scholarly journals How I diagnose and treat splenic lymphomas

Blood ◽  
2011 ◽  
Vol 117 (9) ◽  
pp. 2585-2595 ◽  
Author(s):  
Emilio Iannitto ◽  
Claudio Tripodo

Abstract The incidental finding of an isolated splenomegaly during clinical assessment of patients evaluated for unrelated causes has become increasingly frequent because of the widespread use of imaging. Therefore, the challenging approach to the differential diagnosis of spleen disorders has emerged as a rather common issue of clinical practice. A true diagnostic dilemma hides in distinguishing pathologic conditions primarily involving the spleen from those in which splenomegaly presents as an epiphenomenon of hepatic or systemic diseases. Among the causes of isolated splenomegaly, lymphoid malignancies account for a relevant, yet probably underestimated, number of cases. Splenic lymphomas constitute a wide and heterogeneous array of diseases, whose clinical behavior spans from indolent to highly aggressive. Such a clinical heterogeneity is paralleled by the high degree of biologic variation in the lymphoid populations from which they originate. Nevertheless, the presenting clinical, laboratory, and pathologic features of these diseases often display significant overlaps. In this manuscript, we present our approach to the diagnosis and treatment of these rare lymphomas, whose complexity has been so far determined by the lack of prospectively validated prognostic systems, treatment strategies, and response criteria.

2019 ◽  
Vol 2019 ◽  
pp. 1-7
Author(s):  
Nivedita U. Jerath

Valosin containing protein (VCP) mutations have been reported to present with a high degree of variability and can be present in patients even if they may have an initial normal work up. A 55-year-old woman was labeled as “normal” and “pain medication seeking” after an unrevealing work up of clinical, laboratory, electrodiagnostic, radiographic, pathologic, and genetic testing. She continued to present with chronic neck pain, and had variable features of scapuloperoneal atrophy, which was also seen in her family. The patient and her family were found to have a known pathogenic c.464G>A, p.Arg155His (R155H) mutation in the VCP gene. Despite traditional thinking of attempting to localize neurological syndromes, VCP mutations are difficult to localize as they can present with significant clinical heterogeneity including a scapuloperoneal syndrome with variable neuropathic and myopathic features.


2021 ◽  
Vol 10 (11) ◽  
pp. 2490
Author(s):  
Giulio Francesco Romiti ◽  
Bernadette Corica ◽  
Gregory Y. H. Lip ◽  
Marco Proietti

Background: In patients with COVID-19, cardiovascular complications are common and associated with poor prognosis. Among these, an association between atrial fibrillation (AF) and COVID-19 has been described; however, the extent of this relationship is unclear. The aim of this study is to investigate the epidemiology of AF in COVID-19 patients and its impact on all-cause mortality. Methods: A systematic review and meta-analysis were performed and reported according to PRISMA guidelines, and a protocol for this study was registered on PROSPERO (CRD42021227950). PubMed and EMBASE were systematically searched for relevant studies. A random-effects model was used to estimate pooled odds ratios (OR) and 95% confidence intervals (CI). Results: Overall, 31 studies were included in the analysis, with a total number of 187,716 COVID-19 patients. The prevalence of AF was found to be as high as 8% of patients with COVID-19 (95% CI: 6.3–10.2%, 95% prediction intervals (PI): 2.0–27.1%), with a high degree of heterogeneity between studies; a multiple meta-regression model including geographical location, age, hypertension, and diabetes showed that these factors accounted for more than a third of the heterogeneity. AF COVID-19 patients were less likely to be female but more likely older, hypertensive, and with a critical status than those without AF. Patients with AF showed a significant increase in the risk of all-cause mortality (OR: 3.97, 95% CI: 2.76–5.71), with a high degree of heterogeneity. A sensitivity analysis focusing on new-onset AF showed the consistency of these results. Conclusions: Among COVID-19 patients, AF is found in 8% of patients. AF COVID-19 patients are older, more hypertensive, and more likely to have a critical status. In COVID-19 patients, AF is associated with a 4-fold higher risk of death. Further studies are needed to define the best treatment strategies to improve the prognosis of AF COVID-19 patients.


2014 ◽  
Vol 10 (4) ◽  
pp. 74-77 ◽  
Author(s):  
A Dubey ◽  
S Mujoo ◽  
SB Sakarde ◽  
AK Dubey

Paroxysmal neuralgia is relatively uncommon in children. Neuropathic orofacial pain is a challenge for the clinician, as no obvious dental pathology exists either clinically or radiographically. Dentist and physician should be able to recognize the characteristics of neuropathic pain so as to correctly diagnose these conditions hence avoid unnecessary dental intervention. This article reviews the conditions with paroxysmal neuralgia in children and available treatment strategies. DOI: http://dx.doi.org/10.3126/kumj.v10i4.11000 Kathmandu Univ Med J 2012;10(4):74-77


2018 ◽  
Vol 9 (3) ◽  
pp. 18
Author(s):  
Dayanand Hota ◽  
Kamal Nain Rattan ◽  
Ahmad Khursheed ◽  
Manish Swami ◽  
Harish Bhardwaj

Background: Intramural hematoma of the duodenum is a rare cause of acquired duodenal obstruction. It is often a diagnostic dilemma and a high degree of suspicion is required to make an early and accurate diagnosis in children. Case Report: A 2-year-old child presented with bilious vomiting and abdominal distension. X-ray abdomen showed high intestinal obstruction. Ultrasound and CT scan abdomen gave suspicion of pancreatic pseudo-cyst near head of pancreas. At surgery, an intramural hematoma of the duodenum was found and drained. Conclusion: Intramural duodenal hematoma is a rare entity especially in children and should be considered in differential diagnosis of acquired duodenal obstruction.


1985 ◽  
Vol 31 (5) ◽  
pp. 741-746 ◽  
Author(s):  
D G Deutsch ◽  
R J Bergert

Abstract We evaluated the Hewlett-Packard 5995B benchtop capillary gas chromatograph-mass spectrometer (GC-MS) for its ability to identify drugs commonly detected and (or) measured in the clinical toxicology laboratory. Initial experiments indicated that the instrument as originally configured, with an isolation valve between the gas chromatograph and mass spectrometer, was unsatisfactory for the identification of hypnotics-sedatives. However, with the capillary inserted directly into the ion source, we could detect 10 ng of these drugs on a total-ion chromatogram. The software programs cause the instrument to be highly automated. In terms of ease of operation and speed it was found suitable for use in a routine clinical laboratory. Chromatography of urine extracts on the capillary gas chromatograph-mass spectrometer yielded excellent resolution of parent compounds and metabolites (e.g., diphenhydramine together with approximately four metabolites and propoxyphene with four metabolites). However, the manufacturer's computer program used to evaluate the quality of the match between the experimental mass spectra and the 375 drug reference spectra was only moderately successful in identifying unknown compounds. The ability of this capillary GC-MS to identify most compounds with a high degree of confidence will be increased by enlarging the library to include more drugs and metabolites and by using a more reliable computerized matching program.


Cancers ◽  
2019 ◽  
Vol 11 (8) ◽  
pp. 1169 ◽  
Author(s):  
Sioletic Stefano ◽  
Scambia Giovanni

Soft tissue sarcoma (STS) is a rare malignancy of mesenchymal origin classified into more than 50 different subtypes with distinct clinical and pathologic features. Despite the poor prognosis in the majority of patients, only modest improvements in treatment strategies have been achieved, largely due to the rarity and heterogeneity of these tumors. Therefore, the discovery of new prognostic and predictive biomarkers, together with new therapeutic targets, is of enormous interest. Phosphatase and tensin homolog (PTEN) is a well-known tumor suppressor that commonly loses its function via mutation, deletion, transcriptional silencing, or protein instability, and is frequently downregulated in distinct sarcoma subtypes. The loss of PTEN function has consequent alterations in important pathways implicated in cell proliferation, survival, migration, and genomic stability. PTEN can also interact with other tumor suppressors and oncogenic signaling pathways that have important implications for the pathogenesis in certain STSs. The aim of the present review is to summarize the biological significance of PTEN in STS and its potential role in the development of new therapeutic strategies.


Diagnostics ◽  
2020 ◽  
Vol 10 (2) ◽  
pp. 96
Author(s):  
Alexandra Dadarlat-Pop ◽  
Irina Burian ◽  
Laura Cadis ◽  
Raluca Tomoaia ◽  
Alexandru Oprea

Popliteal venous aneurysms are rare vascular disorders associated with a high risk of pulmonary embolism. We present the case of a 56-year-old woman hospitalized for a third episode of unprovoked pulmonary embolism. Venous ultrasonography identified a popliteal aneurysm, repeatedly missed by two-point compression venous ultrasonography, which was eventually confirmed by a magnetic resonance examination. Because of its highly symptomatic nature despite optimal anticoagulant treatment, the decision was made to undergo surgery, consisting of aneurysmectomy followed by patch angioplasty. The goal of this paper is to report a rare case of popliteal venous aneurysm and its treatment strategies and postoperative evolution.


Author(s):  
Andre Russowsky Brunoni ◽  
Bernardo de Sampaio Pereira Júnior ◽  
Izio Klein

Bipolar disorder is a prevalent condition, with few therapeutic options and a high degree of refractoriness. This justifies the development of novel non-pharmacological treatment strategies, such as the non-invasive techniques of transcranial magnetic stimulation (TMS) and transcranial direct current stimulation (tDCS), as well as the invasive techniques of deep brain stimulation (DBS) and vagus nerve stimulation (VNS). In this chapter, we provide a summary of the development of the techniques as well as the studies carried out with patients with bipolar disorder. Although many promising results regarding the efficacy of theses techniques were described, the total number of studies is still low, highlighting the need of further studies in larger samples as to provide a definite picture regarding the use of clinical neuromodulation in bipolar disorder.


2019 ◽  
Vol 144 (1) ◽  
pp. 18-23 ◽  
Author(s):  
Varsha Manucha ◽  
John Henegan

Context.— Aggressive variant prostate cancer (AVPCa) develops in a subset of patients with metastatic castration-resistant prostate cancer. The clinical and histologic overlap of AVPCa with other neuroendocrine carcinomas of the prostate has resulted in a lack of consensus on its terminology and treatment. Objective.— To review AVPCa to familiarize pathologists with this entity so they can actively participate in the detection, ongoing research, and evolving management of AVPCa. Data Sources.— The English language literature was reviewed. Conclusions.— The current review summarizes the pathologic features of AVPCa, describes how it has been defined clinically, and discusses how biomarkers may inform treatment strategies in the future.


1999 ◽  
Vol 17 (7) ◽  
pp. 2264-2264 ◽  
Author(s):  
John M. Maris ◽  
Katherine K. Matthay

PURPOSE AND RESULTS: Neuroblastoma, the most common solid extracranial neoplasm in children, is remarkable for its clinical heterogeneity. Complex patterns of genetic abnormalities interact to determine the clinical phenotype. The molecular biology of neuroblastoma is characterized by somatically acquired genetic events that lead to gene overexpression (oncogenes), gene inactivation (tumor suppressor genes), or alterations in gene expression. Amplification of the MYCN proto-oncogene occurs in 20% to 25% of neuroblastomas and is a reliable marker of aggressive clinical behavior. No other oncogene has been shown to be consistently mutated or overexpressed in neuroblastoma, although unbalanced translocations resulting in gain of genetic material from chromosome bands 17q23-qter have been identified in more than 50% of primary tumors. Some children have an inherited predisposition to develop neuroblastoma, but a familial neuroblastoma susceptibility gene has not yet been localized. Consistent areas of chromosomal loss, including chromosome band 1p36 in 30% to 35% of primary tumors, 11q23 in 44%, and 14q23-qter in 22%, may identify the location of neuroblastoma suppressor genes. Alterations in the expression of the neurotrophins and their receptors correlate with clinical behavior and may reflect the degree of neuroblastic differentiation before malignant transformation. Alterations in the expression of genes that regulate apoptosis also correlate with neuroblastoma behavior and may help to explain the phenomenon of spontaneous regression observed in a well-defined subset of patients. CONCLUSION: The molecular biology of neuroblastoma has led to a combined clinical and biologic risk stratification. Future advances may lead to more specific treatment strategies for children with neuroblastoma.


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