Critical Care: Student/Resident Case Report Poster - Critical Care IV |

Takotsubo Cardiomyopathy Due to Hemophagocytic Lymphohistiocytosis FREE TO VIEW

Ahmad Abdin, MD
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MHRI/Brown University, Pawtucket, RI

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):435A. doi:10.1016/j.chest.2016.08.448
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SESSION TITLE: Student/Resident Case Report Poster - Critical Care IV

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Hemophagocytic lymphohistocytosis (HLH) is a rare and potentially fatal disease characterized by excessive immune response and cytopenia. We present a patient who presented with a febrile illness, pancytopenia and cardiogenic shock due to Takotsubo cardiomyopathy and diagnosed with HLH. Our case describes a very rare association of HLH with a unique form of reversible left ventricular dysfunction.

CASE PRESENTATION: A 59-year-old man with history of hypertension, presented to the emergency department with altered mental status for three days duration. Upon presentation, he was somnolent; temperature was 102.4 F, blood pressure 70/50 mmHg, heart rate 140 bpm, and oxygen saturating 96% on nonrebreather mask. lungs were clear to auscultation bilaterally. The cardiovascular examination revealed a regular rhythm with no murmur. Abdominal examination revealed no organomegaly. Neurologic examination was non-focal. Labs were notable for WBC of 12.8X103 /mcl, platelets 125X103 /mcl, acute kidney injury (creatinine 2.5 mg/dl), and transaminitis (ALT 48 IU/L, AST 94 IU/L). Troponin rose from 0.046 to 70 ng/ml (normal <0.03 ng/ml). EKG only showed sinus tachycardia. Head CT scan and chest X-ray were unremarkable. Patient was resuscitated with isotonic saline and treated with broad-spectrum antibiotics for presumed septic shock; vasopressors and stress dose steroids were added for hemodynamic support as well. He was intubated and started on mechanical intubation. Echocardiogram showed severely reduced ejection fracture of 15-20% with akinesia of mid and distal segments of myocardium, sparing the basal segments and consistent with Takotsubo cardiomyopathy. On day 2, he developed leukopenia and thrombocytopenia (WBC 2.1X103 /mcl, platelets 25X103/mcl). Extensive infectious workup was negative. In the presence of fever, cytopenia, elevated ferritin (>10,000 ng/ml), and triglycerides (>400 mg/dl), with no alternative explanation, the diagnosis of HLH was made. He was started on high dose steroids with significant improvement. Cardiomyopathy resolved on repeated echocardiogram

DISCUSSION: HLH is a life-threatening condition if not recognized and treated expeditiously. The disease may represent failure of NK cell-mediated clonal T cells apoptosis that controls the immune response following stimulation. This results in uncontrolled proliferation of activated macrophages that engulf hematopoietic cells within the reticuloendothelial system. Excessive inflammation and multi-organ dysfunction result from massive cytokines release. Takotsubo cardiomyopathy (TCM) is reversible left ventricular (LV) dysfunction characterized by apical ballooning and acute hypokinesia of the apical and middle segments of the LV that extends beyond a single coronary territory.

CONCLUSIONS: The acute cytokine storm of HLH may result in TCM and cardiogenic shock. Therefore, cardiac function may need to be assessed in patients with HLH.

Reference #1: Crit Care Med. 2015 Mar. 43 (3):686-93. [Medline].

DISCLOSURE: The following authors have nothing to disclose: Ahmad Abdin

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