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

Abstract

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.

Upadacitinib treatment associated with varicella zoster infection complicated by haemophagocytic lymphohistiocytosis in a patient with severe hidradenitis suppurativa

“…The induction of HLH syndrome by virus infections is well known and always a challenge in patients with a septic condition. 2,3 Our patient also had Crohn's disease in addition to HS. A connection between inflammatory bowel diseases, HLH and viral infections is discussed in the literature.…”

Hemophagocytic Lymphohistiocytosis (HLH) Due to Fulminant Salmonella Sepsis in the Setting of IL12Rβ1 (Interleukin 12 Receptor Beta 1) Deficiency

“…The patient fulfilled six out of eight HLH 2004 diagnostic criteria [ 4 ]. She had a fever, splenomegaly, cytopenia, high ferritin, high triglycerides, and low fibrinogen.…”

“…Due to the high possibility of malignancy, the hematology team was on board from the beginning. The patient fulfilled six out of eight HLH 2004 diagnostic criteria [4]. She had a fever, splenomegaly, cytopenia, high ferritin, high triglycerides, and low fibrinogen.…”

“…This case report discusses an unusual presentation of MSMD as secondary hemophagocytic lymphohistiocytosis (HLH). HLH is a rare life-threatening hyperinflammatory syndrome caused by aberrantly activated macrophages and cytotoxic T-cells, which can rapidly progress to terminal multi-organ failure [ 4 ]. It has two forms, a primary form, which is usually familial with genetic inheritance, and a secondary form, which is usually attributed to various etiologies including infections, hematological malignancies, and others [ 5 ].…”