Low plasma levels of the protein pro-LL-37 as an early indication of severe disease in patients with chronic neutropenia

British Journal of Haematology

Volumn 137, Issue 2, 2007, Pages 166-169

  Karlsson, Jenny ^a   Carlsson, Göran ^b   Ramme, Kim Göransdotter ^b   Hägglund, Hans ^b   Fadeel, Bengt ^a   Nordenskjöld, Magnus ^b   Henter, Jan Inge ^b   Palmblad, Jan ^b   Pütsep, Katrin ^a   Andersson, Mats ^a  

^a KAROLINSKA INSTITUTE   (Sweden)

^b KAROLINSKA UNIVERSITY HOSPITAL   (Sweden)

Author keywords

ELA2; HAX1; LL 37; Myelopoiesis; Severe congenital neutropenia

Indexed keywords

PROTEIN; PROTEIN PRO LL 37; UNCLASSIFIED DRUG;

ADOLESCENT; ADULT; ARTICLE; AUTOIMMUNE DISEASE; BLOOD LEVEL; CHILD; CLINICAL ARTICLE; CONTROLLED STUDY; DIFFERENTIAL DIAGNOSIS; DISEASE SEVERITY; HUMAN; NEUTROPENIA; NEUTROPHIL; PRIORITY JOURNAL;

ADOLESCENT; ADULT; ANTIMICROBIAL CATIONIC PEPTIDES; AUTOIMMUNE DISEASES; BIOLOGICAL MARKERS; CHILD; CHILD, PRESCHOOL; CHRONIC DISEASE; DIAGNOSIS, DIFFERENTIAL; ELECTROPHORESIS, POLYACRYLAMIDE GEL; FEMALE; HUMANS; IMMUNOBLOTTING; LEUKOCYTE ELASTASE; MALE; MUTATION; NEUTROPENIA;

EID: 33947517620     PISSN: 00071048     EISSN: 13652141     Source Type: Journal    
DOI: 10.1111/j.1365-2141.2007.06530.x     Document Type: Article

References (12)

1. Ancliff, P.J., Gale, R.E., Liesner, R., Hann, I.M. & Linch, D.C. (2001) Mutations in the ELA2 gene encoding neutrophil elastase are present in most patients with sporadic severe congenital neutropenia but only in some patients with the familial form of the disease. Blood, 98, 2645-2650.
2. Carlsson, G. & Fasth, A. (2001) Infantile genetic agranulocytosis, morbus Kostmann: presentation of six cases from the original "Kostmann family" and a review. Acta Paediatrica, 90, 757-764.
3. Carlsson, G., Aprikyan, A.A., Ericson, K.G., Stein, S., Makaryan, V., Dale, D.C., Nordenskjold, M., Fadeel, B., Palmblad, J. & Henter, J.I. (2006) Neutrophil elastase and granulocyte colony-stimulating factor receptor mutation analyses and leukemia evolution in severe congenital neutropenia patients belonging to the original Kostmann family in northern Sweden. Haematologica, 91, 589-595.
4. Dale, D.C., Person, R.E., Bolyard, A.A., Aprikyan, A.G., Bos, C., Bonilla, M.A., Boxer, L.A., Kannourakis, G., Zeidler, C., Welte, K., Benson, K.F. & Horwitz, M. (2000) Mutations in the gene encoding neutrophil elastase in congenital and cyclic neutropenia. Blood, 96, 2317-2322.
5. Karlstrom, R., Gustafson, R. & Palmblad, J. (2001) Chronic mild neutropenia in adults: relation to IgG3 deficiency and infection susceptibility. Journal of Internal Medicine, 250, 342-347.
6. Klein, C., Grudzien, M., Appaswamy, G., Germeshausen, M., Sandrock, I., Schaffer, A.A., Rathinam, C., Boztug, K., Schwinzer, B., Rezaei, N., Bohn, G., Melin, M., Carlsson, G., Fadeel, B., Dahl, N., Palmblad, J., Henter, J.I., Zeidler, C., Grimbacher, B. & Welte, K. (2007) HAX1 deficiency causes autosomal recessive severe congenital neutropenia (Kostmann disease). Nature Genetics, 39, 86-92.
7. Maheshwari, A., Christensen, R.D. & Calhoun, D.A. (2002) Immunemediated neutropenia in the neonate. Acta Paediatrica. Supplement, 91, 98-103.
8. Pütsep, K., Carlsson, G., Boman, H.G. & Andersson, M. (2002) Deficiency of antibacterial peptides in patients with morbus Kostmann: an observation study. Lancet, 360, 1144-1149.
9. Rosenberg, P.S., Alter, B.P., Bolyard, A.A., Bonilla, M.A., Boxer, L.A., Cham, B., Fier, C., Freedman, M., Kannourakis, G., Kinsey, S., Schwinzer, B., Zeidler, C., Welte, K. & Dale, D.C. (2006) The incidence of leukemia and mortality from sepsis in patients with severe congenital neutropenia receiving long-term G-CSF therapy. Blood, 107, 4628-4635.
10. Sorensen, O., Cowland, J.B., Askaa, J. & Borregaard, N. (1997) An ELISA for hCAP-18, the cathelicidin present in human neutrophils and plasma. Journal of Immunological Methods, 206, 53-59.
11. Taniuchi, S., Masuda, M., Hasui, M., Tsuji, S., Takahashi, H. & Kobayashi, Y. (2002) Differential diagnosis and clinical course of autoimmune neutropenia in infancy: comparison with congenital neutropenia. Acta Paediatrica, 91, 1179-1182.
12. Welte, K., Zeidler, C. & Dale, D.C. (2006) Severe congenital neutropenia. Seminars in Hematology, 43, 189-195.