Síndrome hemofagocítico asociado a síndrome de Richter. Estudio de un caso autópsico y revisión de la literatura Haemophagocytic syndrome associated to Richter's Syndrome. Study of an autopsy case and review of the literature

SUMMARY Introduction: The haemophagocytic syndrome (HS) is a rare disease of multifactorial aetiology, which has been described in its secondary forms associated to T-cell lymphomas, drug induced immunsupression, concretely with Fludararabine, and in other cases to virus infection, especially with the Epstein-Barr-Virus (EBV). The evolu- tion of chronic lymphatic leukaemia (CLL) to more aggres- sive lymphomas is not unusual and it is known as Richter's Syndrome (RS), also occasionally associated to EBV. Mate- rial and methods: Autopsy of male, 63 years old, with diagnosis of CLL, stable for years, relapsing, and after various cycles of chemotherapy develops a medullar insuf- ficiency which leads to death after multiorganic failure. Results: The macroscopic study reveals non-specific signs. The histological study reveals a HS associated to a complex lymphoproliferative syndrome with two patterns predomi- nating in lymph nodes and spleen, the less differentiated formed of large cells with T-immunophenotype and in other organs persistence of the lymphoid pattern of small B-cells in accordance with the primitive diagnosis. The immunohis- tochemical study does not reveal positivity for EBV in tumour-cells. Conclusions: The association of HS to RS is not described in the revised literature. Being negative the EBV, other possible aetiologies of the HS are the T-cell lymphoma developed on the initial CLL which justifies the final worsening without drug-response or the application of Fludarabine after which the patient develops the final medu- llar insufficiency associated to the HS.