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Spontaneous healing of Langerhans cell histiocytosis (histiocytosis X)

  • Hematology and Oncology
  • Published:
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Abstract

A 3-month-old male infant presented with pallor, hepatomegaly (4.5 cm), splenomegaly (1.5 cm), anaemia (Hb 6 g/dl) and thrombocytopenia (16×109/l). A liver biopsy was diagnostic for Langerhans cell histiocytosis (histiocytosis X). The patient's lymphocytes, co-cultured with neonatal lymphocytes, were positive for virus-like particles without reverse transcriptase activity. The hepatomegaly diminished after 6 months and a second liver biopsy showed decreased histiocytic infiltration. A second viral blood culture remained negative. After 14 months, the hepatomegaly had disappeared completely and there were no more abnormal haematological or clinical findings.

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Abbreviations

LCAT:

lecithin cholesterol acyltransferase

HIV:

human immunodeficiency virus

References

  1. Albers JJ, Adolphson JL, Ching-Hong-Chen (1981) Radioimmunoassay of human plasma lecithin-cholesterol acyltransferase. J Clin Invest 67:141–148

    Google Scholar 

  2. Broadbent V, Davies EG, Heaf D, Pincott JR, Fritchard J, Levinsky RJ, Atherton DJ, Tucker S (1984) Spontaneous remission of multi-system histiocytosis-X. Lancet I:253–254

    Google Scholar 

  3. Ching-Hong Chen, Albers JJ (1982) Characterization of proteiliposomes containing apoprotein A-1: a new substrate for the measurement of lecithin: cholesterol acyltransferase activity. J Lipid Res 23:680–691

    Google Scholar 

  4. Fischer A, Virelizier JL, Arenzana-Seisdedos F, Perez N, Nezelof C, Griscelli C (1985) Treatment of four patients with erythrophagocytic lymphohistiocytosis by a combination of epipodophyllotoxin, steroids, intrathecal methotrexate, and cranial irradiation. Pediatrics 76:263–268

    Google Scholar 

  5. Hashimoto K, Pritsker MS (1973) Electron microscopy study of reticulohistiocytoma. Arch Dermatol 107:263–270

    Google Scholar 

  6. Hashimoto K, Griffin D, Kohsbaki M (1982) Self-healing reticulohistiocytosis: a clinical, histologic, and ultrastructural study of the fourth case in the literature. Cancer 49:331–337

    Google Scholar 

  7. Jaeken J, Casteels-Van Daele M, Harvengt L, Corbeel L, Broeckaert-Van Orshoven A, Van Damme B, Jenis H, Eeckels R (1973) A hyperlipemia syndrome in infancy with rapidly fatal evolution. Helv Paediatr Acta 28:67–71

    Google Scholar 

  8. Nagasaki T, Akanuma Y (1977) A new colorimetric method for the determination of plasma lecithin-cholesterol acyltransferase activity. Clin Chim Acta 75:371–375

    Google Scholar 

  9. Osband ME, Lipton JM, Lavin P, Levey R, Vawter G, Greenberg JS, McCaffrey RP, Parkman R (1981) 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

    Google Scholar 

  10. Wolfson SL, Botero F, Hurwitz S, Pearson HA (1981) “Pure” cutaneous histiocytosis-X. Cancer 48:2236–2238

    Google Scholar 

  11. Wong CG, Ladisch S, Sweeley CC (1983) Hepatic ganglioside abnormalities in a patient with familial erythrophagocytic lymphohistiocytosis. Pediatr Res 17:413–417

    Google Scholar 

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Dedicated to Walter Teller on the occasion of his 60th birthday

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Corbeel, L., Eggermont, E., Desmyter, J. et al. Spontaneous healing of Langerhans cell histiocytosis (histiocytosis X). Eur J Pediatr 148, 32–33 (1988). https://doi.org/10.1007/BF00441809

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  • DOI: https://doi.org/10.1007/BF00441809

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