Unicentric Castleman Disease and Pheochromocytoma

Castleman disease is an uncommon and heterogenous lymphoproliferative disorder which is classified as unicentric or multicentric depending on the number of lymph nodes involved. Each type has a different clinical presentation, aetiology, treatment and prognosis. We report the case of a young woman who presented with cervical lymphadenopathy and a retroperitoneal mass, and was diagnosed with unicentric Castleman disease and pheochromocytoma. We describe the diagnostic steps, the complications that developed, and the importance of the differential diagnosis in the evaluation of these patients.

Hoagland Sign as an Aid for Antimicrobial Stewardship—A Case Report

Hoagland sign is an early and transient bilateral painless upper eyelid edema observed in patients with Epstein-Barr virus (EBV)-related infectious mononucleosis. This sign can predate the appearance of exudative pharyngitis and cervical lymphadenopathy. Usually, this sign disappears by first week of infection. Here, we describe the occurrence of late onset Hoagland sign in a 14-year old boy who presented to us on 10th day of fever. Hoagland sign appeared after 10 days from symptom onset in our patient. Despite persistence of fever, the presence of Hoagland sign which appeared prior to confirmation of EBV infection was a helpful indicator for stopping antibiotics. In view of tonsillar hypertrophy with potential airway compromise and biochemical parameters suggestive of possible secondary hemophagocytic lymphohistiocytosis, he was initiated on steroids with which defervescence and prompt resolution of symptoms occurred. EBV can present as acute undifferentiated febrile syndrome which might result in inappropriate use of antibiotics. This case highlights the importance of using clinical clues like Hoagland sign to optimize antimicrobial stewardship.

Spectrum of Nasopharyngeal Cancers Seen in the Department of Radiation Oncology University College Hospital, Ibadan

Background: Nasopharyngeal cancer was initially thought to be an uncommon disease in Nigeria, but recent studies have shown a steady increase in the incidence of the disease with an associated increase in morbidity and mortality due to late presentation. Method: This was a retrospective review of patients with nasopharyngeal cancer seen in the department of Radiation Oncology U.C.H. Ibadan from 2000 to 2009. Patient's bio-data, clinical-pathologic presentation and site of referral were retrieved using data extraction form and subsequently analysed using Micrsoft Exel (2013). Results: A total of 205 patients with histologically confirmed nasopharyngeal cancer seen during the study period were analysed. The mean age of the patients was 41.7 years with 144 (70.2%) males and 61(29.8%) females. The commonest presenting complaint was cervical lymphadenopathy in 98.5% of patients followed by nasal blockade, nasal discharge and epistaxis with 82.0%, 78.0% and 51.2% respectively. Undifferentiated carcinoma was the commonest histologic type (68.8%). Majority of patients were civil servants (33.2%). The habit of smoking and alcohol ingestion were unspecified in majority of patients (43.4%). Ingestion of smoked fish has been found in majority of patients 122(59.5%) with the disease in this study. Most of the patients came from southern part of the country which coincide with geographic distribution of Burkitt lymphoma, hence the possibility of Ebsteinbarr virus (EBV) as one of the likely causative agents. Conclusion: Nasopharyngeal cancer is common. Cervical lymphadenopathy, nasal blockade with undifferentiated histology and late presentation were the norm. Referrals were from all the regions of the country.

Ocular manifestations and clinical profile of multisystemic inflammatory syndrome in children during COVID-19 pandemic

Background: Multisystemic inflammatory syndrome in children (MIS-C) is a new childhood disease, which is associated with Severe acute respiratory syndrome corona virus 2 (SARS-CoV-2). To evaluate ocular manifestations and clinical characteristics of MIS-C during COVID-19 pandemic.Methods: A cross-sectional observational study was conducted among 48 MIS-C patients (≤19 years) at Burdwan Medical college and hospital in West Bengal, India from April 2021 to June 2021. History taking, clinical examination and necessary investigations of all the patients were done.Results: Out of 48 patients, 18 patients (37.5%) presented with conjunctivitis, 4 (8.3%) with eyelid swelling, 2 (4.2%) with episcleritis, 2 (4.2%) with papilledema, 1 (2.1%) with subconjunctival haemorrhage, 1 (2.1%) with uveitis, 1 (2.1%) with retinitis and 4 (8.3%) with decreased vision. Other clinical manifestations included fever, skin rash, loose stool, vomiting, abdominal pain, cough, peripheral oedema, myalgia, breathlessness, altered sensorium, cervical lymphadenopathy, shock, oliguria, chest discomfort and joint swelling. Serum inflammatory, coagulation and cardiac markers were deranged.Conclusions: Ocular and other clinical manifestations in MIS-C were due to post-COVID immuno-dysregulation resulting in “cytokine storm” and hyper-inflammatory response. Conjunctivitis was the most common ocular manifestation. There was a positive correlation between severity of the conjunctival hyperaemia and level of serum inflammatory markers.

Kikuchi disease: An unusual cause of cervical lymphadenopathy in a young female

Kikuchi-Fujimoto disease (KD), also known as histiocytic necrotizing lymphadenitis, is a rare cause of unilateral cervical lymphadenopathy usually described in adolescents and young adults with female preponderance. Clinically patients present with palpable lymphadenopathy, fever, and loss of weight. Hence, it should be differentiated from other causes of lymphadenopathy such as tuberculosis, lymphoma, or malignancy. Although the majority resolve spontaneously without treatment some may require non-steroidal anti-inflammatory drug or steroids. It may also be associated with systemic lupus erythematosus. Hence early detection, prompt diagnosis, and follow-up of the patient are essential in all cases of unilateral cervical lymphadenopathy in young adults. Here, we describe a 22-year old female who presented with unilateral neck swelling and was clinically diagnosed initially as a case of tuberculous lymphadenitis which on biopsy turned out to be KD.

Lipschütz Ulcer and Epstein-Barr Virus Infection

Aims: Lipschütz ulcer (LU), also known as acute vulvar ulcer, is a rare cause of vulvar ulcerations of nonvenereal origin. Our aim is to alert about this manifestation of the disease and to prevent unnecessary treatment.Case description: we present a 15 years old female, without relevant family and past history, admitted in the emergency room with a painful vulvar ulcer, preceded by five days of fever and sore throat. On physical examination, she had enlarged, and erythematous tonsils and bilateral anterior cervical lymphadenopathy and the genital examination revealed vulvar oedema and a deep ulcer with necrotic plaques in labium minus. The exclusion of transmitted sexual disease led to a diagnosis of Lipschütz ulcer. She started symptomatic treatment, oral antibiotic and corticoid therapy. She was discharged from the hospital after 6 days of admission and returned to a consult one month later when it was observed an almost complete resolution of the lesions. No recurrences occurred until 3 months.Conclusion: LU is a misdiagnosed pathology, probably because doctors, in general, are not familiarized with that, and since the diagnosis is made by exclusion. Infectious, such as Epstein-Barr Virus infections, are proposed etiologies.

Presentation of Acute Lymphoblastic Lymphoma and Colorectal Carcinoma in the Context of Constitutional Mismatch Repair Deficiency Syndrome (CMMRD): a Case Report with Literature Review

Introduction: Constitutional mismatch repair deficiency (CMMRD) is a rare autosomal recessive disease-carrying an increased risk of cancers (pediatric tumors of central nervous system, haemato-lymphoid malignancies along with gastrointestinal (GI) cancer(s), which are usually seen in the second and third decade) leading to syndromic presentation. Causal mutations are detected in DNA mismatch repair (MMR) genes, including MLH1, PMS2, MSH2, and MSH6 that are also known for their established role in Lynch syndrome. We describe a case of CMMRD with an earlier (first decade of life) presentation of mediastinal acute lymphoblastic lymphoma and colorectal malignancy. Case Presentation: A five-year-old boy presented with respiratory complaints, bilateral cervical lymphadenopathy, multiple café au lait macules (CALMs) on the lower back, history of parental consanguinity with the death of three sisters due to brain tumor within 6 months of diagnosis. Computerized tomographic (CT) scan chest revealed a huge mediastinal mass. The patient underwent a trucut-biopsy of the mass. The results were significant for a pre T-cell acute lymphoblastic lymphoma. Suspicion of CMMRD was raised based on a combination of factors described above. A panel of mismatch repair (MMR) proteins was applied on the biopsy tissue that revealed loss of nuclear expression of MLH1 and PMS2 immunostaining in tumor cells with positive external controls. While on maintenance therapy for lymphoma, about a year later, the patient developed sub-acute intestinal obstruction due to a stenosing polypoidal circumferential tumor in the mid-sigmoid colon found on flexible sigmoidoscopy that was followed by endoscopic biopsies and insertion of a fully-covered self-expanding metallic adult biliary stent with a diameter of 10 mm and length of 6 cm leading to immediate relief of obstruction. Biopsies revealed adenocarcinoma with neuroendocrine differentiation. Metastatic tumor deposits were seen in the omentum, anterior abdominal wall, and the left peritoneal wall. Practical Implications: Earlier (first decade) presentation of gastrointestinal malignancy warrants that an earlier screening through radiological scans for any possible tumors and MMR protein expression analysis (loss in tumor plus normal non-tumor cells) are essential in patients having CALMs and family history of pediatric tumors.

A RARE CASE OF ROSAI-DORFMAN DISEASE IN HYPOTHYROIDISM PATIENT A DIAGNOSTIC COEXISTENCE

Rosai-Dorfman disease (RDD) is a rare, benign, and predominantly nodal disease that most commonly presents as bilateral, painless cervical lymphadenopathy; although inguinal, axillary, mediastinal, and hilar lymphadenopathy has also been reported. Apart from nodal involvement, RDD has extra nodal manifestations involving bone, soft tissue, and liver as well as constitutional symptoms of fever, night sweats, and weight loss, which make it reasonable to rule out the infectious, autoimmune, and malignant conditions as the differential diagnosis.

From Telemedicine to the ICU—Fever and Rash in a 9-Year-Old Girl

A 9-year-old girl presented to her primary care pediatrician via telemedicine during the initial months of the coronavirus disease 2019 pandemic because of 4 days of warmth perceived by her mother, decreased energy, and a new rash on her upper extremities. After 10 additional days of documented fever >38°C, worsening fatigue, and 1 day of nausea, vomiting, and diarrhea, she was allowed to schedule an in-person visit with her pediatrician after testing negative for severe acute respiratory syndrome coronavirus 2. She appeared ill on arrival to clinic, and her pediatrician recommended evaluation in an emergency department. Her initial laboratory testing revealed nonspecific elevation in several inflammatory markers and leukopenia, and she responded well to intravenous hydration. Over the next 2 weeks, her fever persisted, constitutional symptoms worsened, and she developed progressively painful cervical lymphadenopathy and pancytopenia. She was evaluated in clinic by several specialists and eventually was urged to present to the emergency department again, at which time she was admitted to the PICU. After consulting additional specialists and waiting for laboratory results, the team reached a definitive diagnosis and initiated therapy; however, she experienced rapid clinical decline shortly thereafter. The specialists who assisted with identification of the underlying etiology of her symptoms were able to work together to manage the subsequent complications.

Kikuchi Fujimoto Disease: A Case Report with Literature Review

Kikuchi-Fujimoto disease (KFD), also referred to as histiocytic necrotizing lymphadenitis, is an uncommon entity, usually benign, self-limiting disease characterised by fever and regional lymphadenopathy of unexplained aetiology. There is an increase in reported cases of KFD, particularly in Asia. It is important to recognize this condition because it may be confused for systemic lupus erythematosus (SLE), tuberculosis or lymphoma. The knowledge of this condition among clinicians and pathologists can help to avoid misdiagnosis and inappropriate treatment. It must be considered among the differential diagnoses of cervical lymphadenopathy. Here, we present a case of 20-year-old male who came to the hospital with complaints of mild fever, chills, fatigue and cervical lymphadenopathy. After radiological investigations an excisional biopsy of the cervical lymph node was done and the diagnosis of KFD was confirmed. Due to the paucity of cases in literature, reporting this case may help shed light on this rare disease.