[Anaplastic large cell lymphoma associated with hemophagocytic lymphohistiocytosis: a case report and review of the literature].

Malignancy-associated hemophagocytic syndrome has been reported mostly in adults, in children it is very rare. It can develop before or during treatment, in association with an infection or without a known triggering factor. Here we present a case report of a 14 years old boy diagnosed with HLH before recognition of anaplastic large cell lymphoma (ALCL). Because of progression of symptoms characteristic for HLH, therapy according to the HLH 2004 protocol was introduced. Finally, histopathological examination of a subsequently excised enlarged lymph node demonstrated lesions typical for ALCL. First-line therapy, according to protocol for this type of lymphoma, was introduced. Because of resistance to standard therapy, change of protocol was necessary. Finally the patients achieved complete remission. He was qualified for allogeneic stem cell transplantation. The patient died in peritransplantation period because of infection complications. Occurrence of HLH can cause diagnostic difficulties, because it often masks the underlying disease, especially when it is associated with infection. Therefore, each case of HLH requires accurate evaluation for malignancy. Poor outcome in patients with malignancy-associated HLH is emphasized in the literature. There is necessity of establishment of more effective therapy.