JPC SYSTEMIC PATHOLOGY
HEMOLYMPHATIC SYSTEM
April 2018
H-M03 (NP)

Signalment:  4-month-old female polled Hereford calf

HISTORY:  This calf had progressive seborrhea since one month of age. Samples include skin and diaphyseal bone marrow.

HISTOPATHOLOGIC DESCRIPTION:  Bone marrow: Diffusely, there is a marked increase in bone marrow cellularity and the majority of cells are 10-15 um in diameter, with brightly eosinophilic cytoplasm, a single round 4-5um nucleus withclumped to condensed nuclear chromatin (erythroid series: rubricyte or late rubricyte); cells are occasionally binucleate or have irregular nuclear profiles; there are also smaller cells with hyperchromatic nuclei and eosinophilic cytoplasm (metarubricytes). Multifocally, there are many 60-70 um diameter multinucleated cells with eosinophilic cytoplasm (megakaryocyte hyperplasia).  Frequently, macrophages contain golden-brown pigment (hemosiderin).  There is a marked decrease in the M:E. There is an absence of adipose tissue and bony trabeculae are rare.

Haired skin: Diffusely, the epidermis is plicated and there is marked epidermal and follicular orthokeratotic hyperkeratosis that often fills and distends hair follicles. Within the epidermis at all levels and within the internal root sheath there are few individual keratinocytes that are detached from surrounding cells, have pyknotic nuclei and hyperchromatic, eosinophilic cytoplasm (dyskeratosis). Multifocally, the granular layer is prominent, and the internal root sheath is fragmented or disrupted, with missing or irregularly shaped hair shafts. Diffusely, periadnexal and perivascular connective tissues are expanded by few lymphocytes, macrophages, rare eosinophils, mildly ectatic lymphatics, and edema.

MORPHOLOGIC DIAGNOSIS:  1. Bone marrow:  Erythroid hyperplasia, marked, with erythroid maturation arrest, polled Hereford, bovine.

  1. Haired skin: Dyskeratosis, epidermal and follicular, multifocal, moderate, with diffuse marked epidermal and follicular orthokeratotic hyperkeratosis and mild lymphohistiocytic perivascular and periadnexal dermatitis.

ETIOLOGIC DIAGNOSIS:  Congenital dyserythropoiesis and dyskeratosis

SYNONYMS:  Congenital anemia, dyskeratosis, and progressive alopecia syndrome; congenital dyserythropoiesis of Herefords

GENERAL DISCUSSION:

PATHOGENESIS:

TYPICAL CLINICAL FINDINGS:

TYPICAL GROSS FINDINGS:

TYPICAL LIGHT MICROSCOPIC FINDINGS:

Bone marrow: 

Skin:

ULTRASTRUCTURAL FINDINGS:

ADDITIONAL DIAGNOSTIC TESTS:

DIFFERENTIAL DIAGNOSIS:

COMPARATIVE PATHOLOGY:

REFERENCES:

  1. Brockus CW. Erythrocytes. In: Latimer KS ed. Duncan and Prasse’s Veterinary Laboratory Medicine: Clinical Pathology. 5th ed. Ames, IA: Iowa State University Press; 2011:37-42.
  2. Burton S, Ikede B, Lofstedt J, Keefe G, Miller L. Congenital dyserythropoiesis and dyskeratosis in a polled Hereford calf. Can Vet J. 1994; 35:519-520.
  3. Holland C, Canfield P, Watson A, Allan G. Dyserythropoiesis, polymyopathy, and cardiac disease in three related English springer spaniels. J Vet Internal Med. 1991;5:151-159.
  4. Mauldin E, Peters-Kennedy J. Integumentary system. In: Maxie MG, ed. Jubb, Kennedy, and Palmer’s Pathology of Domestic Animals. 6th ed. Vol 1. St. Louis, MO: Elsevier limited; 2016:539.
  5. Steffen D, Leipold H, Gibb J, Smith J. Congenital anemia, dyskeratosis, and progressive alopecia in polled Hereford calves. Vet Pathol. 1991;28:234‑240.
  6. Steffen D, Leipold H, Elliott G, Smith J. Ultrastructural findings in congenital anemia, dyskeratosis, and progressive alopecia in Polled Hereford calves. Vet Pathol. 1992;29:203‑209.
  7. Steffen D, Leipold H, Elliott G, Smith J. Congenital dyserythropoiesis and progressive alopecia in polled Hereford calves: hematologic, biochemical, bone marrow cytologic, electrophoretic, and flow cytometric findings. J Vet Diagn Invest. 1992;4:31-37.
  8. Steffen D, Leipold H, Schalles R, Kemp K, Smith J. Epidemiologic findings in congenital anemia, dyserythropoiesis, and dyskeratosis in polled Hereford calves. J Hered. 1993;84:263‑265.

 

 


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