Journal of Clinical Immunology

, Volume 6, Issue 5, pp 355–362 | Cite as

Immunological studies on histiocytosis X. I. Special reference to the chemotactic defect and the HLA antigen

  • Y. Tomooka
  • M. Torisu
  • S. Miyazaki
  • N. Goya
Original Articles


We treated a family with three children with histiocytosis X (H-X). The chemotactic response of the neutrophils in these three patients was depressed and the chemotactic response of the neutrophils of the mother was also depressed compared to that of normal age-matched controls. To elucidate the genetic factors, we examined HLA antigens in five members of this family. All five members had Aw24, B7, Cw7, and DR1. Immunological and genetic studies in an additional 32 patients with H-X were performed. The chemotactic response of 35 patients with H-X (154.9±58.4/HPF) was significantly depressed in comparison with that of 35 age-matched healthy controls (613.3±116.7/HPF). In addition, the value of chemiluminescence of 20 of 35 patients (20.5±6.6 mV) was also significantly depressed in comparison with that of 20 normal controls (45.3±11.4 mV). The frequencies of Bw61 (54.4%) and Cw7 (45.4%) in 33 patients with H-X were significantly increased in comparison with those of 250 normal healthy controls (20.4 and 18.0%, respectively). Studies of immunoglobulin levels and complement titers of patients with H-X showed no consistent abnormalities. We proposed that defects of polymorphonuclear function may lead to an increased susceptibility to bacterial infections in patients with this disorder.

Key words

Histiocytosis X chemotaxis chemiluminescence HLA antigens 


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  1. 1.
    Miller ME, Norman ME, Koblenzer PJ, Schonauer T: Clinical and experimental. A new familial defect of neutrophil movement. J Lab Clin Med 82:1–8, 1973Google Scholar
  2. 2.
    Hill HR, Quie PG: Raised serum-IgE levels and defective neutrophil chemotaxis in three children with eczema and recurrent bacterial infections. Lancet 1:183–187, 1974Google Scholar
  3. 3.
    Scott H, Moynahan EJ, Risdon RA, Harvey BAM, Soothill JF: Familial opsonization defect associated with fatal infantile dermatitis, infections, and histiocytosis. Arch Dis Child 50:311–317, 1975Google Scholar
  4. 4.
    Jacobs JC, Norman ME: A familial defect of neutrophil chemotaxis with asthma, eczema, and recurrent skin infections. Pediat Res 11:732–736, 1977Google Scholar
  5. 5.
    Leikin S, Puruganan G, Frankel A, Steerman R, Chandra R: Immunologic parameters in histiocytosis-X. Cancer 32:796–802, 1973Google Scholar
  6. 6.
    Sims DG: Histiocytosis X. Follow-up of 43 cases. Arch Dis Child 52:433–440, 1977Google Scholar
  7. 7.
    Nezelof C, Herbet FF, Sachot JC: Disseminated histiocytosis X. Analysis of prognostic factors based on a retrospective study of 50 cases. Cancer 44:1824–1838, 1979Google Scholar
  8. 8.
    Osband ME, Lipton JM, Lavin P, Vawter G, Greenberger JS, McCaffrey RP, Parkman R: Histiocytosis-X. Demonstration of abnormal immunity. T-cell histamine H2-receptor deficiency, and successful treatment with thymic extract. N Engl J Med 304:146–153, 1981Google Scholar
  9. 9.
    Nesbit ME, O'Leary M, Dehner LP, Ramsay NKC: The immune system and the histiocytosis syndromes. Am J Pediat Hematol Oncol 3:141–149, 1981Google Scholar
  10. 10.
    Böyum A: Isolation of leukocytes from human blood. A two-phase system for removal of red cells with methylcellulose as erythrocyte-aggregating agent. Scand J Clin Lab Invest 21 Suppl 97:1–29, 1968Google Scholar
  11. 11.
    Ward PA, Lepow IH, Newman LJ: Bacterial factors chemotactic for polymorphonuclear leukocytes. Am J Pathol 52:725–736, 1968Google Scholar
  12. 12.
    Ward PA, Cochrane CG, Müller-Eberhard HJ: The role of serum complement in chemotaxis of leukocytes in vitro. J Exp Med 122:327–346, 1965Google Scholar
  13. 13.
    Easmon CSF, Cole PJ, Williams AJ, Hastings M: The measurement of opsonic and phagocytic function by Luminol-dependent chemiluminescence. Immunology 41:67–74, 1980Google Scholar
  14. 14.
    Kabat EA: Experimental Immunochemistry, 2nd ed, MM Mayer (ed). Springfield, IL, Thomas, 1961Google Scholar
  15. 15.
    Nishioka K, Linscott WD: Components of guinea pig complement. 1. Separation of a serum fraction essential for immune hemolysis and immune adherence. J Exp Med 118:767–793, 1963Google Scholar
  16. 16.
    Mancini G, Carbonara AO, Heremans JF: Immunochemical quantitation of antigens by single radial immunodiffusion. Int J Immunochem 2:235–254, 1965Google Scholar
  17. 17.
    Terasaki PI, McClelland JD: Microdroplet assay of human serum cytotoxins. Nature 204:998–1000, 1964Google Scholar
  18. 18.
    Mowat AG, Baum J: Chemotaxis of polymorphonuclear leukocytes from patients with diabetes mellitus. N Engl J Med 284:621–627, 1971Google Scholar
  19. 19.
    Altman LC, Snyderman R, Blaese RM: Abnormalities of chemotactic lymphokine synthesis and mononuclear leukocyte chemotaxis in Wiskott-Aldrich syndrome. J Clin Invest 54:486–493, 1974Google Scholar
  20. 20.
    Boyden S: The chemotactic effect of mixture of antibody and antigen on polymorphonuclear leucocytes. J Exp Med 115:453–466, 1962Google Scholar
  21. 21.
    Claman HN, Suvatte V, Githens JH, Hathaway WE: Histiocytic reaction in dysgammaglobulinemia and congenital rubella. Pediatrics 46:89–96, 1970Google Scholar
  22. 22.
    Cederbaum SD, Niwayama G, Stiehm ER, Neerhout RC, Ammann AJ, Berman W: Combined immunodeficiency presenting as the Letterer-Siwe syndrome. J Pediat 85:466–471, 1974Google Scholar

Copyright information

© Plenum Publishing Corporation 1986

Authors and Affiliations

  • Y. Tomooka
    • 1
  • M. Torisu
    • 1
  • S. Miyazaki
    • 2
  • N. Goya
    • 3
  1. 1.Department of Pediatrics and Department of First SurgeryKyushu University School of MedicineFukuokaJapan
  2. 2.Department of PediatricsSaga Medical CollegeSagaJapan
  3. 3.Fukuoka Municipal Children's Hospital Medical CenterFukuokaJapan

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