Skip to main content
Log in

Hematological findings in the Norrbottnian type of Gaucher disease

  • Original Investigations
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

A controlled study of hematological changes was performed on 14 patients with the Norrbottnian type of Gaucher disease, seven of whom had been splenectomized at least one year before the study. Each patient had two controls matched to age, sex and habitation. Before splenectomy the patients had slight to moderate microcytic, hypochromic anemia which changed after splenectomy to macrocytic, hypochromic anemia. The splenectomized patients had a high percentage of target cells (mean frequency 33%) and a significantly increased hypo-osmotic resistance of the erythrocytes which showed a positive correlation with the number of target cells. Before removal of the spleen the patients had slight leukopenia with a relative increase of mononuclear cells and moderate thrombocytopenia. In the splenectomized patients the thrombocyte count was normal and there was moderate leukocytosis with a persistent relative increase of lymphocytes.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Ask-Upmark E (1935) The remote effects of removal of the normal spleen in man. Acta Soc Med Upsaliensis 61:197–244

    Google Scholar 

  2. Barrett AM (1938) A special form of erythrocyte possessing increased resistance to hypotonic saline. J Pathol 46:603–618

    Google Scholar 

  3. Bessis M (1970) Etude sur les poikilocytes au microscope a balayage, en particulier dans la thalassémie. Nouv Rev Fr d'Hematol Blood Cells 10:515–534

    Google Scholar 

  4. Bowdler AJ (1963) Dilution anemia corrected by splenectomy in Gaucher disease. Case studies. Ann Intern Med 58:664–669

    PubMed  Google Scholar 

  5. Cartwright GE (1966) The anemia of chronic disorders. Semin Hematol 3:351–375

    PubMed  Google Scholar 

  6. Dreborg S, Eriksson A, Hagberg B (1980) Gaucher disease-Norrbottnian type I. General clinical description. Eur J Pediatr 133:107–118

    PubMed  Google Scholar 

  7. Dybkaer R (1979) International Union of Pure and Applied Chemistry and International Federation of Clinical Chemistry: Approved recommendation (1978) List of quantities in clinical chemistry. Clin Chim Acta 96:18F-204F

    Google Scholar 

  8. Hillborg PO, Svennerholm L (1960) Blood level of cerebrosides in Gaucher disease. Acta Paediatr Scand 49:707–710

    Google Scholar 

  9. Lipson RL, Bayard ED, Watkins CH (1959) The postsplenectomy blood picture. Am J Clin Pathol 32:526–532

    PubMed  Google Scholar 

  10. Lundin P, Persson E, Weinfeld A (1964) Comparison of hemosiderin estimation in bone marrow sections and bone marrow smears. Acta Med Scand 175:383–390

    PubMed  Google Scholar 

  11. Matoth Y, Fred K (1965) Chronic Gaucher disease. Clinical observation on 34 patients. J Med Sci 1:521–530

    Google Scholar 

  12. McBridge JA (1968) The effect of splenectomy on the leukocyte count. Br J Haematol 14:225–231

    PubMed  Google Scholar 

  13. Medoff AS, Bayard ED (1954) Gaucher disease in 29 cases: Hematologic complications and effect of splenectomy. Ann Intern Med 40:481–492

    PubMed  Google Scholar 

  14. Reich C, Seife M, Kessler BJ (1951) Gaucher disease: a review, and discussion of twenty cases. Medicine 30:1–20

    PubMed  Google Scholar 

  15. Singer K, Miller EB, Dameshek W (1941) Hematologic changes following splenectomy in man, with particular reference to target cells, hemolytic index and lysolecithin. Am J Med Sci 202: 171–187

    Google Scholar 

  16. Svennerholm L, Håhansson G, Dreborg S (1980) Assay of the β-glucosidase activity with natural labelled and artificial substrates in leukocytes from homozygotes and heterozygotes with the Norrbottnian type (Type 3) of Gaucher disease. Clin Chim Acta 106:183–193

    PubMed  Google Scholar 

  17. Wilcoxon F (1945) Individual comparisons by ranking methods. Biometrics Bull 1:80–90

    Google Scholar 

  18. Wintrobe MM (1981) Disorders of the granulocyte and monocyte-macrophage systems. In: Clinical hematology. Lea and Febiger, Philadelphia, pp 1341–1344

    Google Scholar 

  19. Zwicker M (1956) Beitrag zum Einfluß der Splenectomie auf Blutbild und Knochenmarksausstrich. Folia Haematol 74: 109–124

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Tibblin, E., Dreborg, S., Erikson, A. et al. Hematological findings in the Norrbottnian type of Gaucher disease. Eur J Pediatr 139, 187–191 (1982). https://doi.org/10.1007/BF01377354

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01377354

Key words

Navigation