title: Successful treatment of recurrent malignancy-associated hemophagocytic lymphohistiocytosis with a modified HLH-94 immunochemotherapy and allogeneic stem cell transplantation.
abstract: Acquired hemophagocytic lymphohistiocytosis (HLH) triggered by a known or still to be recognized malignancy is a life-threatening hyperinflammatory syndrome due to massive cytokine release from activated lymphocytes and macrophages. Malignancy-associated HLH (M-HLH) often impedes adequate treatment of malignancy and has the worst outcome compared with any other form of HLH. The incidence of M-HLH is unknown, and there are no published treatment recommendations addressed to this HLH form. Here, we report the case of a young woman with recurrent ALK1-positive anaplastic large T-cell lymphoma and M-HLH successfully treated with a modified HLH-94 protocol, allogeneic stem cell transplantation (alloSCT) and donor lymphocyte infusion (DLI). More than 3 years after DLI, the patient is alive, in complete remission from her malignancy and HLH-free, although suffering from extensive chronic graft-versus-host disease. AlloSCT and, if needed, DLI performed to consolidate remission of malignancy and HLH may have a curative impact on both entities. We propose that when discussing possible treatment options for patients with M-HLH, alloSCT should be considered in eligible individuals.
authors.affiliation: Hematology Center Karolinska, Karolinska University Hospital Huddinge, 141 86 Stockholm, Sweden. maciej.machaczka@ki.se
authors.forename: Maciej
authors.initials: M
authors.lastname: Machaczka
authors.forename: Hareth
authors.initials: H
authors.lastname: Nahi
authors.forename: Holger
authors.initials: H
authors.lastname: Karbach
authors.forename: Monika
authors.initials: M
authors.lastname: Klimkowska
authors.forename: Hans
authors.initials: H
authors.lastname: Hägglund
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D003561:Cytarabine / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006086:Graft vs Host Disease / Q000517:prevention & control; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D017710:Lymphocyte Transfusion; D051359:Lymphohistiocytosis, Hemophagocytic / Q000276:immunology / Q000628:therapy*; D017728:Lymphoma, Large-Cell, Anaplastic / Q000276:immunology / Q000628:therapy*; D008942:Mitoxantrone / Q000008:administration & dosage; D009364:Neoplasm Recurrence, Local / Q000276:immunology / Q000628:therapy*; D011379:Prognosis; D012074:Remission Induction; D033581:Stem Cell Transplantation; D014184:Transplantation, Homologous; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents; D003561:Cytarabine; D005047:Etoposide; D003907:Dexamethasone; D008942:Mitoxantrone