A 6-Year-Old Girl With Undiagnosed Hemophagocytic Lymphohistiocytosis and Takotsubo Cardiomyopathy A Case Report and Review of the Literature Jaime Kaye Otillio, MD,* Jeffrey K. Harris, MD,† and Rachel Tuuri, MD* Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of highly stimulated immune responses to antigens that leads to life-threatening inflammation and multiple organ dysfunction. At presentation, HLH may uncommonly mimic septic shock. In this case, we present a 6-year-old girl presenting to the pediatric emergency department with a febrile illness and anemia that subsequently developed decompensated shock, initially thought to be septic. She was ultimately diagnosed with HLH. During initial HLH treatment, this patient also developed takotsubo cardiomyopathy, a unique pattern of reversible left ventricular dysfunction characterized by transient apical ballooning and hypokenesis of the left ventricle that spontaneously resolves. There are very few case reports of HLH-associated takotsubo cardiomyopathy. We believe that this is the first case of takotsubo cardiomyopathy in a child with HLH. Key Words: shock, hemophagocytic lymphohistiocytosis, HLH, takotsubo, cardiomyopathy (Pediatr Emer Care 2014;30: 561–565)


emophagocytic lymphohistiocytosis (HLH) is a rare disorder of highly stimulated immune responses to antigens that leads to life-threatening inflammation and multiple organ dysfunction and may present as a septic shock-like picture to the emergency department. In the case presented, secondary HLH also contributed to the development of takotsubo cardiomyopathy, a rare and unique pattern of left ventricular dysfunction that quickly and spontaneously resolves. Two case reports of takotsubo with HLH exist in the adult literature, but to our knowledge, this is the first case in a child with HLH. In this report, we will review the clinical characteristics and treatment of these 2 rare disorders.

CASE A 6-year-old girl presented to the pediatric emergency department (PED) with a 7-day history of fever and fatigue. On the second day of illness, she had been diagnosed at another facility with a urinary tract infection and placed on trimethoprimsulfamethoxazole. On the following day, she had returned to the other facility with fevers of 104°F to 105°F and a new rash on her legs and face, which was diagnosed as a viral exanthem. On day 6 of the illness, the patient had 1 episode of coffee ground emesis, and on day 7, the patient presented to our PED. The patient’s medical history included a brief neonatal intensive care unit stay at birth for meconium aspiration, allergic rhinitis, asthma, constipation, and sickle cell trait. Upon presentation, the patient had a weight of 19.1 kg (42%), a temperature of 40.5°C, heart rate of 148 beats/min, respiratory rate of 37 breaths/min, blood pressure of 81/43 mm Hg, From the Divisions of *Pediatric Emergency Medicine and †Pediatric Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, SC. Disclosure: The authors declare no conflict of interest. Reprints: Jaime Kaye Otillio, MD, 135 Rutledge Ave, MSC 566, Charleston, SC 29425 (e‐mail: [email protected]). Copyright © 2014 by Lippincott Williams & Wilkins ISSN: 0749-5161

Pediatric Emergency Care • Volume 30, Number 8, August 2014

and an oxygen saturation of 95% on room air. She was initially alert and non–toxic appearing. Her oropharynx was without lesions, erythema, or exudate, and her lungs were clear to auscultation bilaterally. The cardiovascular examination revealed a regular rhythm with a new 2/6 systolic ejection murmur. There were 2+ distal pulses and 2-second capillary refill time. Her mucous membranes were dry. During the abdominal examination, the patient denied pain on palpation but appeared uncomfortable. She had normal bowel sounds and absence of guarding or rebound. There was no organomegaly. Her neurological examination was normal. She had a papular rash located in bilateral popliteal fossae, but there were no petechiae or purpura noted. The patient was given ibuprofen for her fever. A chest radiograph showed interstitial markings consistent with a viral process. A complete blood count, blood culture, and basic metabolic panel were obtained, and a 20 mL/kg normal saline fluid infusion was given for mild dehydration. At the conclusion of her fluid infusion, a repeat set of vitals revealed a heart rate of 138 beats/min, respiratory rate of 32 breaths/min, and a blood pressure of 78/ 34 mm Hg. Additional intravenous access was obtained, a second rapid fluid bolus was given, and intravenous administration of ceftriaxone was begun. Subsequently, a rigid, distended abdomen developed. Vancomycin and piperacillin-tazobactam were added, and the surgical team was consulted. Her perfusion failed to improve despite additional fluid boluses and a dopamine infusion. It was titrated to 20 μg/kg per minute without an optimal effect, and a norepinephrine infusion was required to achieve vital sign improvement. A pediatric cardiology consult was obtained secondary to the previously undocumented murmur noted, but a bedside echocardiogram performed showed normal cardiac function without effusion. Because of her deteriorating mental status, she was intubated. A dose of intravenous hydrocortisone 20 mg was administered for a cortisol level of 17 μg/dL (relative adrenal insufficiency defined as 3 cm below the L costal margin) Cytopenia involving ≥2 cell lines • Hemoglobin

A 6-year-old girl with undiagnosed hemophagocytic lymphohistiocytosis and takotsubo cardiomyopathy: a case report and review of the literature.

Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of highly stimulated immune responses to antigens that leads to life-threatening inflammat...
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