6533b85bfe1ef96bd12ba1a9

RESEARCH PRODUCT

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

H. K. Müller-hermelinkJ. E. WeinigJ. D. BeckE. M. Lemmel

subject

Immunity CellularFamilial haemophagocytic reticulosisPathologymedicine.medical_specialtyPancytopeniabusiness.industryGraft versus host reactionHepatosplenomegalyInfantSpleenBlood Coagulation DisordersErythrophagocytosisGraft vs Host Reactionmedicine.anatomical_structureSplenomegalyPediatrics Perinatology and Child HealthImmunologymedicineHumansImmune reactivityFemalemedicine.symptombusinessHistiocyteHepatomegaly

description

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.

https://doi.org/10.1007/bf00442199