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Severe Epstein Barr virus infection

MedGen UID:
1635633
Concept ID:
C4703482
Disease or Syndrome
Synonym: Fulminant infectious mononucleosis
 
HPO: HP:0031693

Definition

An unusually severe Epstein Barr virus (EBV) infection. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSevere Epstein Barr virus infection

Conditions with this feature

X-linked lymphoproliferative disease due to SH2D1A deficiency
MedGen UID:
1770239
Concept ID:
C5399825
Disease or Syndrome
X-linked lymphoproliferative disease (XLP) has two recognizable subtypes, XLP1 and XLP2. XLP1 is characterized predominantly by one of three commonly recognized phenotypes: Inappropriate immune response to Epstein-Barr virus (EBV) infection leading to hemophagocytic lymphohistiocytosis (HLH) or severe mononucleosis. Dysgammaglobulinemia. Lymphoproliferative disease (malignant lymphoma). XLP2 is most often characterized by HLH (often associated with EBV), dysgammaglobulinemia, and inflammatory bowel disease. HLH resulting from EBV infection is associated with an unregulated and exaggerated immune response with widespread proliferation of cytotoxic T cells, EBV-infected B cells, and macrophages. Dysgammaglobulinemia is typically hypogammaglobulinemia of one or more immunoglobulin subclasses. The malignant lymphomas are typically B-cell lymphomas, non-Hodgkin type, often extranodal, and in particular involving the intestine.
Autoinflammatory-pancytopenia syndrome due to DNASE2 deficiency
MedGen UID:
1803642
Concept ID:
C5676977
Disease or Syndrome
Autoinflammatory-pancytopenia syndrome (AIPCS) is an autosomal recessive disorder characterized by severe anemia and thrombocytopenia apparent from early infancy, hepatosplenomegaly, and recurrent fevers associated with a hyperinflammatory state. Additional systemic features may include chronic diarrhea, proteinuria with renal disease, liver fibrosis with elevated liver enzymes, deforming arthropathy, and vasculitic skin lesions. Some patients may have motor delay or learning difficulties associated with subcortical white matter lesions on brain imaging. Laboratory studies show increased levels of proinflammatory cytokines and increased expression of interferon-stimulated genes (ISGs), consistent with a type I interferonopathy (Rodero et al., 2017). Treatment with a JAK (see 147795) inhibitor (baricitinib) may be effective (Hong et al., 2020).

Professional guidelines

PubMed

Beaugerie L, Rahier JF, Kirchgesner J
Clin Gastroenterol Hepatol 2020 May;18(6):1324-1335.e2. Epub 2020 Feb 12 doi: 10.1016/j.cgh.2020.02.009. PMID: 32059920
Schechter S, Lamps L
Arch Pathol Lab Med 2018 Oct;142(10):1191-1195. doi: 10.5858/arpa.2018-0208-RA. PMID: 30281361
Kuwabara S
Drugs 2004;64(6):597-610. doi: 10.2165/00003495-200464060-00003. PMID: 15018590

Recent clinical studies

Etiology

Liu Y, Sun LY, Zhu ZJ, Wei L, Qu W, Zeng ZG
Ann Transplant 2018 Sep 25;23:666-673. doi: 10.12659/AOT.909941. PMID: 30250015Free PMC Article

Diagnosis

Ichikawa G, Negishi Y, Chishima F, Suzuki S
J Obstet Gynaecol Res 2024 Jun;50(6):1056-1060. Epub 2024 Mar 21 doi: 10.1111/jog.15928. PMID: 38511280
Worth AJ, Houldcroft CJ, Booth C
Br J Haematol 2016 Nov;175(4):559-576. Epub 2016 Oct 17 doi: 10.1111/bjh.14339. PMID: 27748521

Therapy

Liu Y, Sun LY, Zhu ZJ, Wei L, Qu W, Zeng ZG
Ann Transplant 2018 Sep 25;23:666-673. doi: 10.12659/AOT.909941. PMID: 30250015Free PMC Article
Okano M
Pediatr Hematol Oncol 1997 Mar-Apr;14(2):109-19. doi: 10.3109/08880019709030897. PMID: 9089739

Prognosis

Worth AJ, Houldcroft CJ, Booth C
Br J Haematol 2016 Nov;175(4):559-576. Epub 2016 Oct 17 doi: 10.1111/bjh.14339. PMID: 27748521
Tazawa Y, Nishinomiya F, Noguchi H, Takada G, Tsuchiya S, Sumazaki R, Takita H, Kanno H, Nose M, Konno T
Hum Pathol 1993 Oct;24(10):1135-9. doi: 10.1016/0046-8177(93)90195-m. PMID: 8406423

Clinical prediction guides

Ichikawa G, Negishi Y, Chishima F, Suzuki S
J Obstet Gynaecol Res 2024 Jun;50(6):1056-1060. Epub 2024 Mar 21 doi: 10.1111/jog.15928. PMID: 38511280
Tazawa Y, Nishinomiya F, Noguchi H, Takada G, Tsuchiya S, Sumazaki R, Takita H, Kanno H, Nose M, Konno T
Hum Pathol 1993 Oct;24(10):1135-9. doi: 10.1016/0046-8177(93)90195-m. PMID: 8406423

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