HEMATOLOGY, ONCOLOGY, ALLERGY,AND IMMUNOLOGYTREATMENT
■ Supportive care if hemolysis occurs
■ Avoid precipitants.
Sickle Cell Disease
This autosomal recessive disease is caused by an abnormal structure to both
beta-globin chains of hemoglobin, resulting in sickling of deoxygenated
RBCs.
Patients with sickle cell trait have the defect in only one beta-globin chain
and sickle only under conditions of severehypoxia.
PATHOPHYSIOLOGY
■ Deoxygenated RBC takes on sickle shape →obstruction of RBC in micro-
circulation →vaso-occlusive ischemic tissue injury.
■ Sickled cells are sequestered and destroyed by liver and spleen.
VASO-OCCLUSIVEPAINCRISIS
Most common manifestation of sickle cell crisis
SYMPTOMS/EXAM
■ Pain in ribs, back, limbs
■ Pattern of pain is usually consistent from crisis to crisis and may last 5–7 days.
DIAGNOSIS
■ CBC: Evaluate for aplastic crisis.
■ Reticulocyte count: Evaluate for aplastic crisis.
■ UA, CXR, cultures: Evaluate for infection.
TREATMENT
■ Fluid hydration (PO if able to tolerate, otherwise IV hydration)
■ Analgesia
■ Antibiotics if indicated
■ Folic acid
ACUTECHESTSYNDROME
This disease is caused by a combination of pulmonary vascular infarctionand/or
infection, most often by Chlamydia,Mycoplasma, RSV, Staphylococcus aureus,
andStreptococcus pneumoniae.
SYMPTOMS/EXAM
■ Chest pain, fever, cough
■ Lung consolidation, hypoxia
DIAGNOSIS
■ CXR: New pulmonary infiltrate
The classic story for hemolysis
due to G6PD deficiency is the
African American male
soldier in Vietnam receiving
treatment for malaria.Patients should avoid hypoxia
or dehydration because they
may precipitate or exacerbate
sickling.