[Constitutional familial leucocytopenia with partial Pelger anomaly and delayed development of bone (author's transl)].
The clinical and hematological findings in 4 boys of a family with a chronic constitutional leukocytopenia are described. Possible X-chromosomal sex-linked genetic transmission, the benign clinical course as well as the association with clinical and cytological hints of the involvement of the lymphatic cellular immunological system suggest a disease of its own. It was classified as a mild form of myelolymphatic insufficiency similar to reticular dysgenesis. The significance of Pelger-Huët anomalies of neutrophils demonstrated in the blood of the mother and of Pelger-like forms in the blood of children are discussed.
D000328:Adult; D001848:Bone Diseases, Developmental / Q000235:genetics*; D001853:Bone Marrow / Q000473:pathology; D001854:Bone Marrow Cells; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007958:Leukocyte Count; D007962:Leukocytes / Q000473:pathology; D007970:Leukopenia / Q000097:blood / Q000235:genetics* / Q000276:immunology; D008297:Male; D009503:Neutropenia / Q000235:genetics; D010375:Pedigree; D010381:Pelger-Huet Anomaly / Q000097:blood / Q000235:genetics*; D013577:Syndrome
Konstitutionelle familiäre Leukocytopenie mit partieller Pelger-Anomalie und ossärer Entwicklungsverzögerung