NCCN Guidelines® Insights: Myelodysplastic Syndromes, Version 3.2022.

The NCCN Guidelines for Myelodysplastic Syndromes (MDS) provide recommendations for the evaluation, diagnosis, and management of patients with MDS based on a review of clinical evidence that has led to important advances in treatment or has yielded new information on biologic factors that may have prognostic significance in MDS. The multidisciplinary panel of MDS experts meets on an annual basis to update the recommendations. These NCCN Guidelines Insights focus on some of the updates for the 2022 version of the NCCN Guidelines, which include treatment recommendations both for lower-risk and higher-risk MDS, emerging therapies, supportive care recommendations, and genetic familial high-risk assessment for hereditary myeloid malignancy predisposition syndromes.

Stereoselective Synthesis of the C1-C16 Fragment of the Purported Structure of Formosalide B.

The first stereoselective synthesis of the C1-C16 fragment possessing stereo-enriched fully substituted tetrahydropyran (THP) along with tetrahydrofuran (THF) rings of the proposed structure of formosalide B is described in 12 longest linear steps with 22% overall yield, starting from two cheap and commercially available 1,5-pentanediol and l-glutamic acid, following a convergent approach. The key steps involve in this synthesis are Horner-Wadsworth-Emmons reaction, Sharpless asymmetric dihydroxylation, and acid-mediated ketalization to assemble the substituted THP ring, one-pot Sharpless dihydroxylation-S2-type cyclization, and Wittig homologation to construct the THF derivative.

Cognition, autonomic function, and intellectual outcomes of the paramedical health-care personnel in the hospital settings.

Background: In the dedicated intensive care settings, health-care providers need to have higher temporal cognition and sympathovagal balance to optimally deliver critical care interventions.

Objective: The objective of the study was to estimate the parameters of the temporal cognition and autonomic function of paramedical staffs in acute health-care settings.

Materials And Methods: In this study on 81 healthy adult paramedical personnel, temporal cognition was assessed using auditory reaction time (ART), visual reaction time (VRT), critical flicker fusion frequency (CFFF), Stroop test (ST), and digits forward test (DFT); Autonomic functions were assessed by heart rate (HR) and blood pressure (BP) variability, and all these outcomes were analyzed with their academic performance.

The Outcome Analysis and Complication Rates of Tracheostomy Tube Insertion in Critically Ill Neurosurgical Patients; A Data Mining Study.

Objectives: To assess the impact, timing, the intra and early post-operative complications and the survival outcome of tracheostomy in critically ill neurosurgery patients.

Methods: This study was a retrospective data mining where data was collected from hospital records from 175 consecutive patients who underwent tracheostomy in the department of Neurosurgery at the Narayna Medical College Hospital, Nellore, India from Jan 2016 to April 2018. A proforma was used to note down the details on the patient status before and after tracheostomy: Glasgow coma scale (GCS), procedure and intra and post-operative complications, type of tracheostomy cannula, details of decannulation, respiration difficulties, and problems with wound, swallowing difficulties, and voice difficulties, stay in intensive care unit (ICU) and hospital and survival status of the patient.

Wilson Disease Presenting With Acute on Chronic Liver Failure: A Single-Center Experience of Outcome and Predictors of Mortality in 68 Patients.

Background And Aims: Acute on chronic liver failure (ACLF) is an emerging entity whose unique pathogenesis, presentation, and outcome are different from those with decompensated cirrhosis. Patients with Wilson disease (WD) often present with ACLF. The outcome in this setting and predictors of mortality have not been well delineated.

Clinical presentation and outcome after anterior cervical discectomy and fusion for degenerative cervical disc disease.

Background: Anterior cervical discectomy and fusion (ACDF) is a well-described surgical approach for symptomatic degenerative cervical disc disease which does not respond to conservative management. In the present study, we assessed clinical presentation and outcomes of ACDF.

Materials And Methods: The present study was conducted from October 1, 2015, to October 31, 2017, in the Department of Neurosurgery, Narayana Medical College and Hospital, Nellore, Andhra Pradesh, among 100 consecutive adult patients who underwent single- or two-level ACDF for degenerative cervical disc disease.

Arthritis in sarcoidosis: A multicentric study from India.

Introduction: Ten to 15% of patients with sarcoidosis have associated arthritis. Chronic arthritis is fairly uncommon. There is a paucity of data on articular manifestations of the disease from India.

Drug-induced acute liver failure in children and adults: Results of a single-centre study of 128 patients.

Background & Aims: Drugs producing acute liver failure (ALF) are uncommon and vary geographically. Here we review the implicated drugs, clinical features, laboratory characteristics and outcome of patients with drug-induced ALF (DIALF). We analysed the predictors of mortality and their relationship with MELD, King's College criteria (KCC) and ALFSG prognostic index.

Pilot phase II study of metronomic chemotherapy in combination with bevacizumab in patients with advanced non-squamous non-small cell lung cancer.

Introduction: The goal of this study was to explore the efficacy and tolerability of metronomic chemotherapy, a novel anti-angiogenic treatment strategy, in combination with bevacizumab in patients with advanced non-small cell lung cancer (NSCLC).

Methods: Subjects with newly diagnosed stage IV NSCLC were treated with 4-week cycles of paclitaxel 80mg/m and gemcitabine 300mg/m weekly for three weeks, plus bevacizumab 10mg/kg every two weeks. Radiologic assessments were performed every 8 weeks.

NCCN Guidelines Insights: Chronic Myeloid Leukemia, Version 1.2017.

The NCCN Guidelines for Chronic Myeloid Leukemia (CML) provide recommendations for the management of chronic-phase and advanced-phase CML in adult patients. The median age of disease onset is 67 years. However, because CML occurs in all age groups, clinical care teams should be prepared to address issues relating to fertility and pregnancy with patients who are of reproductive age at the time of diagnosis.

Fat Embolism Syndrome Secondary to Bone Marrow Necrosis in Patients with Hemoglobinopathies.

Bone marrow necrosis with subsequent embolization of the fat and necrotic tissues into the systemic circulation causing fat embolism syndrome and multiorgan failure is a rare complication of patients with hemoglobinopathies. The exact etiology of this condition is not known. Because it occurs more often in patients with compound heterozygous conditions than in sickle cell disease, some patients are unaware of their predisposition.

Is Low Positive JAK2 V617F Mutation Test Result Clinically Significant?: Multi-Institutional Study.

Objectives: Acquired somatic mutation Janus kinase 2 (JAK2) V617F is associated with various myeloproliferative neoplasms (MPN). Allele-specific real-time polymerase chain reaction has been widely adopted to detect mutation; however, the utility of low positive results is not well understood. The aim of this study is to investigate the clinical significance of low positivity of JAK2 V617F.

Chronic myelogenous leukemia, version 1.2015.

Chronic myelogenous leukemia (CML) is usually diagnosed in the chronic phase. Untreated chronic phase CML will eventually progress to advanced phase (accelerated or blast phase) CML. Tyrosine kinase inhibitors (TKIs) have been shown to induce favorable response rates in patients with accelerated and blast phase CML.

Trisomy 21-associated transient abnormal myelopoiesis involving the maternal space of the placenta: a case report and literature review.

We report a case of a male newborn with trisomy 21 and transient abnormal myelopoiesis at birth whose placenta showed extravasated fetal blasts in the perivillous (maternal) space. Concern for possible maternal spread of fetal malignancy prompted a Kleihauer-Betke test and flow cytometric analysis of the maternal peripheral blood on postpartum day 2. Notably, no evidence of the persistence of fetal cells in the maternal blood was identified, a finding that likely reflected successful maternal immunologic clearance of the fetal blasts and erythrocytes, and/or blast cellular fragility and limited viability.

Out with the bad and in with the good; red cell exchange, white cell reduction, and platelet reduction.

Automated techniques for red cell [red blood cell (RBC)] exchange or depletion of malignant cells from the peripheral blood have allowed patients with life-threatening conditions to survive long enough to receive definitive treatment. Examples of such conditions include acute chest syndrome in sickle cell disease (SCD) or acute respiratory insufficiency due to leukostasis in acute leukemia. Conversely, other patients with SCD undergo RBC exchanges on a chronic basis to maintain a reasonable quality of life and prevent another stroke.

Soluble CD25 in serum: a potential marker for subclinical macrophage activation syndrome in patients with active systemic onset juvenile idiopathic arthritis.

Objective: Laboratory and immunological abnormalities seen in overt macrophage activation syndrome (MAS) may be observed in patients with untreated new onset systemic onset juvenile idiopathic arthritis (SoJIA). We investigated the prevalence of clinical and traditional laboratory markers of MAS as well as soluble CD163 and soluble interleukin (IL)-2Rα (CD25) in active SoJIA patients.

Methods: Thirty-three consecutive patients with active SoJIA (International League of Associations for Rheumatology criteria), 11 patients with active polyarticular JIA (polyJIA) (disease control) and two patients with MAS with SoJIA were included in the study.

Chronic Myelogenous Leukemia, Version 1.2014.

The 2014 NCCN Clinical Practice Guidelines in Oncology for Chronic Myelogenous Leukemia recommend quantitative reverse-transcription polymerase chain reaction (QPCR) standardized to International Scale (IS) as the preferred method for monitoring molecular response to tyrosine kinase inhibitor (TKI) therapy. A BCR-ABL1 transcript level of 10% or less (IS) is now included as the response milestone at 3 and 6 months. Change of therapy to an alternate TKI is recommended for patients with BCR-ABL1 transcript levels greater than 10% (IS) at 3 months after primary treatment with imatinib.

Cardiac device-related invasive aspergilloma assessed by live/real time three-dimensional transthoracic echocardiography.

Cardiac device-related infection caused by Aspergillus species is a rare finding associated with high mortality. Prompt recognition and treatment is imperative, but difficult as blood cultures are often negative and diagnosis requires a high index of suspicion. Live/real time three-dimensional transthoracic echocardiography (3DTTE) provides incremental knowledge in the characterization of valvular vegetations.

Endoscopic ultrasound and endobronchial ultrasound-guided fine-needle aspiration of deep-seated lymphadenopathy: Analysis of 1338 cases.

Background: We retrospectively studied 1338 samples of lymph nodes obtained by endoscopic and endobronchial ultrasound-guided fine needle aspiration biopsy (EUS and EBUS-FNAB) with an objective of characterizing the utility of this diagnostic modality in the assessment of deep-seated lymphadenopathy. The secondary aims were to establish the utility in the diagnosis of lymphoma and to determine the number of passes required to obtain adequate cellularity for flow cytometric analysis.

Materials And Methods: On-site assessment was performed by a cytopathologist using Diff-Quik (American Scientific Products, McGraw Park, IL) stain.

CD138 (syndecan-1) expression in bone-forming tumors.

CD138 (syndecan-1), a cell surface proteoglycan, is sensitive and specific for plasmacytic differentiation in hematologic disorders. Expression of CD138 has been observed in a majority of epithelial neoplasms and, rarely, soft tissue tumors. However, its expression in bone tumors has not been evaluated.

Extramedullary hematopoiesis: an unusual finding in subdural hematomas.

We present a case of a 59-year-old man who was found to have clusters of hyperchromatic, small, round nucleated cells within a subdural hematoma removed after a skull fracture. Immunohistochemistry study confirmed that the cells were hematopoietic components predominantly composed of normoblasts. In this paper, we describe the clinical and pathological findings.

Intranasal eosinophilic angiocentric fibrosis: a case report and review.

Eosinophilc angiocentric fibrosis is a rare fibrosing vasculitis of unknown etiology that is progressive and potentially disfiguring. It has a predilection for the upper airways presenting most commonly as an obstructing mass lesion that is diagnosed histologically. Thus far, it has been nonfatal in the more than 40 reported cases; however, subglottic and ocular involvement has resulted in significant morbidity in several patients.