Hemophagocytic Lymphohistiocytosis and Inflammatory Bowel Disease: Case Report and Systematic Review

Brambilla, Bárbara; Barbosa, Antônio Vinícius Corrêa; Scholze, Cassiano; Riva, F.; Freitas, Leonardo Furtado; Balbinot, Raul Angelo; Soldera, Jonathan

doi:10.1159/000506514

Cited by 24 publications

(17 citation statements)

References 50 publications

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“…All known biologicals used for the treatment of inflammatory bowel disease are reported in association with the development of HLH. However, thiopurins are the leading treatment in patients with HLH and they are well described as a strong immunosuppressor (7). It is still not clear what the role of ustekinumab in this case was.…”

Section: Conflict Of Interestmentioning

confidence: 89%

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Parvovirus B19-triggered hemophagocytic lymphohistiocytosis in a patient with Crohn’s disease

Debels

Cauwelier

Willandt

et al. 2022

AGEB

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Background: Hemophagocytic lymphohistiocytosis (HLH) is a life threatening condition caused by inappropriate immune activity. Infection is often the trigger, both in genetically predisposed and in sporadic cases. Although more commonly seen in the paediatric population, patients of all ages can be affected. Case presentation: A 26-year-old male patient with Crohn’s disease, treated with ustekinumab, presented with high fever, epistaxis and anorexia. Laboratory results showed pancytopenia, and a high serum levels of ferritin and LDH. Colonoscopy revealed only mild signs of disease activity. CT-scan showed splenomegaly and multiple lymphadenopathies. Bone marrow aspirate was suggestive for hemophagocytosis. PCR & serology for parvovirus B19 came back positive. Treatment with ustekinumab was temporarily put on hold and supportive care was given. Viral replication decreased and he recovered completely. Conclusion: There is a known association between HLH and Crohn’s disease. This is probably because they are more susceptible to infections with CMV, EBV and parvovirus B19, all known as triggers for HLH. The role of ustekinumab is unclear: did it play a role in the pathophysiological evolution of this primo-infection with parvovirus B19? On the other hand, did it contribute to the rather mild course of the disease, acting as a immunomodulator that works on interleukin-12, a cytokine that plays a role in HLH? Further study is warranted to answer these questions.

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Section: Conflict Of Interestmentioning

confidence: 89%

“…It binds to the p40 subunit and impedes the interaction with the IL-12Rβ1 on the cell surface of NK, T cells, or antigen-presenting cells. This process results in the blockade of IL-12 and IL-23 mediated downstream cell signaling, gene activation and cytokine production (7).…”

Section: Conflict Of Interestmentioning

confidence: 99%

Parvovirus B19-triggered hemophagocytic lymphohistiocytosis in a patient with Crohn’s disease

Debels

Cauwelier

Willandt

et al. 2022

AGEB

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“…Bambrilla et al presented a systematic review regarding patients with IBD and infectious HLH. They found an association between the use om immunomodulating treatment for IBD, and HLH development ( 80 ). The patient group reviewed presented with the same symptoms as described in the present study.…”

Section: Discussionmentioning

confidence: 99%

Concomitant Hemophagocytic Lymphohistiocytosis and Cytomegalovirus Disease: A Case Based Systemic Review

et al. 2022

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BackgroundHemophagocytic lymphohistiocytosis (HLH) is an immune mediated life-threatening condition. It is driven by an overactivation of the immune system and causes inflammatory tissue damage potentially leading to organ failure and death. Primary HLH is caused by genetic mutations, while secondary HLH is triggered by external factors. Viral infections are a well-known cause of secondary HLH. Cytomegalovirus (CMV) is a virus in the herpes family known to cause HLH in rare cases.MethodsWe report a recent case of CMV-induced HLH, followed by a systematic review of described cases of this rare disease entity, through a structured search in the medical database PubMed. All articles were assessed on a predetermined set of inclusion criteria.ResultsA total of 74 patients (age > 18 years) with CMV-related HLH were identified, 29 men, 42 women, and three patients with unspecified gender. Median age was 37.5 years (range 18–80). Sixty-six patients (88%) had one or more comorbid conditions and 22 patients (30%) had inflammatory bowel disease (IBD), the most frequent comorbidity. Forty patients (54%) received some form of immunomodulating treatment prior to HLH development. The general treatment approach was in general dual, consisting of antiviral treatment and specific immunomodulating HLH treatment approaches. Treatment outcome was at 77% survival, while 23% had fatal outcome.ConclusionThe findings highlight the importance of early diagnostic work up and treatment intervention. Ability to recognize the characteristic clinical traits and perform specific HLH diagnostic workup are key factors to ensure targeted diagnostic work and treatment intervention for this patient group.

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“…Most likely, the hypo-perfused state in critically ill children can increase the risk of AAC [ 7 ]. However, we were able to review four such cases in adults [ 5 , 8 , 9 , 10 ]. Two of them with underlying autoimmune diseases, adult-onset Still’s disease and Kikuchi disease [ 5 , 8 ].…”

Section: Discussionmentioning

confidence: 99%

Rare But Fatal: Hemophagocytic Lymphohistiocytosis (HLH) With Acute Acalculous Cholecystitis

Javed¹,

Crouch²,

Combs³

2022

Cureus

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Acalculous cholecystitis is an acute inflammatory disease of the gall bladder with high morbidity and mortality rate. It can be seen in trauma, burns, sepsis, total parenteral nutrition, prolonged fasting, and autoimmune diseases. However, there are very few reports of acalculous cholecystitis with macrophage activation syndrome (MAS) and hemophagocytic lymphohistiocytosis (HLH) in patients with underlying rheumatic/autoimmune disorders.Here we report a 23-year-old male with a past medical history of granulomatosis with polyangiitis who presented with fever, weight loss, and pancytopenia. A comprehensive infectious evaluation was done including bacterial cultures and viral and fungal serologies. Repeat abdominal imaging obtained later due to developing abdominal pain raised concerns for acute acalculous cholecystitis. Despite aggressive management of sepsis, the patient continued to decline clinically. HLH was suspected when the patient was found to meet the clinical criteria with fever, splenomegaly, cytopenia, hypertriglyceridemia, elevated liver function tests, hypofibrinogenemia, and ferritin of 22K ng/mL, absent NK cell activity, and elevated soluble CD25 receptor levels. Bone marrow biopsy did not reveal hemophagocytosis. Intravenous methylprednisolone was started and the patient showed remarkable clinical improvement with a decrease in all inflammatory markers and did not require any surgical intervention. On the review of the literature, we were able to identify four female patients with underlying adult-onset Still's disease and Kikuchi disease who presented with HLH along with acalculous cholecystitis likely triggered by flare. Our male patient presented with HLH and acute acalculous cholecystitis. He had a history of granulomatosis polyangiitis (GPA) that remained in remission. Hypersecretion of pro-inflammatory cytokines and cytotoxic cells in HLH promotes ischemia of the gall bladder wall. Early initiation of immunosuppressive therapy under careful observation can prevent surgical intervention and mortality in these patients.

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Hemophagocytic Lymphohistiocytosis and Inflammatory Bowel Disease: Case Report and Systematic Review

Cited by 24 publications

References 50 publications

Parvovirus B19-triggered hemophagocytic lymphohistiocytosis in a patient with Crohn’s disease

Parvovirus B19-triggered hemophagocytic lymphohistiocytosis in a patient with Crohn’s disease

Concomitant Hemophagocytic Lymphohistiocytosis and Cytomegalovirus Disease: A Case Based Systemic Review

Rare But Fatal: Hemophagocytic Lymphohistiocytosis (HLH) With Acute Acalculous Cholecystitis

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