Diffuse Large B-Cell Lymphoma with Secondary Hemophagocytic Lymphohistiocytosis Presenting as Acute Liver Failure

Patel, Ruchi; Patel, Haren; Mulvoy, William; Kapoor, Sumit

doi:10.14309/crj.2017.68

Cited by 12 publications

(11 citation statements)

References 18 publications

(15 reference statements)

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“…Of note, ALF due to diffuse large B-cell lymphoma is reported to account for only 0.26% (5/1910) [1]; to date, only ten cases of ALF due to diffuse large B-cell lymphoma have been reported (Table 1) [6][7][8][9][10][11][12][13][14][15], with the time to death following admission in six reported cases being 5 days [12,13], 8 days [7,9], 14 days [14], and 19 days [6], in contrast to only 3 days in the present case. It has been reported that the deaths were caused by multi-organ failure involving cardiovascular collapse, acute lung injury, and renal failure, in addition to hepatic failure [5].…”

Section: Discussionmentioning

confidence: 99%

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Acute liver failure associated with diffuse large B-cell lymphoma: an autopsy case report

Kubo

Kimura

Mabe

et al. 2020

Clin J Gastroenterol

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Acute liver failure (ALF) associated with malignant infiltration of the liver is rare and its pathological and radiologic features remain poorly described. An 87-year-old man was admitted to our hospital for anorexia for several days, high-grade fever from the previous day, and liver dysfunction but suddenly died on day 3 of hospitalization due to ventricular fibrillation. The patient was diagnosed at autopsy with malignant diffuse large B-cell lymphoma. To the best of our knowledge, this report represents a valuable addition to the ALF literature describing a case of ALF associated with diffuse large B-cell lymphoma diagnosed at autopsy.

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Section: Discussionmentioning

confidence: 99%

“…8 [13] 2017 57 M CT: hepatic steatosis, splenomegaly with multiple splenic infarcts, and lymphadenopathy…”

Section: Discussionmentioning

confidence: 99%

Acute liver failure associated with diffuse large B-cell lymphoma: an autopsy case report

Kubo

Kimura

Mabe

et al. 2020

Clin J Gastroenterol

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“…HLH occurs in about 1% of hematologic malignancies[ 12 , 13 ]; sometimes from chemotherapy for these malignancies[ 14 ]. Patients with malignancy have extremely high mortality, partly due to delayed diagnosis of HLH[ 15 , 16 ]. Suggested mechanisms of HLH associated with malignancy include: profound inflammation from immune activation, persistent antigen stimulation by cancer cells, and deranged immune response secondary to chemotherapy.…”

Section: Resultsmentioning

confidence: 99%

Acute liver failure secondary to severe systemic disease from fatal hemophagocytic lymphohistiocytosis: Case report and systematic literature review

Cappell

Hader

Amin

2018

WJH

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AIMTo systematically review liver disease associated with hemophagocytic lymphohistiocytosis (HLH), propose reasonable contraindications for liver transplantation for liver failure in HLH, and report an illustrative case.METHODSSystematic review according to PRISMA guidelines of hepatic manifestations of HLH using computerized literature search via PubMed of articles published since 1980 with keywords (“hemophagocytic lymphohistiocytosis” or “HLH”) AND (“liver” or “hepatic”). Two authors independently performed literature search and incorporated articles into this review by consensus. Illustrative case report presented based on review of medical chart, and expert re-review of endoscopic photographs, radiologic images, and pathologic slides.RESULTSA 47-year-old Caucasian male, was hospitalized with high-grade pyrexia, rash, total bilirubin = 45 g/dL, moderately elevated hepatic transaminases, ferritin of 3300 ng/dL, leukopenia, and profound neutropenia (absolute neutrophil count < 100 cells/mm³). Viral serologies for hepatitis A, B, and C were negative. Abdominal computed tomography scan and magnetic resonance imaging revealed no hepatic or biliary abnormalities. Pathologic analysis of liver biopsy revealed relatively well-preserved hepatic parenchyma without lymphocytic infiltrates or macrophage invasion, except for sparse, focal hepatocyte necrosis. Bone marrow biopsy and aspirate revealed foamy macrophages engulfing mature and precursor erythrocytes, consistent with HLH. Interleukin-2 receptor (CD25) was highly elevated, confirming diagnosis of HLH according to Histiocytic Society criteria. Patient initially improved after high-dose prednisone therapy. Patient was judged not to be a liver transplant candidate despite model for end stage liver disease (MELD) score = 33 because liver failure was secondary to severe systemic disease from HLH, including septic shock, focal centrilobular hepatocyte necrosis from hypotension, bone marrow failure, and explosive immune activation from HLH. The patient eventually succumbed to overwhelming sepsis, progressive liver failure, and disseminated intravascular coagulopathy. Systematic review reveals liver injury is very common in HLH, and liver failure can sometimes occur. Data on liver transplantation for patients with HLH are very limited, and so far the results have shown a generally much worse prognosis than for other liver transplant indications. Liver transplantation should not be guided solely by MELD score, but should include liver biopsy results and determination whether liver failure is from intrinsic liver injury vs multisystem (extrahepatic) organ failure from HLH.CONCLUSIONThis case report illustrates that liver transplantation may not be warranted when liver failure associated with HLH is primarily from multisystem failure from HLH. Liver biopsy may be very helpful in determining the severity and pathophysiology of the liver disease.

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“…The primary form, usually seen in infants and children, results from genetic missense mutations in the perforin genes, responsible for NK cell and cytotoxic T lymphocyte function. 8,9 Secondary haemophagocytic lymphohistiocytosis, seen in adults, is caused by infections, malignancy, rheumatological and metabolic diseases. Epstein-Barr virus is the most consistent, in up to a third of secondary haemophagocytic lymphohistiocytosis.…”

Section: Discussionmentioning

confidence: 99%

A rare cause of acute liver failure due to haemophagocytic lymphohistiocytosis secondary to diffuse large B-cell lymphoma

et al. 2021

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Acute liver failure is a life-threatening condition commonly caused by drug-induced hepatotoxicity or viral hepatitides. However, there are a number of rarer causes such as haemophagocytic lymphohistiocytosis. Haemophagocytic lymphohistiocytosis is a syndrome of uncontrolled immune cell activation, triggered by infection or malignancy, which carries a high mortality. Whilst mild to moderate liver injury is commonly seen with haemophagocytic lymphohistiocytosis, acute liver failure has rarely been reported in adults. We present a case of a 74-year-old man with acute liver failure secondary to haemophagocytic lymphohistiocytosis triggered by undiagnosed large B-cell lymphoma. Initially treated for biliary sepsis, there was a delay in the diagnosis of haemophagocytic lymphohistiocytosis and despite initiating chemotherapy, he died soon after. This case highlights the importance of considering haemophagocytic lymphohistiocytosis as a rare cause of acute liver failure, as given the life-threatening potential of haemophagocytic lymphohistiocytosis, a prompt diagnosis may allow early initiation of chemotherapy for any chance of survival.

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Diffuse Large B-Cell Lymphoma with Secondary Hemophagocytic Lymphohistiocytosis Presenting as Acute Liver Failure

Cited by 12 publications

References 18 publications

Acute liver failure associated with diffuse large B-cell lymphoma: an autopsy case report

Acute liver failure associated with diffuse large B-cell lymphoma: an autopsy case report

Acute liver failure secondary to severe systemic disease from fatal hemophagocytic lymphohistiocytosis: Case report and systematic literature review

A rare cause of acute liver failure due to haemophagocytic lymphohistiocytosis secondary to diffuse large B-cell lymphoma

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