Revision of the diagnosis of T-zone lymphoma in the father of a patient with autoimmune lymphoproliferative syndrome type II

Autoimmune lymphoproliferative syndrome (ALPS) is a disease of childhood ch aracterized by typical clinical and laboratory findings. Here we describe a n adult patient presenting with lymph node enlargement and splenomegaly. Pa thological examination of an adenopathy supported the diagnosis of malignan t T-zone lymphoma. The patient was treated accordingly, 3 years later his c hild was diagnosed with ALPS. Therefore the diagnosis of the father's disea se was reconsidered. Review of the slides and functional tests led to the d iagnosis of ALPS in both father and son. ALPS should be considered as a pos sible differential diagnosis in adult patients presenting with rare types o f T-cell lymphomas.