title: Cutaneous hematologic disorders in children.
abstract: BACKGROUND
  To determine and list the clinical and pathological features of cutaneous hematologic diseases in childhood.
  
  
  METHODS
  We retrospectively analyzed the data for 51 patients up to 15 years of age, who presented with primary cutaneous hematologic disorders according to the WHO-EORTC classification, at Necker-Enfants Malades Hospital, Paris, France, over a 17-year period. The cases were classified into the following diagnostic categories: CD30+ T-cell lymphoproliferative disorders (24) all consisting of lymphomatoid papulosis (LyP, 24), lymphoblastic lymphoma (LL, 7), acute leukemias (AL, 7), mycosis fungoides (MF, 5), Epstein-Barr virus-related lymphoproliferative disorders (EBV-related LPD, 5), T/NK-cell lymphoma, nasal type (1), γ/δ T-cell lymphoma (1), and panniculitis-like T-cell lymphoma (1).
  
  
  RESULTS
  We encountered a majority of LyP. No secondary lymphoma was found in LyP patients with a median follow-up of 8 years. 29% and 80% of LyP and MF patients, respectively, presented with pityriasis lichenoides chronica (PLC) before onset of disease. Owing to a frequently misleading clinicopathological presentation, MF patients were first underdiagnosed. Clinicopathological features of LL and AL were highly stereotypical, leading to the diagnosis being suspected and confirmed more promptly. In the latter patients and in EBV-related LPD patients, skin lesions usually led to the discovery of systemic disease.
  
  
  CONCLUSIONS
  Distribution of cutaneous hematologic diseases seems to be different in adults and in children aged <15-year old. PLC was strongly correlated with MF and LyP. Physicians must be made aware of the stereotypical clinical presentations of LL and AL to allow prompt diagnosis and treatment.
authors: Department of Pathology, Hôpital Necker-Enfants Malades, AP-HP, Université René Descartes, Paris V, France.
authors: Olivia
authors: O
authors: Boccara
authors: Stéphane
authors: S
authors: Blanche
authors: Yves
authors: Y
authors: de Prost
authors: Nicole
authors: N
authors: Brousse
authors: Christine
authors: C
authors: Bodemer
authors: Sylvie
authors: S
authors: Fraitag
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006402:Hematologic Diseases / Q000145:classification / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012871:Skin Diseases / Q000145:classification / Q000150:complications* / Q000175:diagnosis*