Leukemic transformation of the Marchiafava Micheli clone. Inform of a case

Authors

  • Ihosvany Ruiz Hernández Hospital Provincial Clínico Quirúrgico Docente José R. López Tabrane. Matanzas, Cuba.
  • Antonis Cano Soler Hospital Provincial Clínico Quirúrgico Docente José R. López Tabrane. Matanzas, Cuba.
  • Antonio Yosvany Méndez Alonso Hospital Provincial Clínico Quirúrgico Docente José R. López Tabrane. Matanzas, Cuba.
  • Aída García Guell Hospital Provincial Clínico Quirúrgico Docente José R. López Tabrane. Matanzas, Cuba.

Keywords:

paroxysmal hemoglobinuria, clinic, mutation. 

Abstract

The nocturnal paroxysmal hemoglobinuria, also known as Marchiafava-Micheli syndrome, is a clonal and acquired disease, caused by a somatic mutation of the PIG-A gene located in the X chromosome and modified a protein involved in the glicosilfosfatidilinositol synthesis that serves as  anchorage for many proteins of the cell membrane;  it is the only hemolytic anemia acquired by defect of the erythrocyte membrane.  It is characterized by a chronic intravascular hemolytic anemia, hemoglobinuria, hyper coagulation, cytopenia due to the marrow failure, thrombosis and rarely leukemic transformation. Having a patient with these characteristics we decided to present the case.

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Published

2013-05-15

How to Cite

1.
Ruiz Hernández I, Cano Soler A, Méndez Alonso AY, García Guell A. Leukemic transformation of the Marchiafava Micheli clone. Inform of a case. Rev Méd Electrón [Internet]. 2013 May 15 [cited 2025 Jan. 23];35(3):294-9. Available from: https://revmedicaelectronica.sld.cu/index.php/rme/article/view/1000

Issue

Vol. 35 No. 3 (2013)

Section

Presentation of cases

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