A case of Epstein-Barr virus–associated hemophagocytic lymphohistiocytosis with severe cardiac complications

Kawamura, Yoshiki; Miura, Hiroki; Matsumoto, Yuji; Uchida, Hidetoshi; Kudo, Kazuko; Hata, Tadayoshi; Ito, Yoshinori; Kimura, Hiroshi; Yoshikawa, Tetsushi

doi:10.1186/s12887-016-0718-3

Cited by 15 publications

(22 citation statements)

References 14 publications

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“…There is considerable overlap between these conditions in terms of pathophysiology, such as increased serum levels of IL-6, IL-8, TNF-α and soluble IL-2 receptor [ 12 ]. Some studies indicated that EBV-infected CD8 + T cells and high levels of the cytokines in EBV-HLH may play some role in development of coronary artery lesions [ 12 , 13 ]. Recent studies have shown that the serum levels of soluble CD163 and the soluble tumor necrosis factor receptor II/I ratio were significantly elevated in patients with EBV-HLH compared to those in patients with acute-phase KD [ 14 , 15 ].…”

Section: Discussionmentioning

confidence: 99%

Epstein–Barr virus-related hemophagocytic lymphohistiocytosis complicated with coronary artery dilation and acute renal injury in a boy with a novel X-linked inhibitor of apoptosis protein (XIAP) variant: a case report

Chen

Lin

et al. 2020

BMC Pediatr

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Background X-linked lymphoproliferative disease (XLP) is a rare inherited X-linked primary immunodeficiency diseases (PID). One such disease, X-linked inhibitor of apoptosis protein (XIAP) deficiency, is characterized by Epstein–Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH). However, EBV-HLH with coronary artery dilation and acute renal injury (AKI) in children is unusual. Case presentation We report the case of a young boy aged 17 months with a novel XIAP variant. He was initially diagnosed with EBV-HLH based on the HLH-2004 diagnostic criteria and the condition was accompanied by coronary artery dilation and acute renal injury. The comprehensive genetic analysis of peripheral blood-derived DNA revealed a hemizygous variant of the XIAP gene [c.116G > C(p.G39A)], which was inherited from his mother (heterozygous condition). After combined treatment with rituximab, intravenous immunoglobulin, corticosteroids, antiviral drugs, and mycophenolate mofetil (MMF) in addition to supportive therapy, his clinical manifestations and laboratory indexes were improved. The patient achieved complete remission with MMF treatment in the 8-month follow-up. Conclusions We report the [c.116G > C(p.G39A)] variant in the XIAP gene for the first time in a case of XLP-2 associated with EBV-HLH. For male patients with severe EBV-HLH, the possibility of XLP should be considered and molecular genetic testing should be used early in auxiliary diagnosis. Reports of EBV-HLH with coronary artery dilation and AKI in children are rare. In the patients with EBV-HLH, color Doppler echocardiography and urine tests should be monitored regularly. If necessary, renal biopsy can be performed to clarify the pathology. Treatment with rituximab, immunosuppressors and supportive therapy achieved a good effect, but long-term follow-up is required.

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Section: Discussionmentioning

confidence: 99%

Epstein–Barr virus-related hemophagocytic lymphohistiocytosis complicated with coronary artery dilation and acute renal injury in a boy with a novel X-linked inhibitor of apoptosis protein (XIAP) variant: a case report

Chen

Lin

et al. 2020

BMC Pediatr

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“…Neurological complications were not rare, including direct viral damage and secondary immunity damage with diverse and nonspecific manifestations and good prognosis 26 . In addition, EBV infection can cause rare complications of hematological, 27 cardiovascular, 28 and genitourinary 29 systems. EBVRI was prone to multi‐systemic damage.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics of primary and reactivated Epstein‐Barr virus infection in children

Yang

Gao

2020

Journal of Medical Virology

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Epstein‐Barr virus (EBV) infection occurs commonly in children and presents as a primary or reactivated infection, which are difficult for clinicians to distinguish. This study investigated the clinical characteristics of the two types of infections. Children with detectable plasma EBV‐DNA were retrospectively enrolled and divided into primary and reactivated infection group by EBV‐specific antibody. We analyzed the patients' characteristics, clinical manifestations, complications, inflammatory biomarkers, and viral load. A total of 9.3% of children with reactivation were immunocompromised over the long‐term. The primary infection mostly appeared as infectious mononucleosis (99.8%), while reactivation occurred as an infectious mononucleosis‐like disease (65.0%), hemophagocytic syndrome (22.6%), chronic active EBV infection (5.3%) and lymphoma (3.5%). The incidence of fevers, cervical lymphoditis, periorbital edema, pharyngotonsillitis, hepatomegaly and splenomegaly in primary infection were 93.3%, 93.0%, 51.5%, 66.0%, 76.2% and 63.9%, respectively; the incidence of those symptoms in reactivation was 84.0%, 46.9%, 15.4%, 18.5%, 18.5%, and 43.3%, respectively. The incidence of digestive, respiratory, cardiovascular, neurological, hematological, genitourinary complications and multiple serous effusion in primary infection was 68.8%, 18.1%, 8.0%, 0.8%, 2.9%, 0.0% and 2.3%; whereas the incidence of these complications in reactivation was 56.2%, 22.5%, 14.1%, 8.0%, 38.9%, 0.3% and 19.0%. Patients with reactivation were more prone to multi‐systemic damage. B‐cells were lower, and CD8+ T‐cells were higher in primary infection. Viral load was correlated with the level of different cytokines in primary and reactivated infection. EBV primary infection often presents as infectious mononucleosis. The reactivated infection affects more immunocompromised subjects with diverse and complex manifestations. Various complications are more commonly associated with reactivation as a result of different inflammatory responses to different types of infection.

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“…To establish the link between this unusual association, we performed a structured Medline search and found only nine reported cases of cardiac complications associated with HLH 4–12. Most of these cases were reported in children (five in nine) with only three cases reported in adults.…”

Section: Discussionmentioning

confidence: 99%

Takotsubo cardiomyopathy secondary to haemophagocytic lymphohistiocytosis in HIV patients: a comprehensive review

Ullah¹,

Hamid

Grover

et al. 2019

BMJ Case Rep

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Haemophagocytic lymphohistiocytosis (HLH) is an immune dysregulation disorder with variable presentations and non-specific features making it extremely difficult to diagnose early in the clinical course. Here, we are presenting a case of a young man who presented in cardiogenic shock with findings of anterolateral wall ischaemia on ECG. Echocardiography findings were consistent with takotsubo cardiomyopathy (TCM). Cardiac catheterisation showed clean coronary arteries and pulmonary artery pressure measurements showed high output cardiac failure. After extensive workup, the patient was diagnosed with HLH. In spite of aggressive supportive and definitive therapy, he eventually died due to a complicated clinical course. We did a comprehensive literature review and found that this is the first reported case of HLH presenting as TCM as the initial clinical manifestation.

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A case of Epstein-Barr virus–associated hemophagocytic lymphohistiocytosis with severe cardiac complications

Cited by 15 publications

References 14 publications

Epstein–Barr virus-related hemophagocytic lymphohistiocytosis complicated with coronary artery dilation and acute renal injury in a boy with a novel X-linked inhibitor of apoptosis protein (XIAP) variant: a case report

Epstein–Barr virus-related hemophagocytic lymphohistiocytosis complicated with coronary artery dilation and acute renal injury in a boy with a novel X-linked inhibitor of apoptosis protein (XIAP) variant: a case report

Clinical characteristics of primary and reactivated Epstein‐Barr virus infection in children

Takotsubo cardiomyopathy secondary to haemophagocytic lymphohistiocytosis in HIV patients: a comprehensive review

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