Haemophagocytic lymphohistiocytosis in inflammatory bowel disease with virus infection

Li, Yuan; Xia, Xuefeng; Zhang, Jing; Song, Zhiqiang; Zhou, Liya; Zhang, Yaopeng; Huang, Yonghui; Shi, Yanhong; Quigley, Eamonn M.M.; Ding, Shigang

doi:10.5114/pg.2015.48995

Cited by 9 publications

(9 citation statements)

References 30 publications

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“…Pediatric patients with inflammatory bowel disease have been reported to have a 100-times greater risk of developing HLH compared to the general population [ 11 ]. Although it has not been extensively studied in adults, there have been case reports suggesting a relationship between Crohn disease and HLH [ 12 , 13 ]. It is presumed that immunosuppressant therapy in the treatment of inflammatory bowel disease plays a role in facilitating infection and thus predisposing to secondary HLH [ 13 ].…”

Section: Discussionmentioning

confidence: 99%

“…Although it has not been extensively studied in adults, there have been case reports suggesting a relationship between Crohn disease and HLH [ 12 , 13 ]. It is presumed that immunosuppressant therapy in the treatment of inflammatory bowel disease plays a role in facilitating infection and thus predisposing to secondary HLH [ 13 ]. In particular, infliximab is independently associated with the development of Hodgkin lymphoma, as well as secondary HLH [ 14 , 15 ].…”

Section: Discussionmentioning

confidence: 99%

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Hemophagocytic Lymphohistiocytosis Secondary to Unknown Underlying Hodgkin Lymphoma Presenting with a Cholestatic Pattern of Liver Injury

Booth

Osehobo²,

Rodgers-Soriano³

et al. 2018

Case Rep Gastroenterol

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Hemophagocytic lymphohistiocytosis (HLH) is an uncommon disease that often presents with nonspecific findings. A high index of suspicion is necessary to make a prompt diagnosis and prevent fatal disease. A 45-year-old man presented with fever, hypotension, abdominal pain, nausea, and vomiting. Imaging showed hepatosplenomegaly and laboratory tests revealed pancytopenia, increased ferritin, and a cholestatic pattern of injury with elevated alkaline phosphatase and total bilirubin. Due to a history of Crohn disease, systemic lupus erythematous, and rheumatoid arthritis, the patient was on immunosuppressants, including infliximab. After multiple negative cultures, persistent fever, and days of empiric broad spectrum antibiotics, our differential shifted to fever of unknown origin. A liver wedge biopsy revealed areas of sinusoidal dilatation with enlarged, activated macrophages containing erythrocytes and intracytoplasmic iron, consistent with hemophagocytosis due to HLH. The portal tracts showed mixed lymphoplasmacytic inflammation, a prominent bile ductular reaction, periportal fibrosis, and scattered large cells with occasional binucleation and prominent nucleoli. These cells stained positive for Epstein-Barr virus encoding region in situ hybridization, PAX5, CD15, and CD30, and hepatic involvement by classic Hodgkin lymphoma was diagnosed and determined to be the cause of the HLH and cholestatic pattern of injury. Simultaneously, a bone marrow biopsy showed diffuse involvement by Hodgkin lymphoma with a similar staining pattern. Aggressive treatment failed and the patient succumbed to multiorgan failure. HLH is a rare, potentially fatal disease, with nonspecific signs and symptoms, and should be considered in any patient presenting with fever and pancytopenia, especially if they are immune compromised.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis Secondary to Unknown Underlying Hodgkin Lymphoma Presenting with a Cholestatic Pattern of Liver Injury

Booth

Osehobo²,

Rodgers-Soriano³

et al. 2018

Case Rep Gastroenterol

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“…This indicates cellular immunity level and its predominance over the humoral link. Probably, therefore, in many cases, the acute form of the EBV infection ends with a rapid and, as a rule, complete recovery [12][13].…”

Section: Resultsmentioning

confidence: 99%

Features of Cytokine Status in Patients With Chronic Ebv-Infections

Kolesnyk

Sorokina

Malanchuk

et al. 2018

The Journal of v. N. Karazin Kharkiv National University, Series "Medicine"

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Infections caused by EBV are the most common and occupy an important place in the structure of herpes aetiology diseases. The purpose of this work was to study the characteristics of the cytokine status in patients with chronic EBV infection, depending on the level of viral replication. We examined 78 patients with chronic EBV infection, the main clinical manifestations of which were various immunopathological and immunodeficiency states: Group I – with low, Group II – with medium, Group III - with a high degree of viral replication. The Tiff method was used using the Vector-Best reagent kits (Novosibirsk, Russia) to study the cytokine profile in the serum of patients with EBV infection. The determination of alpha and gamma fractions of serum interferon was carried out using the ELISA method by means of the ProCon IF2 plus reagent kit manufactured by Protein Contour LLC (St. Petersburg, Russia). As a result of a study of the cytokine status in patients with chronic EBV infection, it was found that in all three groups there was a significant increase in both pro-inflammatory (IL-1β, IL-6, TNF-α) and antiinflammatory cytokines (IL-10, IL 4, TGFβ1). However, anti-inflammatory cytokinemia was more compensated in group I patients compared with patients in groups II and III. A decrease in IFN-α and IFN-γ was detected in all patients with chronic EBV infection while studying the interferon status. A correlation was found between the level of viral replication and a decrease in the level of IFN-α and IFN-γ. The identified features of the cytokine status in patients with chronic EBV infection can be used to optimize therapy and help develop a differentiated approach to the immunocorrection of these patients, depending on the level of viral replication.

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“…Rarely, patients on immunosuppressive therapy for autoimmune conditions can also develop HLH [7, 9]. Thiopurine treatment for inflammatory bowel disease is associated with an increased risk for developing viral infection driven HLH, and this risk is slightly higher in patients with Crohn’s disease versus ulcerative colitis [10-12].…”

Section: Discussionmentioning

confidence: 99%

An Elusive Diagnosis: Case Reports of Secondary Hemophagocytic Lymphohistiocytosis and Review of Current Literature

Tong

Godbole

Biniwale

et al. 2019

Cureus

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Hemophagocytic lymphohistiocytosis (HLH) is a rare and serious hematologic disorder characterized by severe immune system dysregulation with a cytokine storm and histologic evidence of hemophagocytosis. It can be inherited or develop secondary to other diseases. We present three cases of secondary HLH in patients with distinct backgrounds. Our objective is to characterize the unique features of the disease, its underlying associations, treatment, and potential prognostic variables. The first case was a 20-year-old male with a history of intravenous (IV) drug abuse who presented with multi-organ failure and septic shock. A diagnosis of HLH was suspected after finding a ferritin of >100,000 ng/mL and confirmed with bone marrow biopsy. Furthermore, the patient was found to have significant Epstein-Barr virus (EBV) viremia. He responded well to the HLH-94 protocol with the addition of rituximab and ganciclovir. The second case was a 50-year-old female with a history of human immunodeficiency virus (HIV) who presented with multi-organ failure and severe anemia. Ferritin was also significantly elevated and a bone marrow biopsy confirmed the diagnosis of HLH. She was started on HLH-94 protocol. Despite treatment, the patient expired due to worsening renal failure and shock. Her autopsy report also showed evidence of Hodgkin’s lymphoma. The third case was a 57-year-old male with a history of Crohn’s disease treated with infliximab and adalimumab, who presented with multi-organ failure and pancytopenia. A diagnosis of HLH was made based on clinical findings and later confirmed on bone marrow biopsy. He responded to HLH-94 protocol but experienced fatal gastrointestinal bleeding. Patients presenting with HLH are often critically ill and deteriorate rapidly. The diagnosis is often challenging to establish due to its variable presentation and association with other pathologies. A moderate index of suspicion should be present for patients who have febrile illness with pancytopenia, multi-organ failure, high ferritin, and low fibrinogen levels. We discuss associations with viral infections, hematologic malignancies and immunosuppressive therapy. Treatment is directed at suppressing the immune response and for secondary HLH, addressing the underlying conditions, such as use of rituximab for EBV viremia and treatment of lymphoma.

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Haemophagocytic lymphohistiocytosis in inflammatory bowel disease with virus infection

Cited by 9 publications

References 30 publications

Hemophagocytic Lymphohistiocytosis Secondary to Unknown Underlying Hodgkin Lymphoma Presenting with a Cholestatic Pattern of Liver Injury

Hemophagocytic Lymphohistiocytosis Secondary to Unknown Underlying Hodgkin Lymphoma Presenting with a Cholestatic Pattern of Liver Injury

Features of Cytokine Status in Patients With Chronic Ebv-Infections

An Elusive Diagnosis: Case Reports of Secondary Hemophagocytic Lymphohistiocytosis and Review of Current Literature

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