scholarly journals Subglottic acute lymphoblastic leukaemia

2018 ◽  
Vol 11 (1) ◽  
pp. e226364
Author(s):  
Shilpa Ojha ◽  
Julian Gaskin ◽  
Michael Saunders

Acute lymphoblastic leukaemia (ALL) is one of the the most common malignancies of childhood and can occasionally present as acute airway obstruction. We present the unusual case of a 1-year-old boy who was referred to our Paediatric Otolaryngology (ENT) clinic with a recurrent history of croup. This is the first reported case of localised ALL presenting as a subglottic mass in a paediatric patient. It highlights the need to have a broader differential diagnosis in children presenting with ‘recurrent croup’ including extramedullary presentation of leukaemia and to have a low threshold for performing endoscopy in such cases.

ORL ◽  
2021 ◽  
pp. 1-3
Author(s):  
Krupa R. Patel ◽  
Ashton E. Lehmann ◽  
Aria Jafari ◽  
Daniel L. Faden

Although nasal polyposis is a common clinical entity, there is limited literature describing the rare presentation of sudden prolapse of a massive nasal polyp resulting in an airway emergency in an adult. We present the first case report to our knowledge of a patient without any preceding sinonasal symptoms or history of anticoagulation who experienced acute upper airway obstruction due to sudden hemorrhage and prolapse of a large nasal polyp. Based on our experience treating this patient, we discuss special considerations in all phases of care to ensure safe and effective management of such an exceptional clinical scenario.


2021 ◽  
Vol 14 (5) ◽  
pp. e240503
Author(s):  
Craig John Hickson ◽  
Omar Ahmed ◽  
Juliet Laycock ◽  
Robert Hone

We describe a rare case of hypopharyngeal liposarcoma with an atypical presentation. The patient presented with a 3-month history of intermittent, transient acute airway obstruction. In between episodes, he was asymptomatic. A pedunculated tumour originating in the postcricoid region was seen to be suspended into the oesophagus and intermittently regurgitated into the larynx to cause airway obstruction. The lesion was endoscopically removed and examined histologically to confirm the diagnosis. On-going management of rare lesions such as this should be through multidisciplinary team meetings at a tertiary sarcoma centre.


2019 ◽  
Vol 72 (8) ◽  
pp. 558-561 ◽  
Author(s):  
Grazia Fazio ◽  
Valentina Massa ◽  
Andrea Grioni ◽  
Vojtech Bystry ◽  
Silvia Rigamonti ◽  
...  

Cornelia de Lange syndrome (CdLS) is a rare autosomal-dominant genetic disorder characterised by prenatal and postnatal growth and mental retardation, facial dysmorphism and upper limb abnormalities. Germline mutations of cohesin complex genes SMC1A, SMC3, RAD21 or their regulators NIPBL and HDAC8 have been identified in CdLS as well as somatic mutations in myeloid disorders. We describe the first case of a paediatric patient with CdLS with B-cell precursor Acute Lymphoblastic Leukaemia (ALL). The patient did not show any unusual cytogenetic abnormality, and he was enrolled into the high risk arm of AIEOP-BFM ALL2009 protocol because of slow early response, but 3 years after discontinuation, he experienced an ALL relapse. We identified a heterozygous mutation in exon 46 of NIPBL, causing frameshift and a premature stop codon (RNA-Targeted Next generation Sequencing Analysis). The analysis of the family indicated a de novo origin of this previously not reported deleterious variant. As for somatic cohesin mutations in acute myeloid leukaemia, also this ALL case was not affected by aneuploidy, thus suggesting a major impact of the non-canonical role of NIPBL in gene regulation. A potential biological role of NIPBL in leukaemia has still to be dissected.


Author(s):  
Swati Singh ◽  
Ravinder Ahlawat

Rupture of uterus is characterized by a breach in the wall of the uterus involving its full thickness. An unscarred uterus rupture is uncommon. It has non-specific symptoms and presentation differs according to site and time of rupture. Authors report an unusual case of spontaneous rupture of unscarred uterus. A 32-year-old, pregnant woman, developed postpartum bleeding with no history of prior uterine incision. She was diagnosed as a case of rupture of uterus and emergency laparotomy was done. Early diagnosis and immediate surgical intervention may significantly improve the prognosis. Differential diagnosis of uterine rupture should always be kept in mind in all patients with or without risk factors.


2020 ◽  
Vol 14 (1) ◽  
Author(s):  
Ahmad Al-Mousa ◽  
Mohammad Nour Shashaa ◽  
Mohamad Shadi Alkarrash ◽  
Mohamad Alkhamis ◽  
Lina Ghabreau ◽  
...  

Abstract Background Seminoma is the most common subtype of testicular cancer and occurs most commonly in patients aged 30–49 years, but decreases to a very low level in men in their 60s or older. Case presentation A 90-year-old Syrian man with a 6-year history of an increase in size of his right scrotum, presented to the urological clinic and, on clinical examination, the findings suggested testicular tumor. After orchiectomy and histology results based on microscopic and immunohistochemical examinations, a pure seminoma was diagnosed, so we describe in this case report the second-oldest patient with classical seminoma in the medical literature. Conclusion This case report has been written to focus on the probability of any type of testicular tumor occurring at any age or decade; urologists should consider seminoma as a differential diagnosis with any testicular swelling even in elderly patients.


Author(s):  
Catherine Mark ◽  
Sumit Gupta ◽  
Angela Punnett ◽  
Julia Upton ◽  
Julia Orkin ◽  
...  

Vaccination is a critical tool in the prevention of COVID-19 infection for individuals and for communities. The mRNA vaccines contain polyethylene glycol (PEG) as a stabilizer. Currently in North America only the BNT162b2 (Pfizer-BioNTech) mRNA vaccine is approved individuals 12 to 17 years of age. Most patients treated with contemporary regimens for acute lymphoblastic leukemia receive Peg-asparaginase and 10-30% will develop allergic reactions. Optimizing access and safety for vaccine administration for these patients critical. This report describes a process developed to support COVID vaccination in a cohort of adolescents and young adults with a history of PEG-asparaginase allergy.


2019 ◽  
Vol 7 (1-2) ◽  
pp. 51-56
Author(s):  
Syed Hasan Imam Al Masum ◽  
Ali Jacob Arsalan

Background & objective: Ludwig’s angina (LA) is a potentially life-threatening, rapidly spreading, bilateral cellulitis of the submandibular spaces in children. In the preantibiotic era, the airway obstruction was almost inevitable and case fatality rate was as high as 60%. With the introduction of antibiotics in 1940s the LA has become an uncommon disease. As such, many physicians have limited experience of it. But its early recognition and aggressive management still carries utmost importance to avoid life-threatening acute airway obstruction. Therefore, the present study was undertaken to update the physicians with clinical features and management of Ludwig’s angina. Methods: The present descriptive study was conducted in Bangladesh Institute of Child Health & Dhaka Shisu Hospital, Sher-e-Bangla Nagar, Dhaka between January 2012 to December 2016. Having obtained approval from the Institutional Review Board of the Institute, we retrospectively analyzed the clinical course and management of Ludwig’s angina. During the period a total of 27 patients’ record were found available. Data were collected on demographic and clinical characteristics, causes and predisposing factors, investigations, complications developed and outcome of LA. Penicillin with or without additional anaerobic coverage with clindamycin or metronidazole were used as key patient management strategy. Steroid was given for faster recovery of the patients having airway compromise. Patients who did not recover with conservative treatment underwent surgical treatment with incision and drainage. Result: In the present study children with Ludwig’s angina presented with bilateral swelling of the neck and submandibular region accompanied by pain and induration in the affected region. Systemic symptoms, such as, fever and malaise were also frequently present. Two-thirds (66%) of the children had dehydration and almost half (48%) had toxic look. Over one-third (37%) of the children exhibited, restricted backward and upward elevation of tongue and over half with trismus. Of the systemic signs, high temperature, tachycardia, and tachypnoea were common presentation. One-third of the children exhibited signs of airway obstruction. Fifty percent of the children had the history of toothache (lower molar) one or two weeks prior to the development of Ludwig’s angina, 40% had history of mumps and 3.7% had history of trauma to the mandible. Over one-quarter developed pneumonia with mediastinitis. Airway obstruction, manifested as unable to swallow saliva, dyspnoea, stridor and cyanosis, was exhibited by over 55% of the children and received intravenous steroid for faster recovery from the condition. More than three-quarters (77.7%) of the patients responded to Penicillin with or without clindamycin or metronidazole and those who did not respond to it (22.3%) underwent operative treatment. Conclusion: Despite modern medical and surgical interventions have improved the outcomes of Ludwig’s angina to a great extent, it still remains a potentially lethal disease in the pediatric population. Early recognition of the disease with identification of airway obstruction and prompt intravenous antibiotic therapy could resolve the disease without any complications or need for surgical intervention. Ibrahim Card Med J 2017; 7 (1&2): 51-56


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