hematology

idiopathic thrombocytopenic purpura (ITP)

acquired hemorrhagic disorder characterized by thrombocytopenia, purpura, and normal bone marrow with increase in large immature platelets

petechiae, bruising, bleeding from mucous membranes, prolonged bleeding

s/s of ITP

supportive treatment, IVIG

management of ITP

sickle cell anemia

autosomal recessive disorder that involves partial or complete replacement of normal Hgb with abnormal Hgb (HgbS), most prevalent in African Americans

chronic anemia, pain in affected area, hypoxia, ischemia, damaged tissues and organs, blood pooling, non-functioning spleen by 5

s/s of SCA

decrease stress, meds to increase normal Hgb, pain meds, prophylactic penicillin for 1st 5 years, hydration

management of SCA

vasooclusive sickle cell crisis

type of sickle cell crisis that involves stasis of blood with clumping of cells, leading to ischemia, tissue death, and pain

acute chest syndrome

when VOC or infection causes sickling in lungs, presents like pneumonia

chest pain, fever, cough, tachypnea, wheezing, hypoxia

s/s of acute chest syndrome

aggressive tx of infection, prophylactic penicillin, blood transfusion, hydration, warm packs, monitor reticulocyte count

management of acute chest syndrome

hemosiderosis

iron overload leading to iron in tissues, caused by frequent blood transfusions

deferasirox

tx of hemosiderosis

beta thalassemia

anemia from defective Hgb synthesis and shortened life span of RBCs, leads to chronic hypoxia

headache, irritability, bone pain, exercise intolerance, anorexia, epistaxis

s/s of beta thalassemia

monthly blood transfusions

treatment of beta thalassemia

special needs of preterm infants, periods of rapid growth, babies with excessive milk intake, adolescents

those at risk for developing iron deficiency anemia

hemophilia A

deficiency of clotting factor VIII, sex linked (males more affected)

hemarthrosis, bruising, epistaxis, bleeding after procedures

s/s of hemophilia A

DDVAP, replace missing clotting factors, transfusion, prevent bleeding

management of hemophilia A