European Journal of Pediatrics

, Volume 126, Issue 3, pp 175–184 | Cite as

Reversible graft versus host reaction as cause of erythrophagic splenomegaly in a child?

  • J. -D. Beck
  • J. E. Weinig
  • H. K. Müller-Hermelink
  • E. -M. Lemmel
Article

Abstract

The case history of a 9 months old infant with hepatosplenomegaly, pancytopnaenia and disturbances of clotting and cellular immune reactivity is reported. The spleen was removed and showed striking erythrophagocytosis by proliferating histiocytes, typical of “familial erythrophagocytic reticulosis” (Farquhar).

A graft-versus-host reaction is discussed as a possible underlying cause. The favourable clinical course and full recovery point to an interrelation with primary hypersplenism.

Key words

Familial haemophagocytic reticulosis Cellular immune deficiency Graft versus host reaction 

Abbreviations

FHR

Familial Haemophagocytic Reticulosis

GVHR

Graft Versus Host Reaction

Zusammenfassung

Es wird die Krankengeschichte eines 9 Monate alten Säuglings berichter, der an Hepato-Splenomegalie, lebensbedrohender Pancytopenie, Störungen des Gerinnungssystems und der zellulären Immunreaktivität litt. Die Milz wurde operativ entfernt und zeigte histologisch eine ausgeprägte Erythrophagocytose durch proliferierende Histiocyten, wie es für eine “familiäre erythrophagocytische Reticulose” (Farquhar) typisch ist.

Eine graft-versus-host-Reaktion wird als mögliche zugrundeliegende Ursache diskutiert. Der günstige klinische Verlauf und die völlige Erholung weisen auf die Beziehungen zum primären Hypersplenismus hin.

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References

  1. Bell, R. J. M., Brafield, A. J. E., France, N. E.: Familial haemophagocytic reticuloses. Arch. Dis. Child. 43, 601 (1968)Google Scholar
  2. Buist, N. R. M., Jones, R. N., Gaolus, T. R.: Familial haemophagocystic reticuloses in first cousins. Arch. Dis. Child. 46, 728 (1971)Google Scholar
  3. Cederbaum, S. D., Niwayana, G., Stielem, E. R., Neerhout, R. G., Ammann, A. J., Berman, W.: Combined immunodeficiency presenting as the Letterer-Siewe syndrome. J. Pediatr. 85, 466 (1974)Google Scholar
  4. Douglas, S. D., Fudenberg, H. H.: Graft versus host reaction in Wiskott-Aldrich syndrome: antemortem diagnosis of human GVH in an immunological deficience disease. Vox Sang. 16, 172 (1969)Google Scholar
  5. Farquhar, J. W., Claireaux, A. E.: Familial haemophagocytic reticulosis. Arch. Dis. Child. 27, 519 (1952)Google Scholar
  6. Farquhar, J. W., MacGregor, A. R., Richmond, J.: Familial haemophagocytic reticulosis. Brit. Med. J. 1958II, 1561Google Scholar
  7. Fanchier, C., Benatol, A., Regy, J. M., Jobart, P., Combe, P.: La lympho-histiocytose familial. Arch. Fr. Pediatr. 27, 51 (1970)Google Scholar
  8. Ford, J. M., Lucey, J. J., Cullen, M. H., Tobias, J. S., Lister, T. A.: Fatal GVH disease following transfusion of granulocytes from normal donors. Lancet 7996, 1167 (1976)Google Scholar
  9. Gleichmann, E., Gleichmann, H., Wilke, W.: Autoimmunization and lymphomagenesis in parent F1 combinations differing at the major histocompatibility complex: Model for spontaneous disease caused by altered self-antigen? Transplantation Rev. 31, 156 (1976)Google Scholar
  10. Glucksberg, H., Feger, A.: The effect of splenectomy on graft-versus-host disease (GVHD) in mice. J. Society 12, 537 (1972)Google Scholar
  11. Goodall, H. B., Guthrie, W., Buist, N. R. M.: Familial haemophagocytic reticulosis. Scott. Med. J. 10, 425 (1965)Google Scholar
  12. Gotoff, S. P., Esterly, N. R.: Histiocytosis. J. Pediatr. 85, 592 (1974)Google Scholar
  13. Hittmair, A.: Die Milz und ihre Funktionen im Spiegel des Schrifttums. In: Handbuch der gesamten Hämatologie, Band V. München-Berlin-Wien: Urban u. Schwarzenberg 1969Google Scholar
  14. Koto, A., Morechi, R., Santorineou, M.: Congenital hemophagocytic reticulosis. Ann. J. Clin. Path. 65, 495 (1976)Google Scholar
  15. Lemmel, E.-M., Good, R. A.: Immunosuppressive action of mitomycin C on lymphoid cells. I. Effect on cell-mediated immunity after in vitro treatment, tolerance induction, and recovery in vivo. Int. Arch. Allergy 36, 554 (1969)Google Scholar
  16. Lennert, K., Mohri, N.: Zur Pathologie der Leukämien und malignen Lymphome im Kindesalter. Verh. dtsch. Ges. Path. 55, 216 (1971)Google Scholar
  17. Levine, S.: Graft-versus-host disease. Transplantation 6, 294 (1968)Google Scholar
  18. Maas, B., Goedecke, L., Roloff, D., Drees, N.: Familäre hamophagocytierende Reticulose. Vorstellung eines Krankheitsfalles. Mschr. Kinderheilk. 122, 164 (1974)Google Scholar
  19. Marrian, V. J., Sanerkin, N. G.: Familial histiocytic reticulosis (familial haemophagocytic reticulosis). J. Clin. Path. 16, 65 (1963)Google Scholar
  20. Marshall, A. H. E.: Histiocytic medullary reticulosis. J. Path. Bact. 71, 61 (1965)Google Scholar
  21. MacMahon, H. E., Bedizel, M., Ellis, G. A.: Familial erythrophagocytic lymphohistiocytosis. Pediatrics 32, 868 (1963)Google Scholar
  22. McClusen, P. D., Strachan, P., Saunders, E. F.: Hypofibrinogenemia and thrombocytopenia in familial hemophagocytic reticulosis. J. Pediatr. 85, 67 (1974)Google Scholar
  23. Miller, D. R.: Familial reticuloendotheliosis: Concurrence of disease in five siblings. Pediatrics 38, 986 (1966)Google Scholar
  24. Miller, M. E.: Thymic dysplasia (Swiss agamma-globulinemia) Graft versus host reaction following bone marrow transfusion. J. Pediatr. 70, 730 (1967)Google Scholar
  25. Mozziconacci, P., Nezelof, C., Attal, C., Girard, F., Pahm-hun-trung, Weil, J., Desbuquois, B., Gadot, M.: La lympho-histiocytose familiale. Arch. Fr. Ped. 22, 385 (1965)Google Scholar
  26. Nelson, P., Santamaria, A., Olson, R. L., Nayak, N. C.: Generalized lymphohistiocytic infiltration. Pediatrics 27, 931 (1961)Google Scholar
  27. Nelson, P., Santamaria, A., Olson, R. L., Nayak, N. C.: Generalized lymphohistiocytic infiltration. Pediatrics 27, 931 (1961)Google Scholar
  28. Nezelof, G., Eliachar, E.: La lymphohistiocytose familiale. Nouv. Rev. Franc. Hemat. 13, 319 (1973)Google Scholar
  29. Pöttgen, W.: Die moderne Theorie des Hypersplenismus auf der Grundlage zellkinetischer Befunde. Dtsch. Med. Wschr. 97, 1100 (1972)Google Scholar
  30. Rogers, D. L., Benson, T. E.: Familial Letterer-Siewe Disase. J. Pediatr. 60, 550 (1962)Google Scholar
  31. Schilling, V.: Über erythrophagische Megalosplenie. Med. Klin. 16, 508 (1952)Google Scholar
  32. Schoeck, V. W., Peterson, R. D. A., Good, R. A.: Familial occurence of Letterer-Siewe-disease. Pediatrics 32, 1055 (1963)Google Scholar
  33. Slavin, R. E., Santos, G. W.: The graft versus host reaction in man after bone marrow transplantation: pathology, pathogenesis, clinical features, and implication. Clin. Immun. Path. 1, 472 (1973)Google Scholar
  34. Smith, G. H.: Blood disease of infancy and childhood. Third Edition. Saint Louis: Mosby 1972Google Scholar
  35. South, M.-A., Lemmel, E.-M., Canales, L.: Experimental reconstitution in combined immune deficiency disease (Thymic alymphoplasia, swiss-type agammaglobinemia). Fed. Proc. 28, 376 (1969)Google Scholar
  36. Stutte, H. J.: Hypersplenismus und Milzstruktur. Normale und pathologische Anatomie. Moriographien un zwangloser Folge. Hrsg. Bargmann, S., Doerr, S., Heft 28. Stuttgart: Thieme 1974Google Scholar
  37. Varadi, S., Gordon, R. R., Abbott, D.: Haemophagocytic reticulosis diagnosed during life. Acta Haemat. 31, 349 (1964)Google Scholar
  38. Weinberg, A. G., Rogers, L. E.: Hepatosplenomegaly, pancytopenia and fewer. J. Pediatr. 82, 879 (1973)Google Scholar
  39. Weinreich, J.: Milz und Leber in ihrem Einfluß auf das Blutbild. Untersuchungsergebnis zum Problem des “Hypersplenismus”. Acta Hepato. Splen. 10, 81 (1963)Google Scholar

Copyright information

© Springer-Verlag 1977

Authors and Affiliations

  • J. -D. Beck
    • 1
  • J. E. Weinig
    • 2
  • H. K. Müller-Hermelink
    • 2
  • E. -M. Lemmel
    • 3
  1. 1.Department of PediatricsUniversity of MainzMainzFederal Republic of Germany
  2. 2.Department of Pathological AnatomyUniversity of KielKielFederal Republic of Germany
  3. 3.Department of Internal MedicineUniversity of MainzMainzFederal Republic of Germany

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