Sickle cell anemia

Sickle cell anemias are chronic hemolytic diseases that may induce three types of acute accidents: severe anemia, severe bacterial infections, and ischemic vasoocclusive accidents (VOA) caused by sickle-shaped red blood cells obstructing small blood vessels and capillaries. Many diverse complications can occur.

Immune dysregulation

Altered immune function characterized by lymphoid proliferation, immune activation, and excessive autoreactivity often leading to autoimmune/inflammatory complications.


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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.