AVILA-AGUERO ML, CAMACHO-BADILLA K, CANAS-COTO A, FERNANDEZ W, ABDELNOUR A; Interscience Conference on Antimicrobial Agents and Chemotherapy (43rd: 2003: Chicago, Ill.).

Abstr Intersci Conf Antimicrob Agents Chemother Intersci Conf Antimicrob Agents Chemother. 2003 Sep 14-17; 43: abstract no. G-1556.

Hosp. Natl. Ninos, San Jose, Costa Rica.

BACKGROUND: Secondary HPS is a serious complication of a systemic inflammatory disorder thought to be caused by excessive activation of T lymphocytes and macrophages. Management of these cases continues to be difficult; steroids, and intravenous gamma-globulin (IVIgG) have been used as treatment. The aim of this study was to identify etiological agents, describe clinical course and outcome of HPS in our country. METHODS: This is a retrospective study of all patients (pt) who were discharged from the Costa Rican Natl Children's Hosp between Jan 1987-Mar 2003 with the diagnosis of SHPS. Medical charts were reviewed for: age, sex, etiology, clinical manifestations, diagnostic procedures, laboratory tests, associated medications and outcome. RESULTS: 30 pts were identified, 15 (50%) were males. Median (range) age was 1 years (0-10y). Mean (range) hospital stay was 18 (2-41) days. All pt had fever and splenomegaly; 21/30 (70%) pt had hepatomegaly. Etiology was identified in 21/30 (70%) pt; 19 were infection-associated; 2 associated connective tissue disorders and 9 were idiopathic. Organisms identified were 13 Epstein Barr Virus (EBV), 4 Cytomegalovirus, one Candida albicans and one Acinetobacter. Hemophagocytosis was seen in bone-marrow in all the pt; 22/30 (73%) pt had hipertrygliceredimia; in 24/30 (80%) pt hemoglobin < 9g/dL; 14/30 (47%) pt neutrophils <1000/mm[3]; 27/30 (90%) pt thrombocytopenia. 19/30 (63%) received high doses of methylprednisolone (MP); 7/30 (23%) received prednisone; all pt received IV antibiotics. Overall mortality was 46% (14/30). 6/13 (46%) pt with EBV died. 12/19 (63%) pt who received MP died. 4/6 (66%) pt who received IVIgG and MP died. CONCLUSION: Our results demonstrate the association of EBV infection and HPS. Use of steroids in high doses and IVIgG does not change the outcome. Currently, 46% of HPS cases have a poor outcome despite aggressive treatment. Prospective trials are necessary to define guidelines in the management of this disease.

Publication Types:

• Meeting Abstracts

Keywords:

• Bone Marrow
• Bone Marrow Examination
• Child
• Epstein-Barr Virus Infections
• Genes, Regulator
• Herpesvirus 4, Human
• Humans
• Infection
• Longitudinal Studies
• Lymphohistiocytosis, Hemophagocytic
• Male
• Retrospective Studies
• T-Lymphocytes
• genetics

Other ID:

• GWAIDS0026330

UI: 102265954

From Meeting Abstracts