggests that cariprazine may improve motivation, anhedonia, and cognitive function symptoms. Cariprazine shows promise in alleviating mood-related symptoms, though its impact on anxiety and its effects on agitation and psychomotor retardation remains uncertain. Cariprazine may be particularly beneficial for patients with MDD exhibiting anhedonia, cognitive deficits, and possibly fatigue and hypersomnia. Evaluating cariprazine's efficacy across these symptom domains could reveal patterns that support more personalized treatment approaches for depression. Further research is essential to elucidate the role of cariprazine as an adjunctive therapy for adults with major depressive disorder who have an inadequate response to antidepressant monotherapy.

Atypical Presentation of Oronasal Herpes Simplex Virus (HSV) in an Immunocompromised Patient: A Case Report and Literature Review.

Herpes simplex virus (HSV) typically presents with characteristic mucocutaneous vesicular lesions. However, atypical manifestations can occur and can be challenging to diagnose, especially in immunocompromised individuals. This case report describes a nine-year-old immunocompromised girl who developed a left nasal vestibular ulcer covered by hemorrhagic crustation and granulation tissue, progressively worsening to a friable exophytic lesion with intact nasal mucosa. The lesion was refractory to local treatment, wide-spectrum antibiotics, and antifungal therapy, hence requiring a biopsy. The diagnosis of HSV infection was confirmed by histopathology, and IV acyclovir successfully treated the initial infection. This case emphasizes the importance of considering HSV infection when evaluating persistent, refractory skin lesions, particularly in immunocompromised patients, which can ensure early diagnosis and appropriate antiviral therapy.

Inferior Vena Cava Placement of a Transhepatic Tunneled Dialysis Catheter in a Patient with Atypical Hepatic Venous Anatomy: A Case Report.

A transhepatic hemodialysis (HD) catheter serves as a final option for obtaining HD vascular access in patients whose conventional access sites, including internal jugular veins, external jugular veins, and femoral veins, are no longer viable. This could be due to intravascular thrombosis or central venous stenosis, among others. The ideal catheter tip position in transhepatic tunneled dialysis catheter is the right atrium for optimal blood flow necessary for dialysis. The report presents a case of a 46-year-old female, in whom the traditional vascular access sites for dialysis were not achievable and, thus, required the use of the hepatic access route. However, her case was further complicated due to the unique hepatic vascular anatomy, causing repeated retraction of the catheter tip from the right atrium to the inferior vena cava (IVC) and hepatic vein. This was circumvented by the atypical placement of the catheter tip down to the suprarenal IVC, deep enough to lodge the catheter in place with adequate flow for successful HD.

Treatment of Bisphosphonate-Associated Atypical Femur Fracture With a Combination of Teriparatide and a Novel Surgical Technique.

Background/Objective: Atypical femur fractures (AFFs) caused by long-term bisphosphonate use are associated with high rates of delayed healing and nonunion. Case Report: A 64-year-old woman with osteopenia on alendronate for 15 years sustained a displaced left AFF following a fall from standing height. Imaging showed an acute displaced transverse diaphyseal left femur fracture with lateral cortical thickening and beaking. She underwent an open reduction and internal fixation with insertion of a cephalomedullary nail placed in compression mode, utilizing a novel technique involving intraoperative removal of the endosteal hypertrophied cortical bone at the fracture site. Alendronate was stopped and teriparatide was initiated postoperatively. Radiographs at 3.5 months postsurgery showed evidence of normal fracture union with mature callus formation. Discussion: AFFs caused by prolonged bisphosphonate use have a high rate of delayed healing and nonunion due to abnormal bone remodeling. Use of teriparatide postoperatively has been shown to reduce healing time in small observational studies in surgically treated patients. Our case demonstrates an expedited healing time of 3.5 months using teriparatide combined with a novel surgical technique involving removal of a portion of the abnormally remodeled bone and placement of an intramedullary nail in compression mode. Conclusion: Our case demonstrates an expedited healing time of 3.5 months compared to the average reported healing time for AFF of 10.7 months, supporting the use of the combination of teriparatide and a novel surgical technique.

Data-driven neuroanatomical subtypes of primary progressive aphasia.

The primary progressive aphasias are rare, language-led dementias, with three main variants: semantic, non-fluent/agrammatic, and logopenic. Whilst semantic variant has a clear neuroanatomical profile, the non-fluent/agrammatic and logopenic variants are difficult to discriminate from neuroimaging. Previous phenotype-driven studies have characterised neuroanatomical profiles of each variant on MRI. In this work we used a machine learning algorithm known as SuStaIn to discover data-driven neuroanatomical "subtype" progression profiles and performed an in-depth subtype-phenotype analysis to characterise the heterogeneity of primary progressive aphasia. Our study included 270 participants with primary progressive aphasia seen for research in the UCL Queen Square Institute of Neurology Dementia Research Centre, with follow-up scans available for 137 participants. This dataset included individuals diagnosed with all three main variants (semantic: n=94, non-fluent/agrammatic: n=109, logopenic: n=51) as well as individuals with un-specified primary progressive aphasia (n=16). A data set of 66 patients (semantic n=37, non-fluent/agrammatic: n=29) from the ALLFTD North American cohort study, was used to validate our results. MRI scans were segmented and SuStaIn was employed on 19 regions of interest to identify neuroanatomical profiles independent of the diagnosis. We assessed the assignment of subtypes and stages, as well as their longitudinal consistency. We discovered four neuroanatomical subtypes of primary progressive aphasia, labelled S1 (left temporal), S2 (insula), S3 (temporoparietal), S4 (frontoparietal), exhibiting robustness to statistical scrutiny. S1 correlated strongly with semantic variant, while S2, S3, and S4 showed mixed associations with the logopenic and non-fluent/agrammatic variants. Notably, S3 displayed a neuroanatomical signature akin to a logopenic only signature, yet a significant proportion of logopenic cases were allocated to S2. The non-fluent/agrammatic variant demonstrated diverse associations with S2, S3, and S4. No clear relationship emerged between any of the neuroanatomical subtypes and the unspecified cases. At first follow up 84% of patients' subtype assignment was stable, and 91.9% of patients' stage assignment was stable. We partially validated our findings in the ALLFTD dataset, finding comparable qualitative patterns. Our study, leveraging machine learning on a large primary progressive aphasia dataset, delineated four distinct neuroanatomical patterns. Our findings suggest that separable spatio-temporal neuroanatomical phenotypes do exist within the PPA spectrum, but that these are noisy, particularly for nfvPPA and lvPPA. Furthermore, these phenotypes do not always conform to standard formulations of clinico-anatomical correlation. Understanding the multifaceted profiles of the disease, encompassing neuroanatomical, molecular, clinical, and cognitive dimensions, holds potential implications for clinical decision support.

Distinct clinical profiles and patient outcomes in aCML and CNL.

The classification of atypical chronic myeloid leukemia (aCML) and chronic neutrophilic leukemia (CNL) as a single disease entity remains a topic of debate. To elucidate the characteristics of both entities, this retrospective cohort study was conducted, encompassing 36 cases of aCML and 18 cases of CNL. We discovered that aCML and CNL presented distinct blood counts, genetics, molecular profiles and outcomes. Specifically, hemoglobin levels (P < 0.001) and platelet counts (P < 0.001) were significantly lower in aCML cases than in CNL cases, with no significant difference in mean white blood cells (P = 0.637). The proportion of abnormal karyotypes was higher in aCML cases compared with CNL cases (P = 0.010). Notably, we found that aCML and CNL showed distinct gene expression profiles by transcriptome sequencing technology. The median follow-up duration for the entire cohort was 8 months (rang 0.4 to 36.6 months), and the median overall survival (OS) was significantly shorter in aCML cases (7.3 months, 95%CI 5.4 to 20.5 months) than in CNL cases (median OS not reached). The one-year OS rate for aCML patients was 31.0% (9/29), compared to 92.9% (13/14) for CNL patients. In conclusion, our study supports the notion that aCML and CNL are indeed distinct disease entities characterized by unique hematological features and clinical outcomes.

ACKR3 agonism induces heterodimerization with chemokine receptor CXCR4 and attenuates platelet function.

BACKGROUND: Platelet receptors ACKR3 and CXCR4 play a crucial role in a variety of cardiovascular diseases. Like most chemokine receptors, CXCR4 is a G protein coupled receptor that induces platelet activation. In contrast, the atypical chemokine receptor 3 (ACKR3) lacks the ability to activate heterotrimeric G proteins and its activation leads to platelet inhibition and attenuates thrombus formation. In nucleated cells, heterodimerization of ACKR3 with CXCR4 regulates CXCL12-dependent signalling. The aim of our study was to investigate the formation of ACKR3/CXCR4 heterodimers in platelets and the subsequent consequences for platelet function. METHODS AND RESULTS: Using a proximity ligation assay (PLA, Duolink®) to screen for CXCR4/ACKR3 heterodimerization inducing compounds, we found that ACKR3 agonism but not conventional platelet agonists or endogen ligands lead to heterodimer formation. To further characterize the formation of ACKR3/CXCR4 heterodimers, we studied the CXCL12-dependent platelet activation via CXCR4. Both, CXCL12-dependent platelet aggregation and collagen-dependent ex vivo thrombus formation were significantly downregulated by ACKR3 agonism. Moreover, platelet intracellular calcium and Akt signalling were increased by CXCL12 and again suppressed by ACKR3-specific agonists. Previously, CXCL12 was shown to decrease platelet cAMP levels via CXCR4. Treatment with a specific ACKR3 agonist counteracted this CXCL12/CXCR4-dependent cAMP decrease. CONCLUSION: Our results reveal that the formation of platelet ACKR3/CXCR4 heterodimers is dependent on ACKR3 rather than CXCR4. Furthermore, ACKR3 agonism induced heterodimerization is associated with mitigating CXCL12/CXCR4-dependent platelet activation possibly by modulating CXCR4-dependent G protein signalling. Our results indicate possible ACKR3 agonist functions and reinforce the potential therapeutic applications of ACKR3 agonists.

Eculizumab as first-line treatment for patients with severe presentation of complement factor H antibody-mediated hemolytic uremic syndrome.

BACKGROUND: Complement factor H (FH) antibody-mediated hemolytic uremic syndrome (HUS) has varying prevalence globally. Plasmapheresis and immunosuppressive drugs are the standard treatment. Recently, eculizumab has been reported as an effective alternative. This study aims to report four children with FH antibody-mediated HUS managed with eculizumab plus immunosuppression as first-line therapy. METHODS: A retrospective chart review was conducted for children aged ≤ 18 years old with complement-mediated HUS in two referral centers. Patients with FH antibody-mediated HUS treated with eculizumab as first-line therapy were included. RESULTS: Four children (aged 6-11 years old) were included. Dialysis was necessary in three patients. Eculizumab was administered 5-23 days after onset. None of them received plasmapheresis. Prednisone and mycophenolate mofetil were added after receiving positive FH antibody results. Hematological signs and kidney function improved after the second eculizumab dose. Eculizumab was discontinued in three patients after 6 months. One patient required rituximab due to persistent high FH antibody titers; discontinuation of eculizumab occurred after 15 months without recurrence. No treatment-related complications were observed. During a mean 12-month follow-up (range 6-24 months), no relapses were recorded and all patients ended with normal GFR. CONCLUSION: Our data suggest that a short course of 6 months of C5 inhibitor might be sufficient to reverse thrombotic microangiopathy symptoms and improve kidney function in patients with severe FH antibody-mediated HUS. Simultaneously, adding immunosuppressive agents might reduce the risk of relapse and allow cessation of C5 inhibition in a shorter period of time.

Should we prioritise proper surgical staging for patients with Atypical endometrial hyperplasia (AEH)? Experience from a single-institution tertiary care oncology centre.

OBJECTIVE: The study aimed to evaluate the incidence of concurrent endometrial cancer (EC) and lymph node positivity in patients with Atypical Endometrial Hyperplasia (AEH) who underwent surgical staging with sentinel lymph node evaluation. It also sought to identify the risk factors associated with detecting concurrent endometrial cancer in patients with a preoperative diagnosis of AEH. STUDY DESIGN: A retrospective study was conducted at Amrita Institute of Medical Sciences, involving 54 cases of AEH diagnosed on pre-operative biopsy specimens and undergoing staging surgery between January 1, 2015, and December 31, 2020. The study analysed demographic parameters, clinical presentations, pathological features, and clinical outcomes. Categorical variables were expressed in numbers and percentages, normal distribution data were presented as mean, and non-normal distribution data were presented as median and range. RESULTS: Fifty-four patients diagnosed with AEH underwent surgical staging. The median age was 54 years. Final HistoPathology Report (HPR) showed 48.14 % with AEH and 51.85 % with concurrent EC. Among those with concurrent EC, 96.4 % had type I EC, and one patient was upgraded to type 2 EC. Among them, 17.8 % patients belonged to high-intermediate and high-risk categories. Patients with AEH and concurrent EC were more likely to be diabetic (OR: 3.56, p = 0.04), have a BMI ≥25 kg/m2 (OR: 1.47, p = 0.04), exhibit a thickened endometrial lining of ≥9 mm (OR: 3.13, p = 0.05) on ultrasound, and undergo preoperative biopsy at a non-oncology centre (OR: 8.33, p = 0.001) whereas experiencing heavy menstrual bleeding had a substantially lower likelihood (OR: 0.29, p = 0.01) of developing concurrent EC. CONCLUSION: The study revealed that more than half of patients undergoing staging surgery for AEH were found to be at risk of having concurrent EC in their final HPR. The research also pointed out that surgical staging can help identify both low-risk and high-risk ECs, which may require additional treatment. Higher BMI, diabetes mellitus, and an endometrial thickness of ≥9 mm were identified as significant risk factors for concurrent EC. Additionally, heavy menstrual bleeding was associated with a decreased risk of concurrent EC.