title: Haemophagocytic lymphohistiocytosis in association with granular lymphocyte proliferative disorders in early childhood: characteristic bone marrow morphology.
abstract: Five paediatric cases of haemophagocytic lymphohistiocytosis (HLH) which showed proliferation of granular atypical lymphocytoid cells in bone marrow are reported. All cases were girls aged 8 months to 4 years who had marked hepatosplenomegaly. Marker analysis on peripheral blood mononuclear cells revealed an increase in the CD3+HLADR+ subset in three cases and the CD3- CD56+ subset in one case. An Epstein-Barr virus genome was detected in three cases, and monoclonality was confirmed in two cases. A characteristic morphology of large granular lymphocytes (LGL) was identified, with elongated bizarre features that resembled horsetail-, tadpole-, cucumber- or shooting star-type configurations on the bone marrow smear. Serum concentrations of soluble interleukin-2 receptor and interferon-gamma were elevated in all cases. All five cases required multi-agent chemotherapy which resulted in two complete remission, two partial remissions and one no response. Refinement of treatment is required for these paediatric GLPD cases which probably comprise a specific high-risk subgroup among secondary HLH patients which had previously escaped notice.
authors.affiliation: Division of Paediatrics, Children's Research Hospital, Kyoto Prefectural University of Medicine, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Imashuku
authors.forename: S
authors.initials: S
authors.lastname: Hibi
authors.forename: S
authors.initials: S
authors.lastname: Morinaga
authors.forename: K
authors.initials: K
authors.lastname: Takagi
authors.forename: J
authors.initials: J
authors.lastname: Chen
authors.forename: H
authors.initials: H
authors.lastname: Mugishima
authors.forename: T
authors.initials: T
authors.lastname: Ishii
authors.forename: M
authors.initials: M
authors.lastname: Sako
authors.forename: H
authors.initials: H
authors.lastname: Arakawa
authors.forename: M
authors.initials: M
authors.lastname: Kato
mesh_terms: D001855:Bone Marrow Diseases / Q000473:pathology*; D002455:Cell Division; D002675:Child, Preschool; D005260:Female; D006098:Granulocytes / Q000473:pathology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000473:pathology*; D006801:Humans; D007223:Infant; D008232:Lymphoproliferative Disorders / Q000473:pathology*