Case report
Agranulocytosis after infectious mononucleosis

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Abstract

We report the case of a 5-year-old boy with agranulocytosis after infectious mononucleosis (IM). Antibodies against anti-human neutrophil-specific antigens (HNA)-1 were detected in his serum. A literature review on agranulocytosis after IM and our case suggest that anti-HNA-1 antibodies play important roles in agranulocytosis associated with IM.

Section snippets

Why this case is important?

Infectious mononucleosis (IM) is a common clinical manifestation of primary Epstein–Barr virus (EBV) infection in children. Mild neutropenia is well-known to occur concomitantly with IM in the first weeks of illness,1, 2 but agranulocytosis or severe neutropenia after IM is very rare.2, 3, 4 The pathophysiology of agranulocytosis is thought to be maturation arrest of the myeloid series and/or immunological mechanisms.2, 3, 4 Here, we present a case of agranulocytosis after IM and discuss the

Case description

A previously healthy 5-year-old boy had fever and general malaise for 9 days before admission. He visited a family doctor and was diagnosed with infectious mononucleosis because he had tonsillitis, cervical lymphoadenopathy and hepatosplenomegaly on clinical examination. The total white blood cell (WBC) count was 15,200/μL with 32% neutrophils, 44% lymphocytes and 19% atypical lymphocytes. The serum aspartate aminotransferase (AST) level was 86 IU/mL, the alanine aminotransferase (ALT) level was

Other similar and contrasting cases in the literature

Agranulocytosis or profound neutropenia after IM is very rare, with only 29 cases of agranulocytosis after IM reported in the English-language literature since 1925 (search on PubMed Central). In these cases, agranulocytosis after IM had underlying characteristics of (1) occurrence about 1 month after onset of IM, (2) recurrent or persistent fever, (3) redevelopment of cervical lymphadenopathy, (4) no predominance of age or sex, (5) improvement spontaneously within 5–7 days, (6) complication

Discussion

The pathophysiology of agranulocytosis after IM remains uncertain, but may involve hypoproduction or maturation arrest of myeloid cells in the marrow due to the direct effect of EBV2, 3, 4 or antibody-mediated peripheral destruction of myeloid cells.5

The mechanism of maturation arrest of myeloid cells has been suggested because mature myeloid cells disappear in the patient's bone marrow. In support of this hypothesis, Sumimoto et al. were unable to detect anti-neutrophil antibodies in a case of

Funding

None.

Competing interests

None declared.

Ethical approval

Not required.

References (10)

  • E.H. Husain et al.

    Infectious etiologies of transient neutropenia in previously healthy children

    Pediatr Infect Dis J

    (2012)
  • B. Koziner et al.

    Agranulocytosis following infectious mononucleosis

    JAMA

    (1973)
  • W.P. Hammond et al.

    Severe neutropenia in infectious mononucleosis

    West J Med

    (1979)
  • S. Sumimoto et al.

    Agranulocytosis following infectious mononucleosis

    Eur J Pediatr

    (1990)
  • S. Auvin et al.

    Is agranulocytosis following infectious mononucleosis caused by autoimmunity?

    Pediatr Hematol Oncol

    (2003)
There are more references available in the full text version of this article.

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    Citation Excerpt :

    The occurrence of severe neutropenia following acute IM is an exceedingly uncommon event [4]. A mild transient decline in neutrophils is common in the first weeks following acute infection however the more dramatic and the uncommon profound decline in granulocytes (neutrophils, eosinophils, basophils) is typically not appreciated until 2–6 weeks following acute IM symptom onset [4-6]. Following a period of improvement from the primary IM symptoms, fever, malaise, and often a recurrence of lymphadenopathy develop similar to the acute stages of infection occur.

  • Atypical manifestations of Epstein-Barr virus in children: A diagnostic challenge

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    The hypothesis of maturation arrest of myeloid cells has been suggested because mature myeloid cells disappear in the patient's bone marrow. Anti-human neutrophil antigen-1a (anti-HNA-1a) and anti-HNA-1b antibodies are both associated with the pathophysiology in agranulocytosis after IM, although it is unknown whether anti-neutrophil antibodies are produced in EBV-infected B cells.15 Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disease caused by a dysfunction of cytotoxic T cells and NK cells.16

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