The Clinicopathological Characters Of Hydroa Vacciniforme-like Lymphoproliferative Disorder

BackgroundsHydroa vacciniforme-like lymphoproliferative disorder(HVLPD)is an Epstein-Barr virus(EBV)-associated disease usually occurs in children in East Asia and Latin America.HVLPD usually developes on children and young adolescents,but adults are not spared and in rare conditions the elders are involved.It is characterized by flare of vescular or papular lesions on sun-exposed area including face,auricle,V area of upper chest,dorsum of hand,including fever,wasting,lymphadenopathy,and hepatosplenomegaly.HVLPD usually represented a highly aggressive disease and most patients had fatal outcome in Latin Americans.MethodsWe collected 41 cases of HVLPD in our department between 2007 to 2017 and analyse clinical feathers,histopathological feathers,TCR gene rearrangement and follow-up results of these cases.Results1.Clinical features: 41 cases were enrolled with 28 children or adolescents,and 13 adults.All of the cases presented with papulovesicular lesions mainly distributed on sun-exposed areas.26 cases showed systemic symptoms.Follow-up data was available in 20 cases,16 cases were alive with persistent disease and 4 cases died of disease,including 3 adult patients.2.HE staining: Most cases showed dense lymphocytes infiltration mainly in the dermis and was associated with reticular degeneration,spongiosis or necrosis of epidermis,The lymphocytes usually showed perivascular dominant distribution with angiotropism and angiodestructive features.Cellular polymorphism was noticed only in 4 cases,while in most cases were not obvious.3.Immunohistochemical staining: 2 cases were CD4 positive,2 were CD8 positive,37 cases showed both positive for CD4 and CD8.All of the cases were positive for CD3,CD5,CD7,TIA-1 and were negative for CD20.7 cases were positive for CD30 and only 3 cases showed very focal or scattered positive staining of CD56.4.Molecular study: EBER was variably positive in all case?TCR gene rearrangement was successfully performed in 32 cases and 29 cases had monoclonal rearrangements.ConclusionsHVLPD in Chinese usually shows rather indolent behavior with only a small number of cases proceeding to aggressive phase,which is quite different from that of Latin Americans.We suggested conservative treatment for HVLPD and chemotherapy is not encouraged.The current challenge is how to identify patients with risk to evolve to aggressive phase and how to treat the patients in aggressive phase.