Smá af internetinu "How I treat autoimmune hemolytic anemias in adults Klaus Lechner1 and Ulrich Jäger1"

AIHA frequently has an acute onset, but in most cases it must be considered as a chronic disease with few exceptions. In primary WAIHA, there is only a low chance of spontaneous or drug-induced long-term remission or cure. Thus, the primary goal of treatment is to keep the patient clinically comfortable and to prevent “hemolytic crises” with the use of medical interventions with the lowest possible short- and long-term side effects.

Treatment of (primary) idiopathic WAIHA

First-line treatment.

The mainstay of treatment of newly diagnosed primary WAIHA is glucocorticoids (steroids). According to accepted recommendations we start treatment immediately with an initial dose of 1 mg/kg/d prednisone (PDN) orally or methylprednisolone intravenously. This initial dose is administered until a hematocrit of greater than 30% or a hemoglobin level greater than 10 g/dL (thus, not necessarily a complete normalization of hemoglobin) is reached. If this goal is not achieved within 3 weeks, second-line treatment is started because further improvement with steroid treatment is unlikely.⁹ Once the treatment goal is achieved, the dose of PDN is reduced to 20 to 30 mg/d within a few weeks. Thereafter, the PDN dose is tapered slowly (by 2.5-5 mg/d per month) under careful monitoring of hemoglobin and reticulocyte counts. An alternate-day regimen (reducing the dose gradually to nil on alternate days) may reduce the side effects of steroids. If the patient is still in remission after 3 to 4 months at a dose of 5 mg of PDN/day, an attempt to withdraw steroids is made. All patients on steroid therapy will receive bisphosphonates, vitamin D, and calcium from the beginning according to the recommendation of the American College of Rheumatology. Supplementation with folic acid is recommended. We carefully monitor blood glucose and treat patients with diabetes aggressively because diabetes is a major risk factor for treatment-related deaths from infections.³⁵ We do not treat patients with acute hemolysis routinely with heparin,³⁶ but we always consider the possibility of pulmonary embolism, because symptoms could wrongly be ascribed solely to acute anemia. At particular high risk of thromboembolism are patients with AIHA and lupus anticoagulant³⁷ or recurrent AIHA after splenectomy.³⁸