Hemophagocytic syndrome in renal transplant recipients: report of 17 cases and review of literature.
BACKGROUND
Hemophagocytic syndrome (HPS) combines febrile hepatosplenomegaly, pancytopenia, hypofibrinemia, and liver dysfunction. It is defined by bone marrow and organ infiltration by activated, nonmalignant macrophages phagocytizing blood cells. HPS is often caused by an infectious or neoplastic disease and has rarely been described in renal transplant recipients.


METHODS
We retrospectively analyzed 17 cases of HPS after cadaveric renal transplantation (13 men and 4 women, age 41+/-8 years). The median time between transplantation and hemophagocytosis was 52 days. Eleven patients (64%) had received antilymphocyte globulins during the 3 months before presentation.


RESULTS
Fever was present in all patients, and hepatosplenomegaly was present in 9 of 17 patients. Other nonspecific clinical findings included abdominal, neurologic, and respiratory symptoms. Laboratory tests showed anemia (hemoglobin 6.1+/-1.3 g/dL), thrombocytopenia (34,000+/-32,000/mm3), and leukopenia (1,700+/-1,400/mm3). Elevated liver enzymes were present in 12 of 17 patients, and cholestasis was present in 10 of 17 patients. Elevated triglycerides and ferritin were noted in 75% and 86% of cases, respectively. HPS was related to viral infection in nine patients (cytomegalovirus, Epstein-Barr virus, human herpesvirus 6, and human herpesvirus 8), bacterial infection in three patients (tuberculosis and Bartonella henselae), and other infections in two patients (toxoplasmosis and Pneumocystis carinii pneumoniae). Posttransplant lymphoproliferative disease was present in two patients. Despite large-spectrum anti-infectious treatment and dramatic tapering of immunosuppression, death occurred in eight patients (47%). Graft nephrectomy was performed in four of the nine surviving patients.


CONCLUSIONS
We report here the largest series of HPS after renal transplantation. This rare disease is usually secondary to herpes viridae infections, mostly cytomegalovirus and Epstein-Barr virus in severely immunocompromised patients. Despite aggressive treatment, the prognosis remains poor.
Karras
Alexandre
A
Service de Néphrologie et Transplantation Rénale, Hôpital Saint-Louis, Paris, France.
Thervet
Eric
E
Legendre
Christophe
C
D000328:Adult; D001853:Bone Marrow / Q000473:pathology; D004359:Drug Therapy, Combination; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000473:pathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D016030:Kidney Transplantation / Q000009:adverse effects* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012744:Sex Ratio
D007166:Immunosuppressive Agents