Identical twin brothers concordant for Langerhans? cell histiocytosis and discordant for Epstein-Barr virus-associated haemophagocytic syndrome

Abstract

the occurrence of haemophagocytic syndrome and Langerhans' cell histiocytosis, although genetically based, can be triggered by environmental agents and viruses, in particular Epstein-Barr virus.

The Adverse Prognostic Hallmarks in Identical Twins with Langerhans Cell Histiocytosis: A Clinical Report and Literature Review

“…All the identical-twin patients with LCH were children 0-4 years old with the exception of one adult pair. Among the 17 identical sets of twins with LCH including Twin 1 and Twin 2 reported here, 11 pairs were males (64.71%) and 6 were females (33.29%) (Bierman 1966;Caldarini 1966;Juberg et al 1970;Jakobson et al 1987;Kuwabara and Takahashi 1990;Katz et al 1991;Enjolras et al 1992;Halton et al 1992;Kanold et al 1994;Mader et al 1996;Dufour et al 2001;Patel and Renge 2001;Chen et al 2004;Imai et al 2010;Fernandez-Torres et al 2011). The most frequently affected organs were bone and skin, followed by lymph node and ear, with the next being bone marrow, liver, spleen and lung.…”

“…The data revealed that all of the 5 identical-twin pair cases with LCH (a total of 9 patients, only one child of one pair in the 5 pairs was involved) died as a result of disseminated and infiltrated lesions to the bone marrow, spleen and liver with symptoms of fever and hepatosplenomegaly (Juberg et al 1970;Katz et al 1991;Enjolras et al 1992;Halton et al 1992;Fernandez-Torres et al 2011). Conversely, the other patients, who were not infiltrated to these risk organs, had a better prognosis, even though those patients' multiple bone, the skin, lymph nodes, ear and lung were infiltrated by pathologic Langerhans cells (Lightwood and Tizard 1954;Bierman 1966;Caldarini 1966;Jakobson et al 1987;Kuwabara and Takahashi 1990;Kanold et al 1994;Mader et al 1996;Dufour et al 2001;Patel and Renge 2001;Chen et al 2004;Imai et al 2010). The twin sisters reported here currently undergoing treatment at our hospital had not experienced dissemination to the risk organs to date and recovered well.…”

“…LCH in identical twins is very rare and the clinical characteristics are different. During the past 60 years, only 16 identicaltwin pair patients (16 literatures) with LCH have been reported in PubMed since Lightwood and Tizard (1954) first reported the brothers with LCH (Bierman 1966;Caldarini 1966;Juberg et al 1970;Jakobson et al 1987;Kuwabara and Takahashi 1990;Katz et al 1991;Enjolras et al 1992;Halton et al 1992;Kanold et al 1994;Mader et al 1996;Dufour et al 2001;Patel and Renge 2001;Chen et al 2004;Imai et al 2010;Fernandez-Torres et al 2011). A high (86%) concordance rate for LCH in twins is identical, but is not dizygotic (Arico and Danesino 2001).…”

Human Herpesvirus-6 DNA and Langerhans Cell Histiocytosis

“…LCH is an enigmatic histiocytic proliferative disease of unknown etiology; however, a possible etiologic link between viruses or vaccinations and LCH has been proposed, among other environmental agents (7,8). Epstein-Barr virus (EBV, HHV-4) is known as the etiologic agent of several malignancies, and herpes viruses are reported to cause persistent infections (9,10).…”

Excimer Laser as Adjuvant Therapy for Adult Cutaneous Langerhans Cell Histiocytosis

“…3,5 Although it has been suggested that viral etiologic factors may be a source of the LCH reactive process, studies have only rarely reported EpsteinBarr virus as a possible triggering factor. 8 Environmental pathogenetic factors have not been identified, except that an elevated incidence of smoking has been found in patients with pulmonary LCH.…”