Thirty-Eight Children with Langerhans Cell Histiocytosis / 实用儿科临床杂志

ABSTRACT

Objective To explore the clinical and pathologic features of Langerhans cell histiocytosis(LCH).Method Thirty-eight cases of LCH from both in hospital and outpatient department in dermatology and pediatry department from Jan.1991 to Jun.2006 were analyzed about the features of clinic,pathology and immunohistochemistry.Results The mean age of onset was 2.18 years old.The male /female ratio was 1.71.Skin lesions occurred in 78.9% of the patients.Among them,68.5% were as the first manifestation.The eruptions mainly distributed on trunk,90% of them presented as hemorrhagic maculopapules,nevertheless,3.3% of the eruptions showed as crust with hilar depression,which was similar to pityriasis lichenoides etvarioliformis acuta.Fever,hepatomegalia and splenomegia occurred in patients at a rate of 60.5%,68.4%,55.3% respectively.Thirty-one point six percent of the patients had got lymphadenectasis,the neck and inguinal lymph nodes were the common site to be affected.Ossature involvement occurred in 31.6% of the patients,which 8.3% got multiple injuries,howe-ver,91.7% got a solitary bone involved.Skull was the main site to be injured,else were lumbar,humerus,hipbone,an so on.Respiratory tract,auditory canal,mucosa were also the sites involved in this disease,but the incidence rate was lower than 10%,respectively.The laboratory data showed that 81.3% of the patients were anaemia,60.7% with abnormal subgroup of T-cell,and 32.1% positive for EBV-IgG.The skin histopathology data of 26/30 cases revealed that lichenoid infiltrates of Langerhans cells confined to the upper dermis.Cytologic features were cells with abundant eosinophilic cytoplasms,and longitudinally grooved or reniform nuclei.Lymphocytes and a few eosinophils also could be seen.Four cases of thirty showed that the proliferative Langerhans cells were with pale cytoplasms,besides,there were numerous eosinophils,and sometimes a few multinucleate cells were scattered.The immunity test of 20 cases of thirty displayed that CD1a(+)S100(+)KP-1(-).Biopsy of lymphaden and tumor of the skull of the rest 8 patients were all diagnosed as eosinophilic granuloma through both hematoxylin and eosin-stained section and immune marks.Conclusions Multiple systems can be involved in LCH.Hemorrhagic maculopapules,fever and splenohepatomegalia are common presentations in this disease.The morphous of nucles of histiocytes is particular,and to diagnose definitely,both CD1a(+) and S-100(+) are needed.