Neutropenia with a probable hereditary basis in Border Collies

Abstract

This paper documents a disease of Border Collies characterised by chronic neutropenia which probably resulted in recurrent bacterial infections manifesting as osteomyelitis and sometimes gastroenteritis. The neutropenia occurred despite hyperplasia of the myeloid cells in the bone marrow and a “shift to the right” in myeloid cell maturation. The underlying defect is currently unknown but may result from the inability of neutrophils to escape from the bone marrow into the peripheral circulation. Necrosis and new bone formation commonly involved the metaphyseal regions of long bones. Other findings included a fasting hypercholesterolaemia and the occasional presence of nucleated red blood cells in circulation, despite a non-regenerative anaemia. An autosomal recessive mode of inheritance is suspected. This condition has some features of a rare human disease called myelokathexis which is also believed to have in autosomal recessive mode of inheritance.