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.
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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Abbreviations
- FHR:
-
Familial Haemophagocytic Reticulosis
- GVHR:
-
Graft Versus Host Reaction
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Beck, J.D., Weinig, J.E., Müller-Hermelink, H.K. et al. Reversible graft versus host reaction as cause of erythrophagic splenomegaly in a child?. Eur J Pediatr 126, 175–184 (1977). https://doi.org/10.1007/BF00442199
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DOI: https://doi.org/10.1007/BF00442199