Cutaneous mucormycosis in a critically ill nonagenarian patient caused by Rhizopus species treated with intravenous isavuconazole and topical amphotericin B along with wound debridement.

This case report highlights cutaneous mucormycosis in a 99-year-old patient who was effectively treated with intravenous isavuconazole and local amphotericin B after wound debridement. The patient showed significant improvement within two weeks, highlighting the importance of diagnosis and treatment for this rare fungal infection. It underscores the need for early recognition and suggests that combining antifungal treatments with surgical intervention may be an optimal strategy, even in patients lacking typical risk factors.

Associations of VEGF-D levels with clinical manifestations in lymphangioleiomyomatosis: a cross-sectional analysis of 631 cases.

Background: Lymphangioleiomyomatosis (LAM) is a rare neoplastic disorder characterized predominantly by dyspnea, recurrent pneumothorax, chylous effusion and diffuse pulmonary cystic changes in women. Vascular endothelial growth factor-D (VEGF-D) is an important biomarker for LAM.

Results: This study cohort comprised 631 LAM patients and investigated the correlations between serum VEGF-D levels and clinical manifestations of LAM.

LRG1, a novel serum biomarker for iMCD disease activity.

Idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder characterized by systematic inflammatory symptoms and multiorgan dysfunction caused by a cytokine storm. The current assessment of treatment response in iMCD lack sensitivity due to the heterogeneity of clinical features. We performed proteomic analysis using Data Independent Acquisition (DIA) mass spectrometry (MS) on 33 serum samples in different disease states from 17 patients.

Spontaneous remission of iMCD-TAFRO: a case report.

Idiopathic multicentric Castleman disease (iMCD)- TAFRO (thrombocytopenia, ascites, fever, reticulin fibrosis and organomegaly) is a clinically severe situation characterized by cytokine storms that are potentially fatal, necessitating prompt and decisive medical intervention. The International Castleman's Disease Collaborative Network (CDCN) prioritizes the combination of long-term siltuximab and high-dose steroids as the preferred therapeutic option for iMCD-TAFRO. Here, we report a rare case of spontaneous remission in iMCD-TAFRO.

Whole exome sequencing enhances diagnosis of hereditary bronchiectasis.

Background: Hereditary bronchiectasis refers to a subset of bronchiectasis related to genetic mutations, presenting with common clinical features. Historically, diagnosing this condition has been difficult due to the inaccessibility of diagnostic services coupled with a lack of awareness of the syndrome. We hypothesize that whole exome sequencing (WES) in patients with supporting clinical features, combined with non-genetic testing methods, will enhance the diagnosis of hereditary bronchiectasis.

Quality of life after idiopathic multicentric Castleman disease in China: a cross-sectional, multi-center survey of patient reported outcome and caregiver reported outcome.

The majority of multicentric Castleman disease (MCD) patients in China are of the idiopathic subtype (iMCD) with systemic manifestations. However, the impact of iMCD on life quality, mental and psychological status, social function, and caregiving burden is poorly understood. To address this gap, a cross-sectional web-based survey was conducted with 178 iMCD patients and 82 caregivers, including 42 patient-caregiver dyads.

Sirolimus is effective for refractory/relapsed idiopathic multicentric Castleman disease: A single-center, retrospective study.

Refractory/relapsed idiopathic multicentric Castleman disease (R/R iMCD) has limited treatment options. With studies showing increased mTOR activation in iMCD patients, sirolimus becomes an attractive and promising therapy for R/R iMCD. Here we report the results of a retrospective study involving 26 R/R iMCD patients treated with sirolimus-containing regimen.

Can We Differentiate Between Primary Sjögren Syndrome and Idiopathic Multicentric Castleman Disease Based on the Characteristics of Pulmonary Cysts?

Purpose: To identify radiological characteristics that could help differentiate cystic lung diseases between primary Sjögren syndrome (pSS) and idiopathic multicentric Castleman disease (iMCD).

Patients And Methods: Patients with pSS or iMCD who had cysts were enrolled. Cyst characteristics (number, size, morphology, and distribution) and other accompanying manifestations (nodules, ground-glass opacities, calcification, and thickening of the bronchovascular bundles and interlobular septa) were compared between them.

A prospective study of zanubrutinib, a Bruton tyrosine kinase inhibitor, in relapsed/refractory idiopathic multicentric Castleman disease.

Relapsed and refractory (R/R) idiopathic multicentric Castleman disease (iMCD) is a clinical challenge with no standard treatment. In this preliminary clinical trial, we investigated the efficacy and safety profiles of a Bruton tyrosine kinase inhibitor (BTKi), zanubrutinib, in patients with R/R iMCD. The primary endpoint was the overall response rate at Week 12 according to the Castleman Disease Collaborative Network (CDCN) response criteria.

Idiopathic multicentric Castleman disease (iMCD)-idiopathic plasmacytic lymphadenopathy: A distinct subtype of iMCD-not otherwise specified with different clinical features and better survival.

Idiopathic multicentric Castleman disease (iMCD) is subclassified into iMCD-thrombocytopenia, anasarca, reticulin fibrosis, renal dysfunction, organomegaly (TAFRO) and iMCD-not otherwise specified (NOS) according to the Castleman Disease Collaborative Network (CDCN) consensus criteria. With a deeper understanding of iMCD, a group of patients with iMCD-NOS characterised by polyclonal hypergammaglobulinaemia, plasmacytic/mixed-type lymph node histopathology and thrombocytosis has attracted attention. This group of patients has been previously described as having idiopathic plasmacytic lymphadenopathy (IPL).

Effects of COVID-19 infection in patients with autoimmune pulmonary alveolar proteinosis: a single-center study.

Background: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare interstitial lung disease. COVID-19 is associated with worse prognosis in previous lung diseases patients. But the prognosis of aPAP patients after infection with COVID-19 is unclear.

Evolution of Pulmonary Involvement in Idiopathic Multicentric Castleman Disease-Not Otherwise Specified: From Nodules to Cysts or Consolidation.

Background: Previous studies about multicentric Castleman disease-associated pulmonary manifestations have been limited by small cohorts and not following the Castleman Disease Collaborative Network classification criteria of multicentric Castleman disease. The pulmonary manifestations in idiopathic multicentric Castleman disease-not otherwise specified (iMCD-NOS), a distinct clinical phenotype in the classification criteria, have not been reported.

Research Question: Which pulmonary abnormalities in iMCD-NOS are advanced manifestations and which are reversible after effective treatment?

Study Design And Methods: Patients diagnosed with iMCD-NOS with pulmonary involvement were enrolled.

A prospective, multicenter study of bortezomib, cyclophosphamide, and dexamethasone in relapsed/refractory iMCD.

Relapsed and refractory (R/R) idiopathic Multicentric Castleman disease (iMCD) is a clinical challenge with few treatment options. In this first multicenter, prospective trial which implemented the recently published CDCN response criteria, we evaluated the efficacy and safety profiles of bortezomib-cyclophosphamide-dexamethasone (BCD) regimen in 24 R/R iMCD patients. By 6 months, 15 patients (62.

UCD with MCD-like inflammatory state: surgical excision is highly effective.

Unicentric Castleman disease (UCD) is a rare lymphoproliferative disorder presenting as a single nodal mass with characteristic histopathology. Patients with UCD are typically asymptomatic with normal laboratory markers, whereas patients with multicentric Castleman disease (MCD) demonstrate multicentric lymphadenopathy and cytokine storm-induced systemic inflammatory symptoms. This retrospective analysis of 116 UCD cases identified 19 (16.

Diagnostic Utility of Interferon-Gamma Release Assay in Tuberculous Lymphadenitis.

The aim of this study was to evaluate the diagnostic performance of T-SPOT.TB for tuberculous lymphadenitis. Suspected tuberculous lymphadenitis patients between September 2010 and September 2018 who had both peripheral blood T-SPOT.