Familial hemophagocytic lymphohistiocytosis: How late can the onset be?

Haematologica

Volumn 86, Issue 5, 2001, Pages 499-503

  Allen, Michaela ^a   De Fusco, Carmela ^b   Legrand, Faezeh ^c   Clementi, Rita ^g   Conter, Valentino ^d   Danesino, Cesare ^g   Janka, Gritta ^e   Aricò, Maurizio ^a,f  

^a UNIVERSITY OF PAVIA   (Italy)

^b Ospedale Pausilipon   (Italy)

^c HÔPITAL ROBERT DEBRÉ   (France)

^d SAN GERARDO HOSPITAL   (Italy)

^e UNIVERSITY MEDICAL CENTER HAMBURG EPPENDORF   (Germany)

^f FONDAZIONE IRCCS POLICLINICO SAN MATTEO   (Italy)

^g NONE

Author keywords

Hemophagocytosis; Late onset; Lymphohistiocytosis; Perform

Indexed keywords

ADOLESCENT; ARTICLE; CASE REPORT; CHILD; FAMILY HEALTH; FEMALE; GENETICS; HISTIOCYTOSIS; HUMAN; MALE; ONSET AGE; RECESSIVE GENE;

ADOLESCENT; AGE OF ONSET; CHILD; FAMILY HEALTH; FEMALE; GENES, RECESSIVE; HISTIOCYTOSIS, NON-LANGERHANS-CELL; HUMANS; MALE;

EID: 0035345280     PISSN: 03906078     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (17)

1. Farquhar J, Claireaux A. Familial haemophagocytic reticulosis. Arch Dis Child 1952; 27:519-25.
2. Aricò M, Janka G, Fischer A, et al. Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL Study Group of the Histiocyte Society. Leukemia 1996; 10:197-203.
3. Henter Jl, Aricò M, Egeler RM, et al. HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. HLH study Group of the Histiocyte Society. Med Pediatr Oncol 1997; 28:342-7.
4. Jabado N, de Graeff-Meeder ER, Cavazzana-Calvo M, et al. Treatment of familial hemophagocytic lymphohistiocytosis with bone marrow transplantation from HLA genetically nonidentical donors. Blood 1997; 90:4743-8.
5. Henter Jl, Elinder G, Ost A. Diagnostic guidelines for hemophagocytic lymphohistiocytosis. The FHL Study Group of the Histiocyte Society. Semin Oncol 1991; 18: 29-33.
6. Ohadi M, Lalloz MR, Sham P, et al. Localization of a gene for familial hemophagocytic lymphohistiocytosis at chromosome 9q21.3-22 by homozygosity mapping. Am J Hum Genet 1999; 64:165-71.
7. Dufourcq-Lagelouse R, Jabado N, Le Deist F, et al. Linkage of familial hemophagocytic lymphohistiocytosis to 10q21-22 and evidence for heterogeneity. Am J Hum Genet 1999; 64:172-9.
8. Stepp SE, Dufourcq-Lagelouse R, Le Deist F, et al. Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Science 1999; 286:1957-9.
9. Clementi R, zur Stadt U, Janka G, et al. Six novel mutations in the PRF1 gene in children with hemophagocytic lymphohistiocytosis. J Med Genet, (in press).
10. Graham GE, Graham LM, Bridge PJ, et al. Further evidence for genetic heterogeneity in familial hemophagocytic lymphohistiocytosis (FHLH). Pediatr Res 2000; 48:227-32.
11. zur Stadt U, Kabisch H, Janka G, Schneider M. Mutational analysis of the perform gene and NK-cell function in hemophagocytic lymphohistiocytosis. Proceedings of the Histiocyte Society 16th Annual Meeting. Amsterdam 2000, September 29-October 2.
12. Mozziconacci P, Nezelof C, Mozziconacci P, et al. La lymphohistiocytose familiare. Arch Fr Pediatr 1965; 22:385-408.
13. Perez N, Virelizier JL, Arenzana-Seisdedos F, Fischer A, Griscelli C. Impaired natural killer activity in lymphohistiocytosis syndrome. J Pediatr 1984; 104:569-73.
14. Aricò M, Nespoli L, Maccario R, et al. Natural cytotoxicity impairment in familial haemophagocytic lymphohistiocytosis. Arch Dis Child 1988; 63:292-6.
15. Aricò M, Fiorani O, Lis A, et al. Incidence and geographic distribution of HLH in Italy. Proceedings of the Annual Meeting of the Histiocyte Society, Amsterdam 2000, September 29- October 2, 42.
16. Blanche S, Caniglia M, Girault D, Landman J, Griscelli C, Fischer A. Treatment of hemophagocytic lymphohistiocytosis with chemotherapy and bone marrow transplantation: a single-center study of 22 cases. Blood 1991; 78:51-4.
17. Sullivan KE, Delaat CA, Douglas SD, Filipovich AH. Defective natural killer cell function in patients with hemophagocytic lymphohistiocytosis and in first degree relatives. Pediatr Res 1998; 44:465-8.