TEMPI syndrome: difficult to diagnose, "easy" to treat?

TEMPI syndrome is a rare, acquired disorder with multisystemic manifestations. It is classified as a plasma cell disorder and is characterized by telangiectasias, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunt. Even though TEMPI's pathophysiology remains elusive, it responds to anti-myeloma therapy indicating that the monoclonal protein or clone plays a key role. We present a challenging case of a 73-year-old man with erythrocytosis and deteriorating renal function with nephrotic-range proteinuria in whom after extensive work up, the diagnosis of TEMPI syndrome was made. He was received treatment with daratumumab-bortezomib-cyclophosphamide and dexamethasone (Dara-VCD) and achieved a hematological and clinical response. We also report preliminary data on a multiplex assay for cytokines and growth factors for two patients with TEMPI syndrome and note lower levels for non-specific innate immunity related cytokines. A direct link between renal impairment and TEMPI syndrome is not currently established; cytokine deregulation could potentially be involved in the ischemic changes observed in the renal biopsy of our patient.

Long-term Survival in a Patient With Transformation of Waldenström's Macroglobulinemia into DLBCL.

Background/Aim: Waldenström's macroglobulinemia (WM) is a rare slow-growing B-cell lymphoma that is characterized by lymphoplasmacytic bone marrow infiltration and the production of monoclonal immunoglobulin M (IgM) paraprotein. In 5-10% of patients, WM undergoes transformation into diffuse large B-cell lymphoma (DLBCL), which is more aggressive, with poor prognosis and a low survival rate. Case Report: Α 69-year-old woman was diagnosed with WM in 2009. She received six cycles of chemoimmunotherapy and a remarkable remission was achieved. However, in 2013 the disease transformed into DLBCL. The patient received chemotherapy and after the completion of the first cycle of therapy, the disease was significantly minimized. At the end of the therapy, there was no evidence of disease, and the patient remains disease-free. The cytogenetic profile of the patient did not reveal expression of BCL2 apoptosis regulator, BCL6 transcription repressor, Epstein-Barr virus small RNA, syndecan 1 nor cyclin D1. According to a staging system based on the platelet count, lactate dehydrogenase and previous treatment for WM, the described patient was classified as being at intermediate risk with an expected 2-year survival probability of 47% after WM transformation into DLBCL. However, the patient unexpectedly exceeded these prognostic indications. Conclusion: The findings for this patient are of great interest compared with the existing literature which suggests that the survival and prognosis for patients with transformed DLBCL are not favorable.

Anatomical Variations in the Celiac Trunk: A Short Review.

OBJECTIVE: The objective of the current study was the examination of possible variants of the celiac trunk. METHODS: An advanced review of the literature search was undertaken by means of the PubMed database and Google Scholar, searching for new studies published up to October 2022. Additional articles provided useful information in relation to the aim of this review. Hence, articles that met the inclusion criteria were included in this review and the collected data were organized into a table. RESULTS: The search of the literature retrieved 10 articles that referred to the anatomical variations of the celiac trunk. According to the available literature, the most common anatomical variations are: hepatosplenic trunk where the left gastric artery originates from the abdominal aorta, hepatosplenic trunk, where the left gastric artery originates from the splenic artery, and hepatogastric trunk and splenic artery origin from the superior mesenteric artery. Many other anatomical variations of the celiac trunk may exist, such as tetrafurcation, pentafurcation and hexafurcation, that refer to the division of the celiac trunk into four, five or six branches, respectively, and should be reported as they can affect surgical approaches and the development of the appropriate treatment strategy in patients. CONCLUSION: Every visceral surgeon, interventional radiologist and abdominal imager should be familiar with these variants.

Real World Efficacy and Toxicity of Selinexor: Importance of Patient Characteristics, Dose Intensity and Post Progression Outcomes.

BACKGOUND: Selinexor is an orally available selective inhibitor of exportin-1 that has offered a new treatment option in relapsed or refractory myeloma (RRMM) either in combination with dexamethasone (Sd) or with bortezomib and dexamethasone (SVd). PATIENTS-METHODS: We evaluated the efficacy and toxicity of selinexor combinations in the real world, post progression therapies and their outcomes. The analysis included 44 patients with RRMM treated with Sd (N = 21, 48%) or SVd (N = 23, 52%). RESULTS: On intent-to-treat, response rate (ORR) among all treated patients was 29.5% (13/44, of which CR: 2, VGPR: 3, PR:8); ORR was 35% for SVd and 24% for Sd. Median PFS was 3.0 months for all; 6.9 months for responders (≥PR),2.7 months for Sd and 3.4 months for SVd treated patients. In univariate analysis, serum albumin <3.5 g/dl and LDH >ULN were associated with worse PFS (P = .001 and P = .032, respectively).The OS of the whole cohort exceeded one year while serum albumin <3.5 gr/dl and LDH>ULN were associated with worse OS. After progression to Sd/SVd, 20 patients received further therapy; on ITT, the ORR was 40% (8/20) and the subsequent PFS was 3.4 months. The most common adverse events were fatigue, thrombocytopenia and nausea, while the most recorded grade 3 or 4 side effect was thrombocytopenia; 56% (25/44) of patients required dose reduction, however, this was not associated with inferior PFS. CONCLUSION: In conclusion, selinexor-based therapy provides an additional treatment option in the real word setting and with appropriate dosing and toxicity management a subset of patients may have significant benefit.

The Coronoid Anatomical Variation of the Masseter Muscle: A Case Report.

INTRODUCTION: Masseter muscle is considered to consist of two layers: the superficial and the deep parts. However, in the literature a few cases have been reported of a third layer of the masseter muscle, which is called the coronoid part. The aim of this study is to present such a rare case of the coronoid part of the masseter muscle. MATERIAL AND METHODS: In our study, a case report is presented and enhanced by searching the existing literature in PubMed for other similar cases that recorded the coronoid part of the masseter muscle. RESULTS: The coronoid part of the masseter muscle was found in a male cadaver of Greek origin that was dissected in the Department of Anatomy, Medical School Athens, Greece. CONCLUSIONS: Good knowledge of anatomy and variations of the masseter muscle are of paramount importance due to their clinical and functional applications as well as during surgical procedures in this anatomical region.

Proteasome Inhibitor-Based Regimens in the Frontline Management of Waldenström Macroglobulinemia.

Proteasome inhibitors (PIs) have long been used in myeloma therapy but also for Waldenström macroglobulinemia. Their use has been successful and has also been investigated for the frontline management of the disease. Bortezomib was effective either as a single agent or in combination with other regimens with high response rates observed in most studies, despite its adverse effects, especially neurotoxicity, which remains a major concern. Clinical trials with second-generation PIs such as carfilzomib and ixazomib have also been conducted, always in combination with immunotherapy in previously untreated patients. They have been shown to be active and neuropathy-sparing treatment options.

Duplicated inferior vena cava-trifurcated portal vein: a rare anatomical variation encountered during Whipple procedure.

The inferior vena cava (IVC) is the largest single vein in humans. However, during embryogenesis, abnormalities can occur resulting in a duplicated IVC. The portal vein (PV) offers the main blood flood to the liver, forming by the left and right PV. A number of anatomical variations are noticed, underlying the great importance of the pre-operative imaging workup. This case report presents a duplicated IVC and a trifucated PV that were incidentally found in an 82 year-old Caucasian male with pancreatic ductal adenocarcinoma who underwent pancreatoduodenectomy (Whipple procedure). Although some anatomical variations, including the duplication of the IVC and the trifurcation of PV, may be rare to the general population, the suspicion of their existence should always be taken under consideration from surgeons during hepatobiliary or retroperitoneal operations.

Variations of Ulnar Nerve in the Forearm and Hand: A Review of the Literature and Clinical Significance.

The ulnar nerve originates from the anterior division of the lower trunk of the brachial plexus which continues as the medial cord and gives off branches to the forearm and the hand with motor and sensory fibers. The objective of the current study is to assemble the recognized anatomical variations of the ulnar nerve (UN) and underline their clinical impact. A literature search was undertaken via PubMed database, using the term: "ulnar nerve AND variations." Classical anatomical textbooks were also used for the normal anatomy of the UN. A total of 23 articles met the inclusion criteria, 16 of which are included in this review. Fifty-four additional articles provided useful information according to the aim of this review. Of great interest is the communication between the UN and the radial nerve (RN) in the forearm, as well as the communication between the UN and the median nerve (MN) in both forearm and hand. Furthermore, variations of the UN were observed in the hand and the classification of the UN compression was also described according to the point of the neuropathy. These collecting data are categorized into five tables. Additional aberrations were also included in this review. According to literature, the UN is characterized by numerous variations of its course and branches. Therefore, good knowledge of the normal anatomy is essential. Moreover, the anomalies are of particular importance due to their significant clinical implications and should be taken into consideration by the surgeons during surgical procedures in this region.

Anatomical Variations of Vascular Anatomy in Meckel's Diverticulum.

OBJECTIVE: The objective of the current study was to describe the anatomical variations of vessels observed in patients with Meckel's Diverticulum. METHODS: A narrative review of the literature was undertaken by means of the PubMed database, using the terms: "Meckel's Diverticulum AND vessels", "Meckel's Diverticulum AND anatomical variation" and "Meckel's Diverticulum variation". Classical anatomical textbooks were also used for normal anatomy. Additional articles provided useful information in relation to the aim of this review. Hence, the articles that met the inclusion criteria were included in this review, and the collected data were categorized into a single table. RESULTS: The majority of studies indicated the presence of an abnormal vitelline artery. Other angiographic findings concerned variations of the ileal and the iliac arteries. However, the literature revealed the presence of vascular variations without the existence of Meckel's Diverticulum, whereas a remnant of the vitelline vein may be present, but it is very rare. CONCLUSION: The detection of vascular variations accompanying Meckel's Diverticulum is not always easy and requires the correct choice of imaging method to prevent misdiagnosis.

The Role of Medical Students During COVID-19 Era. A Review.

BACKGROUND AND AIM OF THE WORK: Since December 2019, the world has been facing the coronavirus (COVID-19) pandemic crisis. The rapid and effortless spread of the virus has led the World Health Organization to adopt unprecedented large-scale preventive measures. Our aim is to investigate the impact of COVID-19 on the mental health of medical students, and to evaluate their awareness and aptitude for contributing to the fight against COVID-19 contagion. METHODS: PubMed electronic database was employed for the research using the following terms: "medical students and COVID-19", "mental health of medical students and COVID-19", "knowledge of medical students and COVID-19", "preventive behavior of medical students and COVID-19". RESULTS: Twenty-one articles were included; ten assess the knowledge of medical students on COVID-19, seven present whether they demonstrate appropriate preventive behavior according to global guidelines, seven examine the impact of the pandemic on their mental health, five examine their role on the side of trained healthcare workers. Some articles combine more than one of the above mentioned domains. CONCLUSIONS: Medical students seem to demonstrate an adequate level of knowledge upon COVID-19 and implement proper strategies to prevent its spread. Further training is essential to improve some aspects in this field. Social media has proven to be the optimal source of information for medical students, which is incompatible with their future careers as doctors. The pandemic had a negative impact on medical students, expressed as fear regarding the virus spread; nevertheless, many have felt the need to assist healthcare systems under appropriate precautions.

Three-Dimensional (3D) Printing in Orthopedics Education.

Three-dimensional (3D) printing is a newly established concept in orthopedics compared with other industries. Surgical applications of 3D printing and tissue engineering have been investigated since the early 2000s, almost two decades after Charles Hull had patented the first device currently in use for additive manufacturing, also known as rapid prototyping or more commonly 3D printing, and whose initial formal appellation was stereolithography (SLA). Despite technological progress, substantial principles have largely remained unaltered. Training directly on patients and on cadavers is considered the "gold standard" for learning and developing suitable surgical qualifications. However, restrictions concerning patient safety, ethical dilemmas, lack of availability, etc., have to be taken into account. Thus, 3D representations can be utilized as an educational tool both for patients to improve their understanding of their condition and also medical students, residents, and surgeons to comprehend complex anatomical structures and practice their surgical maneuvers to be prepared and more confident in theater.

The Role of Surgical Masks during the COVID-19 Pandemic. A Mini-Review.

Introduction - The coronavirus disease 2019 (COVID-19) is considered to be highly contagious and restriction of transmission requires the utilization of protective equipment like surgical masks from both healthcare workers and public. The aim of this review is to investigate the role of surgical masks during the COVID-19 pandemic. Materials and Methods - A literature research was conducted via PubMed to detect articles featuring the potential protective role of surgical masks when they were worn by healthcare workers or by the general public. Results - Among 114 articles, only 31 met the inclusion criteria. Thirteen additional articles provided useful information according to the aim of this review. Existing literature supports the positive effect of surgical masks on COVID-19 con-lamination. Debate on the efficacy of surgical masks compared with other forms of facial protective devices exists. There seems to be a tendency to favor the use of particulate-filtering facepiece respirators in healthcare professionals who face higher risk of infection. However, surgical masks primarily and cloth masks secondarily seem to be adequate means of protection for the general public and for healthcare workers during procedures where respiratory droplets are not generated. Rational use of surgical masks is imperative; however, reuse after decontamination is not ideal. Conclusions - For optimum protection from COVID-19, the use of surgical masks should be combined with other infection control measures like hand hygiene and social distancing, since the level of their effectiveness is still being investigated. Shortage of surgical masks should be prevented; therefore, rational use plays a crucial role in this direction.