Kostmann's disease or severe hereditary neutropenia-the man behind the syndrome

Lars Stenhammar; Leif Strömberg; Carl Gustaf Ljunggren

doi:10.1007/s00277-020-04142-y

Kostmann's disease or severe hereditary neutropenia-the man behind the syndrome

Ann Hematol. 2020 Oct;99(10):2339-2341. doi: 10.1007/s00277-020-04142-y. Epub 2020 Jun 23.

Authors

Lars Stenhammar¹, Leif Strömberg², Carl Gustaf Ljunggren²

Affiliations

¹ Department of Pediatrics and Department of Clinical and Experimental Medicine, Norrköping Hospital, Linköping University, SE-60182, Norrköping, Sweden. larsstenhammar@yahoo.com.
² Department of Pediatrics and Department of Clinical and Experimental Medicine, Norrköping Hospital, Linköping University, SE-60182, Norrköping, Sweden.

Abstract

Seventy years ago, the Swedish pediatrician Rolf Kostmann (1909-1982) was the first to report on a previous unknown lethal hereditary neutropenia in infants, Kostmann's disease. This essay presents the man behind the syndrome rather than focusing on the disease itself.

Keywords: Hereditary neutropenia; Kostmann’s disease; Rolf Kostmann.

Publication types

Biography
Historical Article
Portrait

MeSH terms

Adaptor Proteins, Signal Transducing / deficiency
Adaptor Proteins, Signal Transducing / genetics
Congenital Bone Marrow Failure Syndromes / genetics
Congenital Bone Marrow Failure Syndromes / history*
Hematology / history*
History, 20th Century
Humans
Neutropenia / congenital*
Neutropenia / genetics
Neutropenia / history
Pediatrics / history*
Sweden

Substances

Adaptor Proteins, Signal Transducing
HAX1 protein, human

Supplementary concepts

Neutropenia, Severe Congenital, Autosomal Recessive 3

Personal name as subject

Rolf Kostmann