Whole blood phlebotomy is considered the treatment of choice for patients with iron overload due to HH. Therapeutic phlebotomy for HH is generally well-tolerated, low-cost, and effective in reducing iron levels.
Each unit of blood contains approximately 200 to 250 mg of iron. As erythrocytes are removed by phlebotomy, iron stores are mobilized and utilized in the production of new, circulating erythrocytes. Stored iron is removed through periodic phlebotomies until iron-deficient erythropoiesis is induced.
The initial, or iron reduction, phase of treatment typically consists of removing one unit (450 mL) of whole blood once or twice weekly. The patient's hemoglobin and hematocrit must be checked prior to phlebotomy to ensure that the patient is not anemic. A sample for serum ferritin is also collected at this time.
Initial treatment goals include inducing iron deficient hematopoiesis without the development of debilitating symptoms of anemia. A hemoglobin concentration of 10.0 to 12.0 g/dL is often used as a target range. The initial treatment phase continues until excess stored iron is removed and ferritin levels decrease to approximately 50 ng/mL. It may take up to three years for this to be accomplished.(17) Ferritin and hemoglobin levels are periodically monitored during this treatment phase.
The number of phlebotomies needed to reduce iron levels and induce anemia is related to the degree of initial iron overload.
Erythrocytapheresis is a newer therapeutic alternative by which only erythrocytes are removed. Platelets and plasma are returned to the patient. An advantage to this approach over whole blood phlebotomy is that greater amounts of iron may be removed per phlebotomy session. (18)
Patients may be referred to a hematologist or gastroenterologist during the initial treatment phase. Many patients receive therapeutic phlebotomy services in a hospital or doctor's office, but patients may also undergo phlebotomy at a blood center.