Reactive haemophagocytic syndrome in 58 HIV-1-infected patients: clinical features, underlying diseases and prognosis

• Published in: AIDS (London) Vol. 24; no. 9; pp. 1299 - 1306
• Main Authors: FARDET, Laurence, LAMBOTTE, Olivier, MOLINA, Jean-Michel, MARTINEZ, Valérie, MEYNARD, Jean-Luc, KAMOUH, Wassim, GALICIER, Lionel, MARZAC, Christophe, DE LABARTHE, Adrienne, CABANE, Jean, LEBBE, Céleste, COPPO, Paul
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins 01.06.2010
• Subjects: Adult; Age; Aged; Aged, 80 and over; AIDS-Related Opportunistic Infections - mortality; AIDS-Related Opportunistic Infections - virology; AIDS/HIV; Antiretroviral Therapy, Highly Active; Aspartate aminotransferase; Biological and medical sciences; CD4 antigen; CD4 Lymphocyte Count; CD8 antigen; Cell number; Cytomegalovirus; Data processing; Demography; Female; France; Hematologic and hematopoietic diseases; highly active antiretroviral therapy; HIV Infections - complications; HIV Infections - drug therapy; HIV Infections - mortality; HIV-1 - immunology; Hodgkin's disease; Human immunodeficiency virus 1; Human viral diseases; Humans; Immunodeficiencies; Immunodeficiencies. Immunoglobulinopathies; Immunopathology; Infection; Infectious diseases; Kaplan-Meier Estimate; L-Lactate dehydrogenase; Lymphohistiocytosis, Hemophagocytic - complications; Lymphohistiocytosis, Hemophagocytic - mortality; Lymphohistiocytosis, Hemophagocytic - virology; Male; Malignancy; Medical sciences; Middle Aged; Mycobacterium; Other diseases. Hematologic involvement in other diseases; Prognosis; Retrospective Studies; RNA; Splenomegaly; Survival; Tuberculosis; Viral diseases; Viral diseases of the lymphoid tissue and the blood. Aids; Young Adult; France; Immunopathology; Prognosis; Retroviridae; haemophagocytic syndrome; AIDS; Hemopathy; Histiocytosis; Immune deficiency; Lentivirus; Infection; Virus; Symptomatology; Hemophagocytic syndrome; HIV; Viral disease; haemophagocytosis; Human immunodeficiency virus
• ISSN: 0269-9370; 1473-5571
• DOI: 10.1097/qad.0b013e328339e55b

To describe features of reactive haemophagocytic syndrome (RHS) in HIV-1-infected adult patients. To compare characteristics of patients with malignancy-associated RHS and infection-associated RHS. Retrospective study in three departments of Infectious Diseases/Internal Medicine at three French tertiary centres. Medical charts of HIV-1-infected adult patients and RHS seen between January 2006 and December 2007 were reviewed. Demographic, clinical and laboratory data obtained at the time of RHS episode were compared between patients with malignancy-associated RHS and infection-associated RHS using non-parametric tests. The overall survival was assessed using the Kaplan-Meier method. Fifty-eight HIV-1-infected patients were diagnosed with RHS [certain RHS n = 43, possible RHS n = 15, median (range) age 42 (23-85) years, men 76%]. At time of RHS, the median duration of HIV infection was 4 (0-22) years and 57% received HAART. The median CD4 lymphocyte count was 91 (2-387)/microl and 35% of patients had a plasma HIV-1 RNA less than 50 copies/ml. Underlying haemopathy/malignancy (Hodgkin lymphoma n = 10) or infection (tuberculosis n = 9, cytomegalovirus infection n = 5) were evidenced for 31 and 23 patients, respectively. Patients with haemopathy/malignancy-associated RHS presented more frequently with splenomegaly (97 vs. 70%, P < 0.01), lower aspartate aminotransferase (36 vs. 84 UI/l, P < 0.01) and lactate dehydrogenase (530 vs. 911 UI/l, P < 0.01) levels and CD8 cell count (234 vs. 588/microl, P < 0.01). Eighteen (31%) patients died. The overall survival was not statistically different between the two groups (P = 0.68). In the HAART era, RHS is frequently associated with underlying haemopathy/malignancy, especially Hodgkin lymphoma. The prognosis remains poor but seems, however, better than in the pre-HAART era.