Involvement of the CNS in Certain Hematologic Diseases of Infancy and Childhood

  • Reinhard L. Friede


A bleeding disposition in the newborn infant may result from: 1. thrombocytopenia, 2. vitamin K deficiency, 3. a temporary insufficiency in hepatic synthesis of coagulation factors because of immaturity or due to hepatic damage by anoxia or infectious diseases, and 4. inherited anomalies of the coagulation mechanism. Intracranial or cerebral hemorrhages may be caused by any of these disease processes. Their neuropathologic documentation for infants is scanty, but the available observations suggest that the type of bleeding does not vary significantly with the various etiologies. The most common form of intracranial bleeding in hemorrhagic diseases of the newborn appears to be subarachnoid hemorrhage, and the subsequent development of obstructive hydrocephalus has been described in several reports.


Sickle Cell Disease Chronic Granulomatous Disease Multinucleated Giant Cell Hematologic Disease Obstructive Hydrocephalus 
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  1. Bartman, J., Van de Velde, R. L., Friedmann, F.: Pigmented lipid histiocytes and susceptibility to infection: ultrastructure of splenic histiocytes. Pediatrics 40: 1000–1002, 1967.PubMedGoogle Scholar
  2. Beguez-Cesar, A.: Neutropenia cronica maligna familiar con granulaciones atipicas de los leucocitos. Bol. Soc. Cub. Pediat. 15: 900–922, 1943.Google Scholar
  3. Bell, R. J. M., Bradfield, A. J. E., Barnes, N. D., France, N. E.: Familial haemophagocytic reticulosis. Arch. Dis. Child. 43: 601–606, 1968.PubMedCrossRefGoogle Scholar
  4. Berendes, H., Bridges, R. A., Good, R. A.: A fatal granulomatosis of childhood. Minn. Med. 40: 309–312, 1957.PubMedGoogle Scholar
  5. Carson, M. J., Chadwick, D. L., Brubaker, C. A., Cleland, R. S., Landing, B. H.: Thirteen boys with progressive septic granulomas. Pediatrics 35: 405–412, 1965.PubMedGoogle Scholar
  6. Chediak, M.: Nouvelle anomalie leucocytaire de charactère constitutionel et familial. Nouv. Rev. franc. Hémat. 7: 362–367, 1952.Google Scholar
  7. Cooper, H. A., Bowie, E. J. W., Bugert, E. O., Jr., Mellinger, J. F.: Hydrocephalus in child with von Willebrand’s disease. Amer. J. Dis. Child. 121: 340–343, 1971.PubMedGoogle Scholar
  8. Dayan, A. D.: Chronic encephalitis in children with severe immuno-deficiency. Acta neuropath. (Berl.) 19: 234–241, 1971.CrossRefGoogle Scholar
  9. Donohue, W. L., Bain, H. W.: Chediak-Higashi syndrome. Pediatrics 20: 416–430, 1957.PubMedGoogle Scholar
  10. Farquhar, J. W., Claireaux, A. E.: Familial haemophagocytic reticulosis. Arch. Dis. Child. 27: 519–525, 1952.PubMedCrossRefGoogle Scholar
  11. Freireich, E. J., Thomas, L. B., Frei, E., Fritz, R. D., Forkner, C. E.: A distinctive type of intracerebral hemorrhages associated with “blastic crisis” in patients with leukemia. Cancer 13: 146–154, 1960.PubMedCrossRefGoogle Scholar
  12. Gordon, R. R., Dean, T.: Foetal deaths from antenatal anticoagulant therapy. Brit. med. J. 2: 719–721, 1955.PubMedCrossRefGoogle Scholar
  13. Hanissian, A. S., Jabbour, J. T., De Lamerens, S., Garcia, J. H., Horta-Barbosa, L.: Subacute encephalitis and hypogammaglobulinemia. Amer. J. Dis. Child. 123: 151–155, 1972.PubMedGoogle Scholar
  14. Hartmann, J. R., Diamond, L. K.: Haemophilia and related haemorrhagic disorders. Practitioner 178: 179–190, 1957.PubMedGoogle Scholar
  15. Haupt, H.: Störungen der Blutgerinnung infolge perinataler Hirnschädigung. Dtsch. med. Wschr. 90: 1473–1477, 1965.PubMedCrossRefGoogle Scholar
  16. Higashi, O.: Congenital gigantism of proxidase granules. Tohoku J. exp. Med. 59: 315–332, 1954.PubMedCrossRefGoogle Scholar
  17. Holmes, B., Quie, P. G., Windhorst, D. B., Good, R. A.: Fatal granulomatous disease of childhood. Lancet 1: 1225–1228, 1966.PubMedCrossRefGoogle Scholar
  18. Høyerall, H. M., Lamvik, J., Moe, P. J.: Congenital hypoplastic thrombocytopenia and cerebral malformations in two brothers. Acta paed. scand. 59: 185–191, 1970.CrossRefGoogle Scholar
  19. Iversen, T.: Leukaemia in infancy and childhood. Acta paediat. scand. Suppl. 106: 1–219, 1966.Google Scholar
  20. Keely, M. L., Taylor, N., Chard, R. L. Fr.: Spinal cord compression as a complication of haemophilia. Arch. Dis. Child. 47: 826–827, 1972.PubMedCrossRefGoogle Scholar
  21. Landing, B. H., Shirkey, H. S.: A syndrome of recurrent infection and infiltration of viscera by pigmented lipid histiocytes. Pediatrics 20: 431–438, 1957.PubMedGoogle Scholar
  22. Leader, R. W., Padgett, G. A., Gorham, J. R.: Studies of abnormal leukocyte bodies in the mink. Blood 22: 477–484, 1963.PubMedGoogle Scholar
  23. Lockman, L. A., Kennedy, W. R., White, J. G.: The Chediak-Higashi syndrome: Electrophysiologic and electron microscopic observations on the peripheral neuropathy. J. Pediat. 70: 942–951, 1967.PubMedCrossRefGoogle Scholar
  24. Lutzner, M. A., Lowrie, C. T., Jordan, H. W.: Giant granules in leukocytes of the beige mouse. J. Hered. 58: 299–300, 1967.PubMedGoogle Scholar
  25. MacMahon, H. E., Bedizel, M., Ellis, C. A.: Familial erythrophagocytic lymphohistiocytosis. Pediatrics 32: 868–879, 1963.PubMedGoogle Scholar
  26. McCarthy, J. W., Coble, L. L.: Intracranial hemorrhage and subsequent communicating hydrocephalus in a neonate with classical hemophilia. Pediatrics 51: 123–124, 1973.Google Scholar
  27. Moore, E. W., Thomas, L. B., Shaw, R. K., Freireich, E. J.: The central nervous system in acute leucemia. Arch. intern. Med. 105: 451–468, 1960.PubMedCrossRefGoogle Scholar
  28. Padgett, G. A., Leader, R. W., Gorham, J. R., O’Mary, C. C.: The familial occurrence of the Chediak-Higashi syndrome in mink and cattle. Genetics 49: 505–512, 1964.PubMedGoogle Scholar
  29. Pearson, H. A., Shulman, N. R., Marder, V. J., Cone, T. E.: Isoimmune neonatal thrombocytopenic purpura. Blood 23: 154–177, 1964.PubMedGoogle Scholar
  30. Porter, F., Thurman, W. G.: Studies of sickle cell disease. Amer. J. Dis. Child. 106: 35–42, 1963.Google Scholar
  31. Price, D. L., Woolsey, J. E., Rosman, N. P., Richardson, E. P.: Familial lymphohistiocytosis of the nervous system. Arch. Neurol. (Chic.) 24: 270–283, 1971.CrossRefGoogle Scholar
  32. Price, R. A., Johnson, W. W.: The central nervous system in childhood leukemia. I. The arachnoid. Cancer 31: 520–533, 1973.PubMedCrossRefGoogle Scholar
  33. Prieur, D. J., Davis, W. C., Padgett, G. A.: Defective function of renal lysosomes in mice with Chediak-Higashi syndrome. Amer. J. Path. 67: 227–240, 1972.PubMedGoogle Scholar
  34. Reese, A. J. M., Levy, E.: Familial incidence of non-lipoid reticulo-endotheliosis (LettererSiwe disease). Arch. Dis. Child. 26: 578–581, 1951.PubMedCrossRefGoogle Scholar
  35. Schenk, W. D.: Hemorrhage in spinal cord with syringomyelia in a patient with hemophilia. Acta neuropath. (Berl.) 2: 306–308, 1963.CrossRefGoogle Scholar
  36. Silverstein, A.: Intracranial bleeding in hemophilia. Arch. Neurol. 3: 141–157, 1960.PubMedCrossRefGoogle Scholar
  37. Steinbrinck, W.: Über eine neue Granulationsanomalie der Leukozyten. Dtsch. Arch. klin. Med. 193: 577–581, 1948.Google Scholar
  38. Sung, J. H., Meyers, J. P., Stadlan, E. M., Cowen, D., Wolf, A.: Neuropathological changes in Chediak-Higashi disease. J. Neuropath. exp. Neurol. 28: 86–118, 1969.PubMedCrossRefGoogle Scholar
  39. Veiga, S., Vaithianathan, T.: Massive intravascular sickling after exchange transfusion with sickle cell trait blood. Transfusion 3: 387–391, 1963.PubMedCrossRefGoogle Scholar
  40. Verhaart, W. J. C.: Polio-encephalopathy or masked encephalitis in familial hypogammaglobulinemia. J. Neuropath. exp. Neurol. 20: 380–385, 1961.PubMedCrossRefGoogle Scholar
  41. Von Sydow, G.: Hypopotrombinemi och hjarnskada hos barn till dikumarinbehandlad moder. Nordisk. Medicin 34: 1171–1172, 1947.PubMedGoogle Scholar
  42. White, H. H., Kepes, J. H., Kirkpatrick, C. H., Schimke, R. N.: Subacute encephalitis and congenital hypogammaglobulinemia. Arch. Neurol. (Chic.) 26: 359–365, 1972.CrossRefGoogle Scholar
  43. White, J. G.: The Chediak-Higashi syndrome: a possible lysosomal disease. Blood 28: 143–156, 1966.PubMedGoogle Scholar
  44. Willi, H.: Die Blutungskrankheiten des Neugeborenen. Erg. inn. Med. Kinderheilk. 2: 467–543, 1951.CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Wien 1975

Authors and Affiliations

  • Reinhard L. Friede
    • 1
    • 2
  1. 1.Case Western Reserve UniversityClevelandUSA
  2. 2.University of ZurichSwitzerland

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