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Clinical manifestations of paroxysmal nocturnal hemoglobinuria: present state and future problems.

Publication ,  Journal Article
Rosse, WF; Nishimura, J
Published in: Int J Hematol
February 2003

The clinical pathology of paroxysmal nocturnal hemoglobinuria (PNH) involves 3 complications: hemolytic anemia, thrombosis, and hematopoietic deficiency. The first 2 are clearly the result of the cellular defect in PNH, the lack of proteins anchored to the membrane by the glycosylphosphatidylinositol anchor. The hemolytic anemia results in syndromes primarily related to the fact that the hemolysis is extracellular. Thrombosis is most significant in veins within the abdomen, although a number of other thrombotic syndromes have been described. The hematopoietic deficiency may be the same as that in aplastic anemia, a closely related disorder, and may not be due to the primary biochemical defect. The relationship to aplastic anemia suggests a nomenclature that emphasizes the predominant clinical manifestations in a patient. This relationship does not explain cases that appear to be related to myelodysplastic syndromes or the transition of some cases of PNH to leukemia. Treatment, except for bone marrow transplantation, remains noncurative and in need of improvement.

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Published In

Int J Hematol

DOI

ISSN

0925-5710

Publication Date

February 2003

Volume

77

Issue

2

Start / End Page

113 / 120

Location

Japan

Related Subject Headings

  • Venous Thrombosis
  • Immunology
  • Humans
  • Hemoglobinuria, Paroxysmal
  • Hematopoiesis
  • Bone Marrow Diseases
  • Anemia, Hemolytic
  • Anemia, Aplastic
  • 1102 Cardiorespiratory Medicine and Haematology
 

Citation

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Rosse, W. F., & Nishimura, J. (2003). Clinical manifestations of paroxysmal nocturnal hemoglobinuria: present state and future problems. Int J Hematol, 77(2), 113–120. https://doi.org/10.1007/BF02983209
Rosse, Wendell F., and Junichi Nishimura. “Clinical manifestations of paroxysmal nocturnal hemoglobinuria: present state and future problems.Int J Hematol 77, no. 2 (February 2003): 113–20. https://doi.org/10.1007/BF02983209.
Rosse, Wendell F., and Junichi Nishimura. “Clinical manifestations of paroxysmal nocturnal hemoglobinuria: present state and future problems.Int J Hematol, vol. 77, no. 2, Feb. 2003, pp. 113–20. Pubmed, doi:10.1007/BF02983209.
Journal cover image

Published In

Int J Hematol

DOI

ISSN

0925-5710

Publication Date

February 2003

Volume

77

Issue

2

Start / End Page

113 / 120

Location

Japan

Related Subject Headings

  • Venous Thrombosis
  • Immunology
  • Humans
  • Hemoglobinuria, Paroxysmal
  • Hematopoiesis
  • Bone Marrow Diseases
  • Anemia, Hemolytic
  • Anemia, Aplastic
  • 1102 Cardiorespiratory Medicine and Haematology