Hemophagocytic Lymphohistiocytosis: an Under-recognized and Life-threatening Condition.

Diagnosis of hemophagocytic lymphohistiocytosis is a challenge in Nepal because of limited resources and the high prevalence of tropical febrile illness mimicking hemophagocytic lymphohistiocytosis. We retrospectively reviewed medical records of 21 patients who were diagnosed with hemophagocytic lymphohistiocytosis from 2010 to 2015 at a single center in Nepal. Two patients had a mutation in their perforin gene and underwent successful haploidentical stem cell transplantation. Marrow hemophagocytosis was found only in 57% of the patients. Five patients had hematological malignancy and were treated with disease-specific chemotherapy. Seven patients developed hemophagocytic lymphohistiocytosis secondary to an infection, including visceral leishmaniasis, scrub typhus, and Epstein Barr virus. EBV-associated hemophagocytic lymphohistiocytosis was refractory to hemophagocytic lymphohistiocytosis 94 protocol, including the addition of rituximab. Malignancy and infection-associated hemophagocytic lymphohistiocytosis was more common. The most common clinical presentations included fever, splenomegaly, hyponatremia, liver function derangement, hyperfibrinogenemia, hyperferritinemia, and cytopenia. With a mortality of 29% in our study cohort, hemophagocytic lymphohistiocytosis should be considered a lethal disease, and clinicians should maintain a high index of suspicion to diagnose this disease. Keywords: Hemophagocytic lymphohistiocytosis; infection; malignancy.

A Case Report of Aerococcus urinae Urinary Tract Infection in an Elderly Male with Multimorbidity.

Aerococcus urinae is a rare cause of urinary tract infection (UTI) seen in elderly males with multimorbidity. Incidence is estimated between 0.15 and 0.8%. This organism is frequently misidentified for other gram-positive species. Missed or delayed diagnosis of A. urinae UTI can lead to systemic infection with high morbidity and potential mortality. We present a classic case of A. urinae UTI in a 91-year-old male with multiple comorbidities, including heart failure, diabetes mellitus, and metastatic prostate carcinoma. Empiric therapy with nitrofurantoin was unsuccessful, but intravenous ceftriaxone and bladder catheterization resulted in rapid symptomatic improvement. Variable antimicrobial sensitivities and resistance have been reported for A. urinae. Therefore, antimicrobial resistance testing should be performed for all patients with A. urinae infections.

Paget-Schroetter Syndrome: A Rare Case of Upper Extremity Deep Vein Thrombosis in a Young Swimmer.

Paget-Schroetter syndrome (PSS) is a rare form of spontaneous upper extremity deep vein thrombosis associated with vigorous activity of the upper extremities. We present a rare case of a young swimmer who presented with a painful right upper extremity swelling, with compression ultrasound (CUS) revealing extensive venous clots in the basilic, axillary, and subclavian veins. Venous duplex revealed extrinsic compression of the subclavian vein, and catheter-based contrast venography confirmed our diagnosis of PSS. The patient was started on a therapeutic dose of subcutaneous enoxaparin and referred to a higher center for further intervention.

A Rare Case of Leptomeningeal Carcinomatosis Secondary to Metastatic Non-Small Cell Lung Carcinoma.

Leptomeningeal carcinomatosis is a rare complication of metastatic systemic malignancy, with lung cancer being the most common cause. We present a case of a 75-year-old man with a past medical history of right non-small cell lung carcinoma and ischemic stroke who presented with a persistent headache and swallowing difficulties. On evaluation, the patient was initially diagnosed with a subacute infarct of the right posterior frontal lobe following magnetic resonance imaging (MRI). The patient's headache and dysphagia worsened, increasing the possibility of brain metastasis. The patient underwent cerebrospinal fluid analysis including cytology and multiple MRI studies with no obvious explanation for the symptoms. The patient eventually developed multiple cranial nerve palsies, and a diagnosis of leptomeningeal carcinomatosis was made with neuroradiology consultation for the MRI.

A Rare Case of Hemorrhagic Pericardial Effusion With Cardiac Tamponade Caused by Needle Embolism in an Intravenous Drug User.

Needle embolization in the heart leading to pericardial effusion with cardiac tamponade is rare. We present an unusual case of an intravenous drug user presenting with severe anginal pain with a history of intravenous needle use one month back, whose bedside echocardiography demonstrated pericardial effusion leading to tamponade. Emergent open sternotomy and exploration revealed an inadvertent tuberculin needle and hemorrhagic pericardial effusion. After removal of the needle, subsequent multiple follow-up echocardiography did not demonstrate reoccurrence of effusion.

Pseudohyperkalemia Associated With Leukemia.

Elevated potassium levels can be a life-threatening emergency. We describe a case of falsely elevated serum potassium level in a patient with leukemia, which was suspected to be falsely elevated because the patient was asymptomatic with a normal electrocardiogram (EKG). Common reasons behind such a discrepancy in leukemia patients are the use of a tourniquet before collection, use of vacuum/pneumatic tubes for transportation, prolonged periods of incubation, use of heparin for sample collection, and processing of samples via centrifugation. Since the process is related to the method of collection and processing, we recommend using rapid point of care testing in such cases to differentiate between false and true potassium elevation, as it is a well-validated tool. Moreover, there is a good correlation between potassium measured with the blood gas, point of care, and central laboratory analyzers when the concentration of potassium is above 3 mEq/L.

Arrhythmia With Lithium Toxicity Requiring Urgent Dialysis: A Case Report.

Lithium has been widely used as a mood stabilizer. With its narrow therapeutic index, systemic side effects, primarily neurological are a concern. Cardiotoxic effects of lithium are rare, reported as non-specific T-wave flattening, prolonged QT interval, sinus node dysfunction, ventricular tachycardia, cardiomyopathy, and myocardial infarction. We report an interesting case of a young female patient with schizoaffective disorder on lithium who developed life-threatening cardiotoxicity secondary to lithium requiring urgent dialysis.