2016, Number 620
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Rev Med Cos Cen 2016; 73 (620)
Hemoglobinuria paroxística nocturna
García RVA, Mora HGA
Language: Spanish
References: 22
Page: 659-664
PDF size: 186.82 Kb.
ABSTRACT
Paroxysmal nocturnal
hemoglobinuria is a rare
acquired chronic hemolytic
anemia, which affects both
sexes with equal frequency.
It occurs at any age and more
frequently in Southeast Asian
countries. It is the result of non
malignant clonal expansion
of hematopoietic progenitor
cells. It is characterized by
intravascular hemolytic anemia,
recurrent thrombosis and a
variable component of bone
marrow failure. It is associated
with other hematologic
disorders such as aplastic
anemia and myelodysplastic
syndrome. Flow cytometry is the
method of choice for diagnosis.
Eculizumab and allogeneic
bone marrow transplantation is
the only effective therapies.
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