Overuse of organ biopsies in immunoglobulin light chain amyloidosis (AL): the consequence of failure of early recognition

Introduction: The diagnosis of amyloidosis requires histological confirmation of Congo-red (CR) deposits. The tissue source is preferably fat aspiration and/or bone marrow (BM) biopsy, but at times organ biopsy is required.

Methods: We studied 612 patients with systemic immunoglobulin light chain amyloidosis to characterise the tissues used to establish the diagnosis.

Results: The median number of tissue samples was 3. About 95% of BM biopsies were stained for CR, while 79% of patients had fat aspiration CR-stained. CR stain sensitivity was 69% in BM, 75% in fat aspiration and 89% for both sources combined. In comparison, CR sensitivity was 97–100% for heart, renal and liver biopsies. About 42% of patients with renal involvement, 21% of patients with liver involvement and 13% of patients with heart involvement underwent organ biopsy, when a less invasive biopsy would have established the diagnosis. Predictors for the requirement for organ biopsy were male sex, limited organ involvement and lack of fat aspiration.

Discussion: Fat aspiration is underutilised for histologic confirmation of amyloidosis. A high rate of organ biopsies represents a failure to recognise the disease. Early awareness of amyloidosis in patients with organ dysfunction may lead to more judicious use of organ biopsies in this disease.

• Key messages

• Fat pad aspiration is underutilised to establish the diagnosis of amyloidosis.

• Bone marrow and fat pad aspiration obviates the need for invasive biopsies.

• The excessive use of organ biopsy in AL amyloidosis reflects failure to recognise the disease early in its course.

     

Role of bronchoscopy in early diagnosis of lung cancer

Lung cancer is a leading cause of cancer deaths and the incidence is rising. Most patients with lung cancer present to the clinician in a fairly advanced stage and at best only 25-30% of patients can be offered curative resection. Screening tests using sputum cytology and chest radiograph have been used with limited success. Value of low dose spiral CT scan as screening tool for lung cancer is being evaluated and its limitations include high costs, need for repeated scanning and necessity to obtain histological confirmation with additional procedures. There have been significant advances in the early diagnosis of lung cancer in high risk patient groups using bronchoscopic methods such as white light bronchoscopy, autofluorescence bronchoscopy, high magnification bronchoscopy, narrow band imaging and endobronchial ultrasound. These techniques appear to be promising tools as they might allow to visualise changes of early lung cancer and also permit sampling for histological confirmation.     

Management of adenovirus infection in patients after haematopoietic stem cell transplantation: State‐of‐the‐art and real‐life current approach

The important insights gained over the past years in diagnosis and treatment of invasive adenoviral infections provide new paradigms for the monitoring and clinical management of these life‐threatening complications. A meeting was held to discuss and subsequently disseminate the current advances in our understanding of the aetiology/pathogenesis and future treatment options facilitating effective control or prevention of adenovirus‐related diseases in the allogeneic haematopoietic stem cell transplant setting. Invited experts in the field discussed recent progress with leading members of the Infectious Diseases Working Party of the European Society of Blood and Marrow Transplantation at the “State‐of‐the‐art” Meeting in Poznan, Poland, in October 2017. In this review article, the panel of experts presents a concise summary of the current evidence based on published data from the last 15 years and on recent achievements resulting from real‐life practice. The present position statement reflects an expert opinion on current approaches to clinical management of adenovirus infections in patients undergoing allogeneic haematopoietic stem cell transplant and provides graded recommendations of the panel for diagnostic approaches and preemptive therapy reflecting the present state of knowledge.     

High throughput tissue microarray analysis of FHIT expression in diffuse large cell B-cell lymphoma from Saudi Arabia.

Recent studies have suggested a potential prognostic role of alterations of the fragile histidine triad (FHIT) gene in diffuse large B-cell lymphoma. To evaluate possible mechanisms of FHIT inactivation and to further clarify its potential prognostic relevance, we analyzed a set of 114 diffuse large B-cell lymphoma with clinical follow-up information. Tissue microarrays were analyzed by immunohistochemistry for protein expression, and corresponding DNA samples were analyzed for FHIT promotor hypermethlyation. Reduced or absent FHIT expression was found in 75 of 114 diffuse large B-cell lymphoma (66%), but was unrelated to clinical tumor stage or patient prognosis. FHIT promotor hypermethylation was observed in 29 of 93 (23%) interpretable diffuse large B-cell lymphoma. Hypermethylation was not significantly correlated to protein expression loss, which could be explained by competing mechanisms for FHIT inactivation in a substantial fraction of non FHIT hypermethylated diffuse large B-cell lymphoma. Hypermethylation was significantly associated with poor prognosis of diffuse large B-cell lymphoma patients and predominantly seen in nongerminal center diffuse large B-cell lymphoma (27%), but less frequent (13%) in germinal center diffuse large B-cell lymphoma. In summary, these data suggest that promotor hypermethylation is responsible for reduced FHIT expression in a substantial subset of diffuse large B-cell lymphoma, which is primarily composed of nongerminal center subtype with poor patient prognosis.     

Prevalence of chronic obstructive pulmonary disease and chronic bronchitis in eight countries: a systematic review and meta-analysis

ObjectiveTo estimate the prevalence of chronic obstructive pulmonary disease (COPD) and chronic bronchitis in eight countries in South Asia through a systematic review and meta-analysis.MethodsWe searched MEDLINE® Complete, Web of Science, Embase®, Scopus, CINAHL and reference lists of screened studies for research on the prevalence of COPD and chronic bronchitis in South Asian countries published between January 1990 and February 2021. We used standardized diagnostic criteria for definitions of COPD and chronic bronchitis. Two reviewers undertook study screening, full-text review, quality appraisal and data extraction.FindingsOf 1529 studies retrieved, 43 met the inclusion criteria: 32 provided data from India; four from Bangladesh; three from Nepal; two from Pakistan; and two from both India and Sri Lanka. Twenty-six studies used standardized diagnostic definitions and 19 were included in the meta-analysis. The estimated pooled prevalence of COPD was 11.1% (95% confidence interval, CI: 7.4–14.8%), using the Global Initiative for Chronic Obstructive Lung Disease fixed criteria and 8.0% (95% CI: 5.6–10.4%) using the lower limit of normal criteria. The prevalence of COPD was highest in north India (19.4%) and Bangladesh (13.5%) and in men. The estimated pooled prevalence of chronic bronchitis was 5.0% (95% CI: 4.1–6.0%) in India and 3.6% (95% CI: 3.1–4.0%) in Pakistan.ConclusionIncluded countries have a high prevalence of COPD although it varied by geographical area and study characteristics. Future research in South Asia should use standardized diagnostic criteria to examine the contribution of setting-specific risk factors to inform prevention and control strategies.     

A Novel Obturator Device for Management of Dilated Trachea-esophageal Puncture Tract Fistulas

Voice rehabilitation in laryngectomized patients by tracheoesophageal puncture is a time tested technique. In some patients the tracheoesophageal puncture gets inordinately dilated leading to leakage around the prosthesis. Most of these fistulas are managed by a variety of conservative treatments like temporary removal of prosthesis, placement of silastic ring over the prosthesis, placement of nasogastric tube and airway protection by a cuffed tracheostomy tube. Intractable fistulas are tackled by surgical closure but are fraught with failures. We hereby suggest a novel temporary obturator that can be can be easily made at a very low cost in any hospital having prosthetic rehabilitation services and obviates the need for a tracheostomy tube, nasogastric tube and repeated hospital visits.     

Preoperative Factors Predicting Remission of Type 2 Diabetes Mellitus After Roux-en-Y Gastric Bypass Surgery for Obesity

Background  

Roux-en-Y gastric bypass (RYGB) is an effective treatment for morbid obesity. This bariatric procedure has also been noted to resolve hyperglycaemia in up to 70% of obese diabetics. We evaluated outcomes in diabetic patients undergoing RYGB in our institution, aiming to identify factors predicting diabetes remission.     

Neonatal Purpura Fulminans Due to Methicillin Resistant Staphylococcus aureus

Abstract: Neonatal purpura fulminans is rare and may be inherited or acquired. It may ultimately lead to multiorgan failure and death. Purpura fulminans in a premature neonate resulting from Staphylococcus aureus septicemia is illustrated. Unfortunately, the baby succumbed to septicemia.