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2021, Number 2

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Rev Hematol Mex 2021; 22 (2)

Autoimmune acquired hemophilia A

Corona-Rodarte E, García-Hernández F, Henríquez-Santos G, Pérez-Sámano D

Language: Spanish
References: 18
Page: 116-120
PDF size: 186.34 Kb.


ABSTRACT

Background: Acquired hemophilia is a rare disease resulting from the presence of inhibitors against endogenous factor VIII (FVIII) that can lead to spontaneous and significant bleeding.
Clinical case: A 66-year-old farmer from Oaxaca was referred to our institution because of intense abdominal pain located in the right flank. The hematic cytology reported normochromic normocytic anemia, leukocytosis with neutrophilia and normal platelets. An abdominal tomography was performed and revealed a right retroperitoneal hematoma that conditioned anterior displacement of the retroperitoneal structures. He was admitted to the cardiopulmonary resuscitation area where he received fluid resuscitation and transfusion of four units of fresh frozen plasma and a blood unit. Due to manifest coagulopathy, clotting times were requested and no correction was observed after plasma administration, therefore complementary studies were performed where low factor VIII (13.8%), low factor IX (56.6%), negative lupus anticoagulant and inhibitor to factor VIII with 12 Bethesda units were observed. The diagnosis of acquired hemophilia A was integrated, and more studies were performed in order to identify the etiology.
Conclusion: Acquired hemophilia is a rare disease with high morbidity and mortality that requires a high diagnostic suspicion.


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