Literature DB >> 7527662

Effective stimulation of neutropoiesis with rh G-CSF in dyskeratosis congenita: a case report.

L Oehler1, E Reiter, J Friedl, E Kabrna, O A Haas, A Rosenkranz, K Lechner, K Geissler.   

Abstract

Dyskeratosis congenita (DC) is a rare congenital X-linked disorder. The major clinical manifestations are abnormal skin pigmentation, nail dystrophy, and leukoplakia of mucosal membranes. About 50% of the patients develop bone marrow failure, which is partly responsible for the poor prognosis. Recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) has been administered to some neutropenic patients with DC, but only a moderate stimulation of neutropoiesis has been observed. We report on a patient with DC treated with recombinant human granulocyte-colony-stimulating factor (rhG-CSF). This treatment resulted in a substantial dose-dependent increase in the neutrophil count.

Entities:  

Mesh：

Substances：

Year:  1994        PMID： 7527662     DOI： 10.1007/bf01696564

Source DB:  PubMed          Journal:  Ann Hematol        ISSN： 0939-5555            Impact factor:   3.673


  15 in total

1.  Myelokathexis treated with recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF).

Authors:  U Hess; A Ganser; H G Schnürch; G Seipelt; O G Ottmann; S Falk; G Schulz; D Hoelzer
Journal:  Br J Haematol       Date:  1992-02       Impact factor: 6.998

2.  Treatment of neutropenia associated with dyskeratosis congenita with granulocyte-macrophage colony-stimulating factor.

Authors:  C L Russo; B E Glader; R J Israel; F Galasso
Journal:  Lancet       Date:  1990-09-22       Impact factor: 79.321

3.  "Stem cell" origin of the hematopoietic defect in dyskeratosis congenita.

Authors:  J C Marsh; A J Will; J M Hows; P Sartori; P J Darbyshire; P J Williamson; D G Oscier; T M Dexter; N G Testa
Journal:  Blood       Date:  1992-06-15       Impact factor: 22.113

4.  Clastogen-induced fragility may differentiate pancytopenia of congenital dyskeratosis from Fanconi anaemia.

Authors:  A Schneider; U Mayer; E Gebhart; D Harms; J Gromball; U Glöckel; J D Beck
Journal:  Eur J Pediatr       Date:  1988-10       Impact factor: 3.183

5.  Effects of recombinant human granulocyte colony-stimulating factor on neutropenia in patients with congenital agranulocytosis.

Authors:  M A Bonilla; A P Gillio; M Ruggeiro; N A Kernan; J A Brochstein; M Abboud; L Fumagalli; M Vincent; J L Gabrilove; Karl Welte; Lawrence M Souza; Richard J O'Reilly
Journal:  N Engl J Med       Date:  1989-06-15       Impact factor: 91.245

6.  Hematopoietic progenitors in cyclic neutropenia: effect of granulocyte colony-stimulating factor in vivo.

Authors:  A R Migliaccio; G Migliaccio; D C Dale; W P Hammond
Journal:  Blood       Date:  1990-05-15       Impact factor: 22.113

7.  Granulocyte and granulocyte-macrophage colony-stimulating factors for treatment of neutropenia in glycogen storage disease type Ib.

Authors:  H Schroten; J Roesler; T Breidenbach; U Wendel; J Elsner; S Schweitzer; C Zeidler; S Burdach; M L Lohmann-Matthes; V Wahn
Journal:  J Pediatr       Date:  1991-11       Impact factor: 4.406

8.  Treatment of the hematological manifestations of dyskeratosis congenita.

Authors:  C Putterman; R Safadi; J Zlotogora; R Banura; A Eldor
Journal:  Ann Hematol       Date:  1993-04       Impact factor: 3.673

9.  Dyskeratosis congenita. Report of a large kindred.

Authors:  J M Connor; R H Teague
Journal:  Br J Dermatol       Date:  1981-09       Impact factor: 9.302

10.  Dyskeratosis congenita with hypoplastic anemia: a stem cell defect.

Authors:  M Friedland; J D Lutton; R Spitzer; R D Levere
Journal:  Am J Hematol       Date:  1985-09       Impact factor: 10.047
View more
  1 in total

Review 1.  The inherited bone marrow failure syndromes.

Authors:  S Deborah Chirnomas; Gary M Kupfer
Journal:  Pediatr Clin North Am       Date:  2013-12       Impact factor: 3.278
  1 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.