SESSION TITLE: ICU Cases
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 27, 2013 at 01:15 PM - 02:45 PM
INTRODUCTION: Hemophagocytic Syndrome also known as Hemolymphocytic histiocytosis (HLH) is a life threatening disorder characterized by severe dysregulated activation of the immune system leading to systemic inflammatory response syndrome (SIRS) and multiorgan failure. Its acquired form occurs secondary to infections, autoimmune disorders and malignancies. Although usually treated with immunosuppressants, allogenic bone marrow transplantation is the only therapy with long term survival. Bone marrow transplant is generally performed when patients are hemodynamically stable. We present a unique case of patient with HLH who successfully underwent hematopoetic stem cell transplant while he was critically ill in the medical intensive care unit (MICU).
CASE PRESENTATION: A 26 year old male with HLH secondary to myelodysplastic syndrome who was receiving preparatory regimen for allogenic stem cell transplant presented with fever, dyspnea and fatigue. He subsequently became unresponsive and developed cardiac arrest but successfully regained spontaneous circulation and was admitted to the MICU. He was treated for presumed sepsis with antibiotics, intravenous fluids and vasopressors. Over the next few hours, he became extremely hypotensive requiring four vasopressors. Laboratory abnormalities included pancytopenia, hyponatremia and extremely high ferritin levels. He was also found to have shock liver and acute kidney injury. All his cultures remained negative. He underwent sternal bone marrow biopsy that suggested active hemophagocytosis (Image1) . He was treated with high dose corticosteroids for overwhelming HLH with remarkable improvement in hemodynamics and liberation from mechanical ventilation. He underwent allogenic bone marrow transplant in the MICU without any complications. His post-transplant course remained unremarkable except moderate graft versus host disease that resolved with systemic steroids.
DISCUSSION: HLH is characterized by mutual stimulation of the innate and adaptive arms of the immune system leading to persistent hypercytokinemia and SIRS. Early treatment is critical to prevent progressive multi-organ failure. Our patient represents a unique case of this disorder that received bone marrow transplant when he was hemodynamically unstable requiring vasopressors.
CONCLUSIONS: Intensivists should have a low threshold to suspect this disorder in otherwise healthy individuals presenting with fulminant sepsis like picture without any identifiable source who do not respond to conventional treatment and have profound hyperferritinemia.
Reference #1: Okabe T, Shah G, Mendoza V, Hirani A, Baram M, Marik P. What intensivists need to know about hemophagocytic syndrome: an underrecognized cause of death in adult intensive care units. J Intensive Care Med. 2012 Feb;27(1):58-64
Reference #2: Chan KW, Mullen CA, Korbling M. Allogeneic peripheral blood stem cell transplantation for active hemophagocytic lymphohistiocytosis. Bone Marrow Transplant. 1998 Aug;22(3):301-2.
DISCLOSURE: The following authors have nothing to disclose: Asif Najmuddin, Kashif Hussain
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