title: Malignancy-associated hemophagocytic lymphohistiocytosis in adults: a retrospective population-based analysis from a single center.
abstract: A retrospective, population-based study was conducted to evaluate the incidence, clinical features, and outcome of malignancy-associated hemophagocytic lymphohistiocytosis (M-HLH) in adults. Between January 1996 and December 2009, eight out of 887 (0.9%) patients diagnosed with hematological malignancies developed aggressive M-HLH in an area inhabited by approximately 160,000 people. Thus the estimated annual incidence of M-HLH in adulthood was 0.36/100,000 individuals/year. The clinical course of M-HLH was aggressive in all patients. Six patients were treated with a modified HLH-94 protocol; three achieved remission (durable in one case) while the others did not respond and died within an average of 2.4 months (range 1.5-3.5) after M-HLH diagnosis. Infection complicating the course of M-HLH occurred in four (50%) patients, all of whom developed fulminant M-HLH and died. Although the small study population limits the results, the long observation period strengthens its value.
authors.affiliation: Hematology Center Karolinska, Karolinska University Hospital Huddinge, Stockholm, Sweden. maciej.machaczka@ki.se
authors.forename: Maciej
authors.initials: M
authors.lastname: Machaczka
authors.forename: Johan
authors.initials: J
authors.lastname: Vaktnäs
authors.forename: Monika
authors.initials: M
authors.lastname: Klimkowska
authors.forename: Hans
authors.initials: H
authors.lastname: Hägglund
mesh_terms: D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D005260:Female; D019337:Hematologic Neoplasms / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents