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2010, Number 3

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Acta Cient Estud 2010; 8 (3)

Chronic evolution Evans syndrome in female pediatric patient

Pérez-Rojas JJ, Da Silva-De Abreu AJ
Full text How to cite this article

Language: Spanish
References: 5
Page: 83-85
PDF size: 112.83 Kb.


Key words:

Evans syndrome, autoimmune thrombocytopenia, autoimmune anaemia.

ABSTRACT

Evans syndrome is a rare disorder in which thrombocytopenia and anaemia are present; both of them of autoimmune etiology, which may occur simultaneously or consecutively. The following is the case of a four-year old female preschool patient who consults to the emergency for generalized petechiae and ecchymosis. Her parents deny any recent history of viral affection and they refer a family background of immune thrombocytopenic purpure (PTI). Paraclinical studies were developed, finding thrombocytopenia (automatic platelet count in cero and manual count in 6000 platelets per mL) in absence of anaemia (haemoglobin in 13, 3 g /dL), normal bone marrow aspirate and negative serology for anti-DNA, ANA, HIV and IGM for CMV. The patient is treated with prednisone at 25 mg /day and evolves properly but then develop hemolytic anaemia (Hb 6.9 g /dL and positive direct Coombs), establishing the diagnosis of Evans syndrome. The patient did not show viral infection previous to thrombocytopenia, neither gums nor mucous membranes bleeding, which does not coincide with the reviewed literature, which describes that such manifestations are very common in most cases of PTI. Also outstands the chronic course of both the thrombocytopenia and hemolytic anaemia, which is usually observed in older patients.


REFERENCES

  1. Luzzatto L. Anemia hemolítica y anemia consecutiva a hemorragias. En: Braunwald E, Isselbacher KJ, Petersdorf RG, Wilson JD, Martin JB, Fauci AS, editores. Harrison. Principios de medicina interna. 17 ed. México: McGraw-Hill; 2009. p. 652-63.

  2. Konkle BA. Trombocitopenia. En: Braunwald E, Isselbacher KJ, Petersdorf RG, Wilson JD, Martin JB, Fauci AS, editores. Harrison. Principios de medicina interna. 17a ed. México: McGraw-Hill; 2009. p. 719-24.

  3. Laso FJ. Anemia: estudio general; anemia con VCM bajo. En: Laso FJ. Diagnostico diferencial en medicina interna. 2 ed. México: Elsevier; 2004. p. 259-264.

  4. Segel GB. Anemia hemolítica. En: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, editores. Nelson. Tratado de pediatría. 18a ed. Barcelona, España: Elsevier; 2009. p. 2082-4.

  5. Scott JP, Montgomery RR. Púrpura trombocitopénica idiopática. En: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, editores. Nelson. Tratado de pediatría. 18a ed. Barcelona, España: Elsevier; 2009. p. 2082-4.




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Acta Cient Estud. 2010;8