An Unusual Cause of Acute Liver Failure: Three Cases of Hemophagocytic Lymphohistiocytosis Presenting at a Transplant Center

Schneier, Amanda; Stueck, Ashley; Petersen, Bruce; Thung, Swan N.; Perumalswami, Ponni V.

doi:10.1055/s-0036-1571299

Cited by 18 publications

(10 citation statements)

References 13 publications

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“…[15] A transplant center reported 3 patients with liver failure induced by HLH in 1 year. [20] Therefore, increase awareness is one of the possible reasons for increased reports of ALF caused by HLH. However, whether the incidence of HLH in adult patients has been increased is currently unknown.…”

Section: Discussionmentioning

confidence: 99%

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Acute liver failure caused by hemophagocytic lymphohistiocytosis in adults

et al. 2016

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Background:Hemophagocytic lymphohistiocytosis (HLH) is a rare condition that can be caused by a primary or acquired disorder of uncontrolled immune response. Liver injury is a common complication of HLH; however, HLH presenting as acute liver failure (ALF) has rarely been reported in adults.Case summary:A 34-year-old man was admitted to our hospital with nausea and fatigue persisting for 2 weeks and jaundice for 1 week. He had hyperthermia at the onset of disease. At admission, he had severe liver injury with unknown etiology. The laboratory data showed that he had hyperferritinemia, thrombocytopenia, anemia, hypertriglyceridemia, and hypofibrinogenemia. Finally, a bone marrow biopsy revealed hemophagocytic cells, and he was diagnosed with HLH. The patient was treated with prednisone and plasma exchange. However, the liver function of the patient deteriorated, and he finally died of multiorgan failure.Conclusions:Reports of adult patients with ALF caused by HLH have increased, and HLH should be suspected in patients with ALF of indeterminate cause. Although the efficacy of the treatment strategy recommended by the HLH 2004 remains to be confirmed in adult patients with ALF caused by HLH, early diagnosis and prompt combined treatment with steroids and cyclosporin A or etoposide should be emphasized.

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Section: Discussionmentioning

confidence: 99%

“…The prognosis of adult patients with HLH is very poor, [19,20,30–33] with mortality ranging from to 41% to 75%, [6,11] depending on underlying diseases, [26] age, [34] ferritin level, [35] and so on. Whether the severity of liver injury determines the outcome of patients with HLH remains unknown.…”

Section: Discussionmentioning

confidence: 99%

Acute liver failure caused by hemophagocytic lymphohistiocytosis in adults

et al. 2016

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“…Very few patients have been documented to survive with their native liver. In recent years, HLH first presenting as ALF was becoming increasingly noticed while the mortality remained high[ 4 - 8 ]. HLH was thus considered an important differential diagnosis for ALF.…”

Section: Discussionmentioning

confidence: 99%

“…Patients with HLH almost always have evidence of liver inflammation, commonly being mild to moderate elevations of transaminases. Acute liver failure (ALF) associated with HLH is rarely reported and generally recognized to be extremely fatal[ 4 - 8 ]. Currently, there is a paucity of information on the successful treatment of ALF associated with HLH[ 9 ].…”

Section: Introductionmentioning

confidence: 99%

Successful rescue of acute liver failure and hemophagocytic lymphohistiocytosis following varicella infection: A case report and review of literature

Zhang

Guo

Zhu

et al. 2018

WJCC

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Herein we report a case of acute liver failure (ALF) and hemophagocytic lymphohistiocytosis (HLH) induced by varicella infection, successfully rescued by a combination therapy of acyclovir, supportive care, and immunosuppression with dexamethasone and etoposide. A previously healthy 16-year-old boy presented with generalized rash, fever, severe abdominal pain, and abnormal liver function within 4 d. Chickenpox was suspected, and acyclovir and intravenous immunoglobulin were started on admission. However, the patient’s condition deteriorated overnight with soaring transaminases, severe coagulopathy and encephalopathy. On the fourth day of admission, pancytopenia emerged, accompanied by hypofibrinogenemia and hyperferritinemia. The patient was diagnosed with ALF. He also met the diagnostic criteria of HLH according to the HLH-2004 guideline. Polymerase chain reaction (PCR) amplifications of varicella-zoster virus (VZV) were positive, confirming that VZV was a causative trigger for ALF and HLH. In view of the devastating immune activation in HLH, immunosuppression therapy with dexamethasone and etoposide was administered, in addition to high dose acyclovir. The patient’s symptoms improved dramatically and he finally made a full recovery. To our knowledge, this is only the second report of a successful rescue of ALF associated with HLH, without resorting to liver transplantation. The first case was reported in a neonate infected by herpes simplex virus-1. However, survival data in older children and adults are lacking, most of whom died or underwent liver transplantation. Our report emphasizes the clinical vigilance for the possible presence of HLH, and the necessity of extensive investigation for underlying etiologies in patients presenting with indeterminate ALF. Early initiation of specific therapy targeting the underlying etiology, and watchful immunosuppression such as dexamethasone and etoposide, together with supportive therapy, are of crucial importance in this life-threatening disorder.

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“…There have been case reports of HLH presenting as acute liver failure, which is associated with poor prognosis 1,2,5,9,11. The mechanism of liver injury is related to infiltration by hemophagocytic histiocytes or due to overproduction of cytokines.…”

Section: Discussionmentioning

confidence: 99%