A boy with fever, lymphadenopathy, hepatosplenomegaly, and lymphocytosis

Allergy and Asthma Proceedings

Volumn 29, Issue 2, 2008, Pages 216-220

  Kaza, Ujwala ^a   Knight, Adina Kay ^a   Jeroudi, Majed ^b   Bocchini Jr , Joseph A ^c   Anga, Amal ^d   Bahna, Sami L ^a,d  

^a Section of Allergy and Immunology

^b Section of Hematology and Oncology

^c Department of Pediatrics

^d Louisiana State University School of Medicine Shreveport   (United States)

Author keywords

EBV; Hemophagocytosis; Hepatosplenomegaly; Lymphadenopathy; Lymphocytosis; Lymphoproliferative disease; Rituximab

Indexed keywords

CYCLOSPORIN; DEXAMETHASONE; ETOPOSIDE; MONOCLONAL ANTIBODY; RITUXIMAB;

ARTICLE; CASE REPORT; DIFFERENTIAL DIAGNOSIS; FEVER; HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; HEPATOMEGALY; HUMAN; LYMPHATIC SYSTEM DISEASE; LYMPHOCYTOSIS; MALE; PRESCHOOL CHILD; SPLENOMEGALY; VIRUS INFECTION;

ANTIBODIES, MONOCLONAL; CHILD, PRESCHOOL; CYCLOSPORINE; DEXAMETHASONE; DIAGNOSIS, DIFFERENTIAL; EPSTEIN-BARR VIRUS INFECTIONS; ETOPOSIDE; FEVER; HEPATOMEGALY; HUMANS; LYMPHATIC DISEASES; LYMPHOCYTOSIS; LYMPHOHISTIOCYTOSIS, HEMOPHAGOCYTIC; MALE; SPLENOMEGALY;

EID: 43149094893     PISSN: 10885412     EISSN: None     Source Type: Journal    
DOI: 10.2500/aap.2008.29.3103     Document Type: Article

References (13)

1. Janka G, and Schneider EM. Modern management of children with haemophagocytic lymphohistiocytosis. Br J Haematol 124: 4-14, 2004.
2. Milone MC, Tsai DE, Hodinka RL, et al. Treatment of primary Epstein-Barr virus infection in patients with X-linked lymphoproliferative disease using B-cell-directed therapy. Blood 105: 994-996, 2005.
3. Held G, Poschel V, and Pfreundschuh M. Rituximab for the treatment of diffuse large B-cell lymphomas. Expert Rev Anti-cancer Ther 6:1175-1186, 2006.
4. Janka G, and Zur Stadt U. Familial and acquired hemophagocytic lymphohistiocytosis. Hematology Am Soc Hematol Educ Program 00:82-88, 2005.
5. Arico M, Allen M, Brusa S, et al. Haemophagocytic lymphohistiocytosis: Proposal of a diagnostic algorithm based on perform expression. Br J Haematol 119:180-188, 2002.
6. Dupre L, Andolfi G, Tangye SG, et al. SAP controls the cytolytic activity of CD8+ T cells against EBV-infected cells. Blood 105: 4383-4389, 2005.
7. Stepp SE, Dufourcq-Lagelouse R, Le Deist F, et al. Perform gene defects in familial hemagophagocytic lymphohistiocytosis. Science 286:1957-1959, 1999.
8. Feldmann J, Callebaut I, Raposo G, et al. Munc13-4 is essential for cytolytic granules fusion and is mutated in a form of familial hemophagocytic lymphohistiocytosis (FHL3). Cell 115:461-473, 2003.
9. Imashuku S, Teramura T, Tauchi H, et al. Longitudinal follow-up of patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. Haematologica 89:183-188, 2004.
10. Tangye SG, Lazetic S, Woollatt E, et al. Cutting edge: Human 2B4, an activating NK cell receptor, recruits the protein tyrosine phosphatase SHP-2 and the adaptor signaling protein, SAP. J Immunol 162:6981-6985, 1999.
11. Nakajima H, and Colonna M. 2B4: An NK cell activating receptor with unique specificity and signal transduction mechanism. Human Immunology 61:39-43, 2000.
12. Sumegi J, Huang D, Lanyl A, et al. Correlation of mutations of the SH2D1A gene and Epstein-Barr virus infection with clinical phenotype and outcome in X-linked lymphoproliferative disease. Blood 96:3118-3125, 2000.
13. Imashuku S. Clinical features and treatment strategies of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. Crit Rev Oncol Hematol 44:259-272, 2002.