Immunodeficiency to Epstein-Barr Virus in Chediak-Higashi Syndrome

  • Fernando Merino


In 1943 Bequez (4) described three patients of the same family affected by autosomal recessive process characterized by albinism, pale hair, lymphadenomegaly, hepatosplenomegaly, lympho-monocytosis, and thrombocytopenia (Figure 1). Death was due to bone marrow failure. Large intracytoplasmatic azurophilic granules in polymorphonuclear neutrophils and lymphocytes were seen. Based on the symptoms, “Downey-McKinley” cells, and a positive Paul Bunnell reaction in one case, Cesar postulated that infectious mononucleosis (IM) “an organism in the patient lacking host defenses, was the reason...for the bone marrow failure.”


Natural Killer Natural Killer Cell Infectious Mononucleosis Accelerate Phase Bone Marrow Failure 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Abo, T., Roder, J.C., Abo, W., Cooper, M.D., and Balch, C.M. Natural killer (HNK-1+) cells in Chediak-Higashi patients are present in normal numbers but are abnormal in function and morphology. J. Clin. Invest., 70: 193, 1982.PubMedCrossRefGoogle Scholar
  2. 2.
    Balsano, V., Arena, F., and Amato, G. Un caso di sindrome di Begues Cesar-Steinbrinck-Chediak Higashi. Minerva Pediat., 19:780, 1967.Google Scholar
  3. 3.
    Bedoya, V., Grimley, P.M., and Duque, O. Chediak-Higashi syndrome. Arch. Pathol., 88:340–349, 1969.PubMedGoogle Scholar
  4. 4.
    Bequez Cesar, A. Neutropenia cronica maligna familiar con granulaciones atipicas de lose leucocitos. Bol. Soc. Cubana Pediat., 15:900, 1943.Google Scholar
  5. 5.
    Bernard, J., Bessis, M., Seligman, N.N., Chassigneux, J., and Chome, J. Un cas de maladie de Chediak-Steinbrinck-Higashi. Presse Med., 68:563, 1960.PubMedGoogle Scholar
  6. 6.
    Blume, R.S., Glade, P.R., Gralnick, H.R., Chessin, L.N., Haase, A.T., and Wolff, S.M. The Chediak-Higashi syndrome: continuous suspension cultures derived from peripheral blood. Blood, 33:821, 1969.PubMedGoogle Scholar
  7. 7.
    Blume, R.S. and Wolff, S.M. The Chediak-Higashi syndrome: studies in four patients and a review of the literature. Medicine, 51:247, 1972.PubMedCrossRefGoogle Scholar
  8. 8.
    Boxer, L., Watanabe, A.M., Rister, M., Besch, H.R., Allen, J., and Baehner, R.L. Correction of leukocyte function in Chediak-Higashi syndrome by ascorbate. N. Engl. J. Med., 295:1041, 1976.PubMedCrossRefGoogle Scholar
  9. 9.
    Boxer, G.J., Holmsen, H., Robkin, L., Bang, N.U., Boxer, L.A. and Baehner, R.L. Abnormal platelet function in Chediak-Higashi syndrome. Brit. J. Hemaet., 35: 521, 1977.CrossRefGoogle Scholar
  10. 10.
    Boxer, L.A., Rister, M., Allen, J., and Baehner, R.L. Improvement of Chediak-Higashi leukocyte function by cyclic guanosine monophosphate. Blood, 49: 9, 1977.PubMedGoogle Scholar
  11. 11.
    Boxer, L.A., Allen, J.M., Watanabe, A.M., Besch, H.R., and Baehner, R.L. Role of microtubules in granulocyte adherence. Blood, 51:1045, 1978.PubMedGoogle Scholar
  12. 12.
    Boxer, L.A., Albertini, D.F., Baehner, R.L., and Oliver, J. M. Impaired microtubule assembly and polymorphonuclear leucocyte function in the Chediak-Higashi syndrome correctable by ascorbic acid. Brit. J. Hemaet., 43:207, 1979.CrossRefGoogle Scholar
  13. 13.
    Chediak, M. Novelle anomalie leucocytaire de caractere constitutionnel et familial. Rev. Hemat., 7: 362, 1952.PubMedGoogle Scholar
  14. 14.
    Clawson C.C., White, J.G., and Repine, J.E. The Chediak- Higashi syndrome. Evidence that defective leukotaxis is primarly due to an impediment by giant granules. Am. J. Pathol., 92:745, 1978.PubMedGoogle Scholar
  15. 15.
    Clawson, C.C., Repine, J.E., and White, J.G. The Chediak-Higashi syndrome. Quantitation of a deficiency in maximal bactericidal capacity. Am. J. Pathol., 94:539, 1979.PubMedGoogle Scholar
  16. 16.
    Costa, J.L., Fauci, A.S., and Wolff, S.M. A platelet abnormality in the Chediak-Higashi syndrome of man. Blood, 48:517, 1976.PubMedGoogle Scholar
  17. 17.
    De Bastos, O. and Resende Barros, O. Sindrome de Bequez Cesar-Steinbrinck-Chediak-Higashi. Sangre, 5:367, 1960.Google Scholar
  18. 18.
    De Beer, H.A., Anderson, R., and Findlay, G.H. Chediak-Higashi syndrome in a ‘black’ child. S. Afr. Med. J., 60:108, 1981.PubMedGoogle Scholar
  19. 19.
    Dent, P.B., Fish, L.A., White, J.G., and Good, R.A. Chediak-Higashi syndrome. Observations on the nature of the associated malignancy. Lab. Invest., 15:1634, 1966.PubMedGoogle Scholar
  20. 20.
    Deprez, P., Laurent, R., Griscelli, C., Buriot, D., and Agache, P. La maladie de Chediak-Higashi. A propos d’une nouvelle observation. Ann. Dermatol. Venereol., 105:841, 1978.PubMedGoogle Scholar
  21. 21.
    Dondo Lescano, F., Ferreyra, M.E., and Seoane, M. Enfermedad de Chediak-Higashi. Presentacion de dos casos. Prensa Med. Arg., 56:127, 1969.Google Scholar
  22. 22.
    Donohue, W.L. and Bain, H.W. Chediak-Higashi syndrome. A lethal familial disease with anomalous inclusions in the leukocytes and constitution stigmata: report of a case with necropsy. Pediatrics, 20:416, 1957.PubMedGoogle Scholar
  23. 23.
    Douglas, S.D., Blume, R.S., Glade, P.R., Chessin, L.N., and Wolff, S.M. Fine structure of continuous long term lymphoid cell cultures from a Chediak-Higashi patient and heterozygote. Lab. Invest., 21:225, 1969.PubMedGoogle Scholar
  24. 24.
    Efrati, P. and Jonas, W. Chediak’s anomaly of leukocytes in malignant lymphoma associated with leukemic manifestations: case report with necropsy. Blood, 13:1063, 1958.PubMedGoogle Scholar
  25. 25.
    Gallin, J.I., Elin, R.J., Hubert, R.T., Fauci, A.S., Kaliner, M.A., and Wolff, S.M. Efficacy of ascorbic acid in Chediak-Higashi syndrome (CHS): studies in humans and mice. Blood, 53:226, 1979.PubMedGoogle Scholar
  26. 26.
    Gillon, J.R., Pease, G.L., and Mills, S.D. Chediak-Higashi anomaly of the leukocytes: report of a case. Proc. Mayo Clin., 35:635, 1960.Google Scholar
  27. 27.
    Haak, R.A., Ingrahara, L.M., Baehner, R.L., and Boxer, L.A. Membrane fluidity in human and mouse Chediak-Higashi leukocytes. J. Clin. Invest., 64:138, 1979.PubMedCrossRefGoogle Scholar
  28. 28.
    Haliotis, T., Roder, J., Klein, M., Ortaldo, J., Fauci, A.S., and Herberman, R.B. Chediak-Higashi gene in humans. I. Impairment of natural-killer function. J. Exp. Med., 151:1039, 1980.PubMedCrossRefGoogle Scholar
  29. 29.
    Hansson, H., Linell, F., Nilsson, L.R., Soderhjelm, L., and Undritz, U. Die Chediak-Steinbrinck-anomalie resp. erblich-konstitutionelle risengranulation (granulagiganten) der leukozyyten in Nordschweden. Folia Haematol., 3: 152, 1959.Google Scholar
  30. 30.
    Herberman, R.B. Natural killer cells. Hosp. Pract., 17:9, 1982.Google Scholar
  31. 31.
    Higashi, O. Congenital gigantism of peroxidase granules. The first case ever reported of qualitative abnormity of peroxidase. Tohuku J. Exp. Med., 59:315, 1954.CrossRefGoogle Scholar
  32. 32.
    Hinds, K. and Dane, B.S. Microtubular defect in Chediak-Higashi syndrome. Lancet, ii: 146, 1976.CrossRefGoogle Scholar
  33. 33.
    Ingraham, L.M., Burns, C.P., Boxer, L.A., Baehner, R.L., and Haak, R.A. Fluidity properties and lipid composition of erythrocyte membranes in Chediak-Higashi syndrome. J. Cell Biol., 89:510, 1981.PubMedCrossRefGoogle Scholar
  34. 34.
    Iseki, S., Ebina, T., and Ishida, N. Mode of paracrystal formation in Chediak-Higashi fibroblasts. Lancet, i:1409, 1979.CrossRefGoogle Scholar
  35. 35.
    Ito, J., Tokumaru, M., and Okazaki, T. Chediak-Higashi syndrome: report of case with autopsy and electron microscopic studies. Acta Path. Jap., 22:755, 1972.PubMedGoogle Scholar
  36. 36.
    Janini, P., Pinto Lima, X., Franca, H.H., Tricta, F., and Tannos, D. Sobre tres casos de anoraalia leucocitaria identica a la descrita pos Bequez Cesar, Steinbrinck, Chediak, Higashi y Sato. Sangre, 8:138, 1963.Google Scholar
  37. 37.
    Kearny, P.J., Swift, P.G.F., Brow, N.J., and Savage, D.C.L. The Chediak-Higashi syndrome in a boy of Irish parents. J. Irish Med. Assoc, 72:22, 1979.Google Scholar
  38. 38.
    Khan, A., Hill, J.M., Loeb, E., MacLellan, A., and Hill, N.A. Management of Chediak-Higashi syndrome with transfer factor. Am. J. Dis. Child., 126: 797, 1973.PubMedGoogle Scholar
  39. 39.
    Kimball, H.R., Ford, G.H., and Wolff, S.M. Lysosomal enzymes in normal and Chediak-Higashi blood leukocytes. J. Lab. Clin. Med., 86:616, 1975.PubMedGoogle Scholar
  40. 40.
    Klein, G. and Purtilo, D. Symposium on Epstein-Barr virus-induced diseases in immunodeficient patients. Cancer Res., 41:4209, 1981.PubMedGoogle Scholar
  41. 41.
    Klein, G. Lymphoma development in mice and humans: diversity of initiation is followed by convergent cytogenetic evolution. Proc. Natl. Acad. Sci. USA, 76:2442, 1979.PubMedCrossRefGoogle Scholar
  42. 42.
    Klein, M., Roder, J., Haliotis, T., Korec, S., Jett, J.R., Herberman, R.B., Katz, P., and Fauci, A.S. Chediak-Higashi gene in humans. II. The selectivity of the defect in natural-killer and antibody-dependent cell-mediated cytotoxicity function. J. Exp. Med., 151:1049, 1980.PubMedCrossRefGoogle Scholar
  43. 43.
    Kritzler, R.A., Terner, J.Y., Lindenbaun, J., Magidson, J., Williams, R., Preisig, R., and Phillips, G. Chediak-Higashi syndrome. Cytologic and serum lipid observations in a case and family. Am. J. Med., 36:583, 1964.PubMedCrossRefGoogle Scholar
  44. 44.
    Krueger, G.R.F., Bedoya, V., and Grimley, P.M. Lymphoreticular tissue lesions in Steinbrinck-Chediak-Higashi syndrome. Virchows Arch. Abt. A. Path. Anat., 353:273, 1971.CrossRefGoogle Scholar
  45. 45.
    Maggi, R., Gutierrez, E., Penalber, J., and DiMenna, A., Roccotagliata, M., Matera, F., Etchegaray, E., and Millan, J. Sindrome de Bequez Cesar-Chediak-Higashi. Arch. Arg. Pediat., 48:323, 1957.Google Scholar
  46. 46.
    Merino, F., Klein, G., Henle, W., Ramirez-Duque, P., Forsgren, M., and Amesty, C. Elevated antibody titers to Epstein-Barr virus and low natural killer cell activity in patients with Chediak-Higashi syndrome. Clin. Immunol. Immunopathol., in press.Google Scholar
  47. 47.
    Merino, F., Lipscomb, J., Amesty, C., and Purtilo, D. Lymphocyte subsets in Chediak-Higashi syndrome patients. Immunol. Letters, 6:81, 1983.CrossRefGoogle Scholar
  48. 48.
    Moran, T.J. and Estevez, J.M. Chediak-Higashi disease. Morphological studies of a patient and her family. Arch. Pathol., 88:329, 1969.PubMedGoogle Scholar
  49. 49.
    Nath, J., Flavin, M., and Gallin, J.I. Tubulin tyrosylation in normal and Chediak-Higashi syndrome neutrophils. J. Cell. Biol., 87:256a, 1980.Google Scholar
  50. 50.
    Nishi, Y., Sawano, K., Kagosaki, Y., Tanaka, Y., Kobayashi, Y., and Usui, T. High plasma, erythrocyte, lymphocytic and granulocyte zinc concentrations in a patient with the Chediak-Higashi syndrome. Eur. J. Pediatr., 134:9, 1980.PubMedCrossRefGoogle Scholar
  51. 51.
    Oliver, J.M. Impaired microtubule function correctable by cyclic GMP and cholinergic agonists in the Chediak-Higashi syndrome. Am. J. Pathol., 85:395, 1976.PubMedGoogle Scholar
  52. 52.
    Oliver, J.M. and Zurier, R.B. Correction of characteristic abnormalities of microtubule function and granule morphology in Chediak-Higashi syndrome with colinergic agonists. J. Clin. Invest., 57:1239, 1976.PubMedCrossRefGoogle Scholar
  53. 53.
    Ostlund, R.E., Tucker, R.W., Leung, J.T., Okun, N., and Williamson, J.R. The cytoskeleton in Chediak-Higashi syndrome fibroblasts. Blood, 56: 806, 1980.PubMedGoogle Scholar
  54. 54.
    Pachioli, R., Caramia, G., and DiBattista, C. L’anomalia di Beguez-Steinbrinck-Chediak-Higashi. Rassegna sintetica e descrizione di un caso. Minerva Pediatr., 22:368, 1970.PubMedGoogle Scholar
  55. 55.
    Padgett, G.A. The Chediak-Higashi syndrome. Adv. Vet. Sci., 12:239, 1968.Google Scholar
  56. 56.
    Padgett, G.A., Reiquara, C.W., Gorham, J.R., Henson, J.B., and O’Mary, C.C. Comparative studies of the Chediak-Higashi syndrome. Pathology Am. J. Pathol., 51:553, 1967.Google Scholar
  57. 57.
    Padgett, G.A., Reiquam, C.W., Henson, J.B., and Gorham, J.R. Comparative studies of susceptibility to infection in the Chediak-Higashi syndrome. J. Path. Bact., 95:509, 1968.PubMedCrossRefGoogle Scholar
  58. 58.
    Page, A.R., Berendez, H., Warner, J., and Good, R.A. The Chediak-Higashi syndrome. Blood, 20:330, 1962.PubMedGoogle Scholar
  59. 59.
    Parmley, R.T., Poon, M.-C., Crist, W.M., and Malluh, A. Giant platelet granules in a child with the Chediak-Higashi syndrome. Am. J. Hematol., 6:51, 1979.PubMedCrossRefGoogle Scholar
  60. 60.
    Pierini, D.D., Turro, D., Torales, E., and Piantanida, J.J. Chediak-Higashi disease. Arch. Arg. Dermatol., 19:143, 1969.Google Scholar
  61. 61.
    Prchal, J.T., Crist, W.M., Malluh, A., Tauxe, W.N. and Carroll, A.J. A new glucose-6-phosphate dehydrogenase deficient variant in a patient with Chediak-Higashi syndrome. Blood, 56: 476 1980.PubMedGoogle Scholar
  62. 62.
    Prieur, A.-M., Griscelli, C., and Gaugillard, F. Lymphotoxin (LT) production by lymphocytes from children with primary immunodeficiency disease. Clin. Immunol. Immunopathol., 10:468, 1978.PubMedCrossRefGoogle Scholar
  63. 63.
    Prieur, D.J. and Collier, L.L. Animal model: Chediak-Higashi syndrome of animals. Am. J. Pathol., 90:533, 1978.PubMedGoogle Scholar
  64. 64.
    Purtilo, D.T. Immunopathology of infectious mononucleosis and other complications of Epstein-Barr virus infections. Pathol. Ann., 15:253, 1980.Google Scholar
  65. 65.
    Ramirez-Duque, P., Arends, T., and Merino, F. Chediak-Higashi syndrome: description of a cluster in a Venezuelan Andean isolated region. J. Medicine, in press.Google Scholar
  66. 66.
    Ratto, L.-A., Hliba, E., Yatorno, C.-E., and Rupil, A. Sindrome de Chediak-Higashi-Steinbrinck. Estudio hema-tologico y ultraestructural. Sangre, 22:263, 1977.PubMedGoogle Scholar
  67. 67.
    Rausch, P.G., Pryzwansky, K.B., and Spitnagel, J.K. Immunocytochemical identification of azurophilic and specific granule markers in the giant granules of Chediak-Higashi neutrophils. N. Engl. J. Med., 298:693, 1973.CrossRefGoogle Scholar
  68. 68.
    Roder, J.C., Haliotis, T., Klein, M., Korec, S., Jett, J.R., Ortaldo, J., Herberman, R.B., Katz, P., and Fauci, A.S. A new immunodeficiency disorder in humans involving NK cells. Nature, 284:553, 1980.PubMedCrossRefGoogle Scholar
  69. 69.
    Root, R.K., Rosenthal, A.S., and Balestra, D.J. Abnormal bactericidal, metabolic and lysosomal functions of Chediak-Higashi syndrome leukocytes. J. Clin. Invest., 51:649, 1972.PubMedCrossRefGoogle Scholar
  70. 70.
    Rosenszajn, L.A., Ben David, E., and Bar Sela, S. Large granules and lysosomal fusion in human Chediak-Higashi white blood cells. Acta Haemat., 27:279, 1977.CrossRefGoogle Scholar
  71. 71.
    Rosenzajn, L.N., Radnai, J., Tatarski, A., and Benderlei, A. Blood cell culture and chromosomal findings in Chediak-Higashi syndrome. Israel J. Med. Sci., 5:1087, 1969.Google Scholar
  72. 72.
    Sadan, N., Yaffe, D., Rosensjan, L., Adar, H., Soroker, B., and Efrati, P. Cytochemical and genetic studies in four cases of Chediak-Higashi-Steinbrinck syndrome. Acta Haemat., 34:20, 1965.PubMedCrossRefGoogle Scholar
  73. 73.
    Saitoh, H., Komiyama, A., Narose, N., Morosawa, H., and Akabane, T. Development of the accelerated phase during ascorbic acid therapy in Chediak-Higashi syndrome and efficacy of colchicine on its management. Br. J. Haemat., 48:79, 1981.CrossRefGoogle Scholar
  74. 74.
    Salamanca-Gomez, F., Salazar-Mallen, M., and Amezcu, M.E. Chromosome one polymorphism in a girl with the Chediak-Higashi syndrome. Acta Cytologica, 22:402, 1978.PubMedGoogle Scholar
  75. 75.
    Saraiva, L.G., Azevedo, M., Correa, J.M., Carvalho, G., and Prospero, J.D. Anomalous panleukocytic granulation. Blood, 14:112, 1959.Google Scholar
  76. 76.
    Sato, A. Chediak-Higashi’s disease, preliminary report. Probable identity of Chediak’s and Higashi’s diseases. Tohuku J. Exp. Med., 60:22, 1954.CrossRefGoogle Scholar
  77. 77.
    Saxena, R.K., Saxena, Q.B., and Adler, W.H. Defective T-cell responses in beige mutant mice. Nature, 295:240, 1982.PubMedCrossRefGoogle Scholar
  78. 78.
    Sawano, K., Sakono, T., Kagosaki, Y., Yoshimitsu, H., Miyake, Y., Kobayashi, Y. and Usui, T. Chemotaxis of granulocytes in Chediak-Higashi syndrome with agarose plate and filter chamber methods. Hiroshima J. Med. Sci., 28:107, 1979.Google Scholar
  79. 79.
    Say, B., Tuncbilek, E, Yamack, B., and Balci, S. An unusual chromosomal aberration in a case of Chediak-Higashi syndrome. J. Med. Genet., 7:417, 1970.PubMedCrossRefGoogle Scholar
  80. 80.
    Sheramata, W., Kott, S., and Cyr, D.P. The Chediak-Higashi-Steinbrinck syndrome. Presentation of three cases with features resembling spinocerebellar degeneration. Arch. Neurol., 25:289, 1971.PubMedCrossRefGoogle Scholar
  81. 81.
    Spencer, W.H. and Hogan, M.J. Ocular manifestations of Chediak-Higashi syndrome. Report of a case with histo-pathological examination of ocular tissues. Am. J. Ophtal., 50:1197, 1960.Google Scholar
  82. 82.
    Steinbrinck, W. Uber eine neue granulationsanomalie der leukocyten. Dtsch. Arch. Klin. Med., 193:577, 1948.Google Scholar
  83. 83.
    Stossel, T.P., Root, R.K., and Vaughan, M. Phagocytosis in chronic granulomatosis disease and the Chediak-Higashi syndrome. N. Engl. J. Med., 286:120, 1972.PubMedCrossRefGoogle Scholar
  84. 84.
    Tan, C., Etcubanas, E., Leiberman, P., Isenberg, H., King, O., and Murphy, M.L. Chediak-Higashi syndrome in a child with Hodgkin!s disease. Am. J. Dis. Child., 121:135, 1971.PubMedGoogle Scholar
  85. 85.
    Tanaka, T. Chediak-Higashi syndrome: abnormal lysosomal enzymal levels in granulocytes of patients and family members. Pediat. Res., 14:901, 1980.PubMedCrossRefGoogle Scholar
  86. 86.
    Tanaka, T. and Orii, T. High sensitivity but normal DNA-repair activity after UV irradiation in Epstein-Barr-transformed lymphoblastoid cell lines for Chediak-Higashi syndrome. Mutation Res., 72:143, 1980.PubMedCrossRefGoogle Scholar
  87. 87.
    Tanaka, H., Ito, T., and Orii, T. DNA repair mechanisms in Chediak-Higashi syndrome cells. J. Inher. Metab. Dis., 5:65, 1982.PubMedCrossRefGoogle Scholar
  88. 88.
    Tay, C.H., Lopez, C.G., and Lazarus, A.R. The Chediak-Higashi syndrome. Med. J. Aust., 2:1027, 1970.Google Scholar
  89. 89.
    Van de Griend, R. J., Astaldi, A., de Bruin, H.G., and Weening, R.S. T cells in Chediak-Higashi syndrome. N. Engl. J. Med., 306:1368, 1982.PubMedGoogle Scholar
  90. 90.
    Vasalli, J.-D., Granelli-Piperno, A., Griscelli, C., and Reich, E. Specific protease deficiency in polymorphonuclear leukocytes of Chediak-Higashi syndrome and beige mice. J. Exp. Med., 147:1285, 1978.CrossRefGoogle Scholar
  91. 91.
    Weening, R.S., Schoorel, E.P., Vanschaik, M.L.J., Voetman, A.A., Bot, A.A.M., Batenburg-Plenter, A.M., Willeras, Ch., Zeijlemaker, W.P., and Astaldi, A. Effect of ascorbate on abnormal neutrophil, platelet, and lymphocyte function in a patient with the Chediak-Higashi syndrome. Blood, 57:856, 1981.PubMedGoogle Scholar
  92. 92.
    White, J.G. Virus-like particles in the peripheral blood cells of two patients with Chediak-Higashi syndrome. Cancer, 19:877, 1966.PubMedCrossRefGoogle Scholar
  93. 93.
    White, J.G. The Chediak-Higashi syndrome: a possible lysosomal disease. Blood, 28:143, 1966.PubMedGoogle Scholar
  94. 94.
    White, J.G. The Chediak-Higashi syndrome: cytoplasmic sequestration in circulating leukocytes. Blood, 29:4335, 1967.Google Scholar
  95. 95.
    White, J.G. Platelet microtubules and giant granules in the Chediak-Higashi syndrome. Am. J. Med. Tech., 44; 273, 1978.Google Scholar
  96. 96.
    White, J.G. and Gerrard, J.M. The ultrastructure of defective human platelets. Mol. Cell. Biochem., 21:109, 1978.PubMedCrossRefGoogle Scholar
  97. 97.
    White, J.G. and Clawson, C.C. The Chediak-Higashi syndrome. Ring-shaped lysosomes in circulating monocytes. Am. J. Pathol., 96:781, 1979.PubMedGoogle Scholar
  98. 98.
    White, J.G. and Clawson, C.C. The Chediak-Higashi syndrome: microtubules in monocytes and lymphocytes. Am. J. Hematol., 7:349, 1979.PubMedCrossRefGoogle Scholar
  99. 99.
    White, J.G. and Clawson, C.C. The Chediak-Higashi syndrome: the nature of the giant neutrophil granules and their interactions with cytoplasm and foreign particles. I, II, III. Am. J. Pathol., 98:151, 1980.PubMedGoogle Scholar
  100. 100.
    Wolff, S.M., Dale, D.C., Clark, R.A., Root, K., and Kimball, H.R. The Chediak-Higashi syndrome: studies of host defenses. Ann. Int. Med., 76:293, 1972.PubMedGoogle Scholar
  101. 101.
    Zabucchi, G., Cramer, R., Soranzo, M.R., Tamaro, P., and Panizon, F. Biochemical studies on the leukocytes in Chediak-Higashi syndrome. Acta Haemat., 58:50, 1977.PubMedCrossRefGoogle Scholar

Copyright information

© Plenum Publishing Corporation 1984

Authors and Affiliations

  • Fernando Merino
    • 1
  1. 1.Institute for Scientific InvestigationCaracasVenezuela

Personalised recommendations