Literature DB >> 8485209

Treatment of the hematological manifestations of dyskeratosis congenita.

C Putterman1, R Safadi, J Zlotogora, R Banura, A Eldor.   

Abstract

Dyskeratosis congenita is a congenital multisystem disorder, characterized by skin pigmentation, dystrophic nails, and leukoplakia. Hematologic abnormalities progressing to severe pancytopenia play a significant role in the poor prognosis of afflicted patients. We report on a patient with dyskeratosis congenita and severe aplastic anemia, complicated by life threatening infection. The patient was treated with recombinant granulocyte-macrophage colony-stimulating factor. This therapy resulted in a moderate and transient improvement in absolute neutrophil counts. Current concepts regarding the pathogenesis and etiology of dyskeratosis congenita are discussed, while reviewing the available therapeutic options.

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Year:  1993        PMID： 8485209     DOI： 10.1007/bf01703237

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


  23 in total

1.  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

2.  "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

3.  Cytogenetic abnormalities in dyskeratosis congenita--report of five cases.

Authors:  A Aguilar-Martinez; M J Lautre-Ecenarro; F Urbina-González; M C Cristóbal-Gil; P Guerra-Rodriguez; A García-Pérez
Journal:  Clin Exp Dermatol       Date:  1988-03       Impact factor: 3.470

Review 4.  Bone marrow transplantation for Fanconi anemia.

Authors:  E Gluckman; R Berger; J Dutreix
Journal:  Semin Hematol       Date:  1984-01       Impact factor: 3.851

5.  Dyskeratosis congenita (Zinsser-Cole-Engman syndrome). An autopsy case presenting with rectal carcinoma, non-cirrhotic portal hypertension, and Pneumocystis carinii pneumonia.

Authors:  K Kawaguchi; H Sakamaki; Y Onozawa; M Koike
Journal:  Virchows Arch A Pathol Anat Histopathol       Date:  1990

Review 6.  Dyskeratosis congenita associated with elevated fetal hemoglobin, X-linked ocular albinism, and juvenile-onset diabetes mellitus.

Authors:  M Reichel; A C Grix; R R Isseroff
Journal:  Pediatr Dermatol       Date:  1992-06       Impact factor: 1.588

Review 7.  Dyskeratosis congenita: report of a case and review of the literature.

Authors:  G R Ogden; E Connor; D M Chisholm
Journal:  Oral Surg Oral Med Oral Pathol       Date:  1988-05

8.  Abnormality of platelet size and T-lymphocyte proliferation in an autosomal recessive form of dyskeratosis congenita.

Authors:  H S Juneja; F F Elder; F H Gardner
Journal:  Eur J Haematol       Date:  1987-10       Impact factor: 2.997

9.  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

10.  Granulocytic macrophage colony stimulating factor restores in vitro growth of granulocyte-macrophage bone marrow hematopoietic progenitors in dyskeratosis congenita.

Authors:  R Michalevicz; S Baron; U Nordan; R Rahmani
Journal:  Isr J Med Sci       Date:  1989-04
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  1 in total

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

Authors:  L Oehler; E Reiter; J Friedl; E Kabrna; O A Haas; A Rosenkranz; K Lechner; K Geissler
Journal:  Ann Hematol       Date:  1994-12       Impact factor: 3.673
  1 in total

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