Bruce, Keelie


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Purpose: Langerhans cell histiocytosis (LCH) is a proliferative disorder of Langerhans dendritic cells that has a wide range of clinical presentations. It may present with involvement of almost any system in the body. It can present in one organ system, or multiple organ systems. There have been rare reports of cardiac involvement, and a literature search revealed only one previously reported case with a possible pericardial effusion. We describe a case of Langerhans Cell Histiocytosis presenting with pericardial effusion in a 16 month-old child. Methods: A 16 month old Hispanic female presented to the emergency department with neck swelling for one week. The patient had a history of mild neck enlargement since birth, but over the last week had developed redness over the area with increased size. She appeared comfortable with no fever, dyspnea, or dysphagia. Her past medical and surgical histories were unremarkable, and her family history was noncontributory. Results: On physical exam, there was a non-tender 4-5 cm longitudinal mass in the midline. Her labs were normal. A neck ultrasound showed a 5x4.5 cm mass with mildly prominent lymph node on the left side. MRI showed a mass in the anterior neck and upper chest with an unknown origin. The patient had a chest x-ray and then underwent an excisional biopsy of the mass. After three days, hematology-oncology service was consulted and the chest x-ray was read as showing the known mass with cardiomegaly. A subsequent echocardiogram showed a large pericardial effusion with evidence of early tamponade and atrial collapse. The pathology results confirmed a diagnosis of LCH. The patient was admitted to the intensive care unit and underwent a pericardiocentesis where a large amount of clear serous fluid was obtained. Cytology revealed the presence of Langerhans cells. With a diagnosis of LCH, the patient was started on chemotherapy the next day. The patient's repeat chest x-ray showed no increase in size. The patient tolerated the chemotherapy well; the effusion did not re-accumulate. She was discharged after her third day of treatment. Conclusions: This case illustrates an unusual presentation of LCH. Pickens and Rosenheim reported a case of a 60 year old woman who was diagnosed with LCH and pericardial effusion. Pericardial fluid contained histiocytes, but it was unclear if these were truly Langerhans cells or mesothelial cells. Our case is unique because the patient was very young and the pericardial fluid contained cells with Langerhans cell markers.