Evans syndrome

A rare chronic hematologic disorder characterized by the simultaneous or sequential association of autoimmune hemolytic anemia (AIHA; a disorder in which auto-antibodies are directed against red blood cells causing anemia of varying degrees of severity) with immune thrombocytopenic purpura (ITP; a coagulation disorder in which auto-antibodies are directed against platelets causing hemorrhagic episodes) and occasionally autoimmune neutropenia, in the absence of a known underlying etiology.

Hypertension

The presence of chronic increased pressure in the systemic arterial system.


Total: 3

                      


(per page)
PMID (PMCID)
18363174
FEMALE Infant
Pulmonary hypertension in children with Evans syndrome.
Connor P, Veys P, Amrolia P, Haworth S, Ashworth M, Moledina S.
Pediatr Hematol Oncol. 2008;25(2):93-8.
The authors speculate that the pulmonary hypertension was caused by the underlying immune dysregulation and hemolysis and that Evans syndrome joins the list of other hemolytic anemias that cause pulmonary hypertension, such as sickle cell disease, thalassemia, and paroxysmal nocturnal hemoglobinuria.
18363174
FEMALE Infant
Pulmonary hypertension in children with Evans syndrome.
Connor P, Veys P, Amrolia P, Haworth S, Ashworth M, Moledina S.
Pediatr Hematol Oncol. 2008;25(2):93-8.
The authors speculate that the pulmonary hypertension was caused by the underlying immune dysregulation and hemolysis and that Evans syndrome joins the list of other hemolytic anemias that cause pulmonary hypertension, such as sickle cell disease, thalassemia, and paroxysmal nocturnal hemoglobinuria.
18363174
FEMALE Infant
Pulmonary hypertension in children with Evans syndrome.
Connor P, Veys P, Amrolia P, Haworth S, Ashworth M, Moledina S.
Pediatr Hematol Oncol. 2008;25(2):93-8.
The first patient to present, case A, received an allogeneic bone marrow transplant with subsequent cure of both Evans syndrome and pulmonary hypertension and is now on a weaning dose of sildenafil.