Successful treatment of recurrent malignancy-associated hemophagocytic lymphohistiocytosis with a modified HLH-94 immunochemotherapy and allogeneic stem cell transplantation

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.

Nahi, HarethKarolinska Institutet (author)
Karbach, Holger (author)
Klimkowska, Monika (author)
Hägglund, HansHematology Center Karolinska, Karolinska University Hospital Huddinge, Stockholm, Sweden (author)
Karolinska InstitutetHematology Center Karolinska, Karolinska University Hospital Huddinge, Stockholm, Sweden (creator_code:org_t)

In:Medical Oncology: Springer Science and Business Media LLC29:2, s. 1231-12361357-05601559-131X

By the author/editor: Machaczka, Macie ...; Nahi, Hareth; Karbach, Holger; Klimkowska, Moni ...; Hägglund, Hans

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