Lesions in Brangus Cattle with Chediak-Higashi Syndrome

Abstract

Abstract. Hair, peripheral blood leukocytes, and other tissues from two related Brangus calves with phenotypic characteristics of Chediak-Higashi syndrome were examined by light and electron microscopy. Enlarged, pleomorphic, cytoplasmic granules, morphologically compatible with lysosomes, were seen in several neutrophils, many eosinophils, renal tubular epithelial cells, and Kupffer cells. Hair shafts of the calves showed irregular distribution and clumping of melanin granules. Severe infection and a possible hemorrhagic tendency were recognized. These Brangus calves represent the third breed of cattle affected with this genetic disease.Chediak-Higashi syndrome (CHS) is a genetic disease occurring in man,2s26 mink,I5 cattle,I5 beige (CHS) mice,6 cats,g killer whales,23 and f o x e~.~J~ CHS is manifested by partial oculocutaneous albinism, photophobia, marked susceptibility to infection~,'~ platelet pool storage deficiency,' lack of natural killer cell activity,21 impaired microtubule function, l4 and the occurrence of large pleomorphic granules in the cytoplasm of leukocytes, and most, but not all, granule-producing cell^.^^^ Many of these enlarged granules have ultrastructural and cytochemical characteristics of 1ysosomes.8 The granules in neutrophils and eosinophils are strongly peroxidase-positive or Sudan Black B-positive.2 The enlarged granules in CHS cells often stain positively with periodic acid-Schiff (PAS).I3J7 CHS was originally described in Hereford cattle as being characterized by abnormal leukocytes, hypopigmentation, and increased susceptibility to disease, conditioned by an autosomal recessive gene.16 All CHS cattle reported were incomplete albinos, with gray irises instead of the normal dark brown to black. Several had hemorrhagic tendency, and melanin granules were scant in the skin. Comparative studies of CHS and non-CHS cattle have demonstrated the presence of PAS-positive granular inclusions in renal tubular epithelium of CHS but not non-CHS cattle.I7An 8-month-old female Japanese black heifer diagnosed as affected with CHS was reported to have the following lesions: growth retardation, brownish skin and hair, and enlarged granules in the cytoplasm of all eosinophils and basophils. Similar granules were seen less frequently in all other granulocytic leukocytes except the monocyte. Many inflammatory changes were present in various organs and tissues. Histological and ultrastructural findings of eosinophilic or brownishyellow pigment in the neurons and renal tubular epithelium coincided with those previously reported in CHS.24This study describes ultrastructural and light microscopic studies of hair, leukocytes, and other cells of two related Brangus calves with phenotypic and microscopic characteristics of CHS.This study was done as a service to people in the livestock industry to help make them aware of the negative economic implications associated with genetically transmitted disease. Materials and MethodsThe occurrence of related Brangus calves affected with a pigmentary deficiency was br...

Changes of Platelet Cytoskeletal Proteins Following Platelet Aggregation Induced by Collagen and Platelet Membrane Glycoprotein in Japanese Black Cattle with .DELTA.-Storage Pool Deficiency.

“…The lower platelet aggregation rate induced by collagen and the larger sized and less numbered granules in eosinophils observed in all cases in this study were in agreement with those in Japanese Black cattle with δ-SPD reported previously [1]. These findings were also reported in cattle with CHS [2,5,18,19,26], which was one of diseases belonged to δ-SPD in cattle. Affected Hereford cattle and Brangus cattle are reported to have shown partial albinism of hair coat and iris, marked suceptibility to infections and photophobia [2,5,19], but those were not observed in any of the cases in this study.…”

“…These findings were also reported in cattle with CHS [2,5,18,19,26], which was one of diseases belonged to δ-SPD in cattle. Affected Hereford cattle and Brangus cattle are reported to have shown partial albinism of hair coat and iris, marked suceptibility to infections and photophobia [2,5,19], but those were not observed in any of the cases in this study. Therefore, the cases and those cattle examined in our previous report [1] were unlikely classified in CHS.…”

“…Briefly, platelet cytoskeletons obtained from 1.5 × 10 7 platelets at 0, 10, 20, 30, 60 and 300 sec after the platelet aggregation induced by collagen were electrophoretically analyzed by sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE) using 10% gel and stained with Coomassie brilliant blue, and then absorbance of each fraction was measured by a densitometer (DENSITRON CRChediak-Higashi syndrome (CHS) [2,5,18,19,26], thrombocytopathy [22,23] and plasma thromboplastin antecedent (Factor X I) deficiency [11] were reported as the hereditary hemorrhagic diseases in cattle. We reported on δ-storage pool deficiency (δ-SPD) occurred in Japanese Black cattle [1].…”

Platelet Dysfunction in Chediak-Higashi Syndrome-Affected Cattle.

“…In most human patients affected with CHS, death often occurs in the first decade of life due to infection, bleeding, or development of the 'accelerated phase', which is characterized by a lymphoproliferative syndrome with lymphohistiocytic infiltration. CHS in cattle is found in Hereford [49], Japanese Black [69] and Brangus breeds [2]. In Japan, Japanese Black cattle affected with CHS are frequently seen in South Kyusyu area (Miyazaki and Kagoshima Prefectures) [46] and infrequently in other places.…”

“…Prolonged bleeding time has been reported in most of affected animal species [2,5,6,12,16,35,46,63]. On the other hand, coagulation system is normal in humans, Japanese Black cattle and cats affected with CHS [12,35,46].…”