Diagnostic Dilemma of Disseminated Histoplasmosis Mimicking Hemophagocytosis Lymphohistiocytosis in Patient with Rheumatoid Arthritis on Anti-TNF Therapy: Case Report and Review of the Literature.

Tumor necrosis factor inhibitors (TNFi) have become the cornerstone for the treatment of rheumatoid arthritis and other systemic autoimmune conditions. However, these biologic DMARDs can lead to various opportunistic infections such as viral infection, tuberculosis, and histoplasmosis. Furthermore, these biologics can also cause severe systemic inflammatory reactions known as hemophagocytosis lymphohistiocytosis (HLH) that can lead to multiorgan failure and high mortality. Due to overlapping clinical features and time-intensive microbiological culture methods, distinguishing between HLH and opportunistic infections can be challenging early in the disease course. We present a similar situation with our patient where the patient met the diagnostic criteria for HLH however was found to have disseminated histoplasmosis. This case uniquely evaluates the utility of the HLH diagnostic criteria and hemophagocytosis for accurate diagnosis of HLH.

Back Pain: An Ominous Harbinger of Aortitis.

Isolated aortitis is a rare entity and was recently included in the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides under the single organ vasculitis group. Isolated aortitis represents a challenging situation due to the lack of reliable diagnostic methodology. Here, we describe the case of a 46-year-old woman who presented with severe upper back pain. She tested negative for pulmonary embolism, myocardial infarction, and other usual causes of back pain. The case highlights the clinical presentation of a rare disease with a usual symptom.

Acute Liver and Renal Failure: A Rare Adverse Effect Exclusive to Intravenous form of Amiodarone.

Amiodarone is an antiarrhythmic drug which is highly effective against a wide spectrum of ventricular tachyarrhythmias making it irreplaceable in certain group of patients. We report an unusual case of acute liver and renal failure within 24 hours of initiation of intravenous (IV) amiodarone which resolved after stopping the medication. The mechanism of acute liver and renal toxicity is not clearly known but is believed to be secondary to amiodarone induced (relative) hypotension, idiosyncratic reaction to the drug, and toxicity of the vector that carries the medication, polysorbate-80. In this case review, we discuss the hyperacute drug toxicity caused by IV amiodarone being a distinctly different entity compared to the adverse effects shown by oral amiodarone and support the suggestion that oral amiodarone can be safely administered even in patients who manifest acute hepatitis with the IV form.

A Hematological Menace: Multiple Venous Thrombosis Complicated by Acquired Factor VIII Deficiency.

BACKGROUND: Acquired hemophilia A (AHA) classically presents with spontaneous bleeding of mucosal sites, GI tract, and subcutaneous tissues, often leading to large hematomas and ecchymosis. Among documented cases, 50% are idiopathic and few have been associated with trauma or surgery. We present a case of life-threatening bleeding caused by AHA, following trauma and complicated by multiple venous thrombi. CASE REPORT: A 21-year-old man presented with multiple injuries secondary to trauma leading to extensive life-saving surgery. Two weeks post-operatively, he developed multiple deep venous thrombi and was started on anticoagulation. Twenty-four days post-operatively, he started bleeding from multiple mucosal sites and developed an abdominal hematoma. Anticoagulation was stopped, with administration of fresh frozen plasma and vitamin K. Diagnosis of AHA was made based on low factor VIII level and presence of factor VIII inhibitors after an appropriate battery of tests ruled out other possible diagnoses. He was started on steroids and recombinant factor VIIa, leading to immediate improvement. Once stable, Rituximab infusions resulted in decreasing factor VIII inhibitor levels, with gradual normalization of PTT. CONCLUSIONS: AHA remains a diagnostic challenge because of its rarity, leading to delay in diagnosis and causing significant morbidity and mortality. Elevated PTT relative to PT/INR is a strong clue which should be followed by mixing studies. Very few cases have been associated with surgery or trauma and relatively few large, controlled trials have compared different treatment modalities for AHA. Growing evidence supports anti-CD20 (Rituximab) as an effective treatment option, as in this case.

Desmoid Tumors: A Clear Perspective or a Persisting Enigma? A Case Report and Review of Literature.

Desmoid tumors are benign but locally aggressive tumors of mesenchymal origin which are poorly circumscribed, infiltrate the surrounding tissue, lack a true capsule and are composed of abundant collagen. History of trauma or surgery to the site of tumor origin is elicited in up to one in four cases and they most commonly develop in the anterior abdominal wall and shoulder girdle but they can arise in any skeletal muscle. The clinical behavior and natural history of desmoid tumors are unpredictable and management is difficult with many issues remaining controversial, mainly regarding early detection, the role, type and timing of surgery and the value of non-operative therapies. We report a case of anterior abdominal wall desmoid tumor in a 40-year-old male with a previous history of surgery.

Choroid Plexus Papilloma Arising From the Temporal Horn With a Bilateral Hypersecretory Hydrocephalus: A Case Report and Review of Literature.

Cerebrospinal fluid (CSF) within the cerebral ventricular system is secreted by a neuroepithelial tissue which is called as the choroid plexus. Tumors arising from these tissues are rare. Choroid plexus papillomas (CPPs) have been denoted as WHO grade I of the choroid plexus tumors. Among the intracranial tumors, neoplasms of the choroid plexus constitute around 0.36-0.6%. CPPs are mostly slow growing and cause symptoms due to mass effect and obstructive hydrocephalus, resulting in increased intracranial pressure. We report a case of CPP arising from the temporal horn in a 7-year-old girl presenting with progressive head enlargement since birth due to bilateral massive hydrocephalus without any obstruction, making it purely a hypersecretory hydrocephalus. A drainage procedure followed by complete tumor resection was carried out in our case and the patient showed marked relief from her symptoms.

Epidemiology and histopathological spectrum of head and neck cancers in Bihar, a state of Eastern India.

Head and neck cancers are amongst the commonest malignancies, accounting for approximately 20% of the cancer burden in India. The major risk factors are tobacco chewing, smoking and alcohol consumption, which are all preventable. This retrospective study presents data from the histopathology register for a five year period from 2002-2006 at Patna Medical College and Hospital, a tertiary care hospital drawing patients from the entire Bihar state, the 3rd most populous state of India with the majority of the population residing in rural areas. Incidence rates based on sex, age, site of lesion, including age standardized incidence rates for males and females, with mean age of presentation, distribution of histological variants and year wise trend were calculated. Out of 455 head and neck neoplasias, 241 were benign while 214 were malignant. The most common age group for all malignant biopsies was 7th decade for males and the 5th decade for females. Malignant cases were commoner in males than females with the male:female ratio of 3.1:1, which was found to be statistically significant by the chi-square (χ2) test. The crude rate and age standardized incidence rate was 0.05 and 0.06 per 100,000 population respectively. Squamous cell carcinoma (SCC) contributed about 96% of all cases, with grade I being the most common. Larynx was the most common site for malignancy, the supraglottic region being its most commonly affected sub-site. This observed incidence patterns in the region are a reminder of widespread unawareness, low healthcare utilization with virtually non-existent cancer programs. It also underlines the need to advocate for reliable cost-effective programs to create awareness, for early detection and plan appropriate management strategies. There is a compelling demand for a cancer registry in this region as well as proper implementation of preventive measures to combat this growing threat of cancer, many of whose risk factors are preventable.