title: Secondary histiocytic sarcoma may cause apparent persistence or recurrence of minimal residual disease in childhood acute lymphoblastic leukemia.
abstract: Histiocytic sarcoma (HS) is a rare disease with poor prognosis which may develop subsequent to acute lymphoblastic leukemia (ALL). Here we report two children treated within the AIEOP-BFM ALL 2009 trial: one patient succumbed to fulminant hemophagocytic lymphohistiocytosis triggered by HS during ALL maintenance therapy, the other patient had a smoldering course of HS for over 2 years, and subsequently died after allogeneic stem cell transplantation. In both cases, HS and ALL were clonally related and apparent return of minimal residual disease (MRD) was detected by qPCR in bone marrow. Thus, HS should be considered in ALL when MRD appears to persist or reappear.
authors.affiliation: Department of Pediatrics, University Medical Center Schleswig-Holstein, Campus Kiel, Germany.
authors.forename: Julia
authors.initials: J
authors.lastname: Alten
authors.affiliation: Department of Pathology, Lymph Node Registry Kiel, Institute of Hematopathology University Hospital Schleswig-Holstein, Campus Kiel, Germany.
authors.forename: Wolfram
authors.initials: W
authors.lastname: Klapper
authors.affiliation: Department of Pathology, Kiel Pediatric Tumor Registry, Institute of Pediatric Pathology, University Hospital Schleswig-Holstein, Campus Kiel, Germany.
authors.forename: Ivo
authors.initials: I
authors.lastname: Leuschner
authors.affiliation: Department of Pediatric Oncology/Hematology, Charit, é, University Medicine, Berlin, Germany.
authors.forename: Cornelia
authors.initials: C
authors.lastname: Eckert
authors.affiliation: Division of Pediatric Hematology and Oncology, Department of Pediatrics, Hannover Medical School, Hannover, Germany.
authors.forename: Rita
authors.initials: R
authors.lastname: Beier
authors.affiliation: Department of Pediatrics, Klinikum Lippe Detmold, Detmold, Germany.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Vallo
authors.affiliation: Department of Pediatrics, University Medical Center Schleswig-Holstein, Campus Kiel, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Krause
authors.affiliation: Department of Pediatrics, University Medical Center Schleswig-Holstein, Campus Kiel, Germany.
authors.forename: Alexander
authors.initials: A
authors.lastname: Claviez
authors.affiliation: Department of Pediatrics, University Medical Center Schleswig-Holstein, Campus Kiel, Germany.
authors.forename: Simon
authors.initials: S
authors.lastname: Vieth
authors.affiliation: Department of Pediatrics, University Medical Center Schleswig-Holstein, Campus Kiel, Germany.
authors.forename: Kirsten
authors.initials: K
authors.lastname: Bleckmann
authors.affiliation: Department of Pediatrics, University Medical Center Schleswig-Holstein, Campus Kiel, Germany.
authors.forename: Anja
authors.initials: A
authors.lastname: Möricke
authors.affiliation: Department of Pediatrics, University Medical Center Schleswig-Holstein, Campus Kiel, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Schrappe
authors.affiliation: Department of Pediatrics, University Medical Center Schleswig-Holstein, Campus Kiel, Germany.
authors.forename: Gunnar
authors.initials: G
authors.lastname: Cario
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D054338:Cell Transdifferentiation; D002999:Clone Cells / Q000473:pathology; D019008:Drug Resistance, Neoplasm; D017809:Fatal Outcome; D015329:Gene Rearrangement, T-Lymphocyte; D019942:Genes, p16; D018380:Hematopoietic Stem Cell Transplantation; D054747:Histiocytic Sarcoma / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology; D014411:Neoplastic Stem Cells / Q000473:pathology; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D012008:Recurrence
keywords: acute lymphoblastic leukemia; histiocytic sarcoma; minimal residual disease; molecular MRD reappearance