In a patient hospitalized for several days with a refractory pain crisis would you ever consider transfusion, either simple or possibly exchange in an attempt to get the patient over the crisis?

While chronic transfusion therapy has been shown to prevent acute vaso-occlusive pain episodes (see studies that used chronic transfusion to prevent primary and secondary stroke), there are limited data to determine if they are effective for treatment of acute pain caused by vaso-occlusion. There has been one randomized pilot trial (n=10 enrolled) in the last decade (Kelly et al., PMID 25833727) that showed non-significant improvement in pain outcomes when transfusion was given within 24 hours of admission.

While it theoretically could make sense to offer transfusion, pain from acute vaso-occlusion is complex and also involves endothelial and inflammatory responses that may not respond to transfusion. There are also risks and other underlying disease morbidities to consider, such as iron status, transfusion reactions, and alloimmunization (which may be more likely to occur when a transfusion is given in the acute, hyper-inflammatory setting like vaso-occlusive crisis). There may also be other underlying issues that may be leading to refractory pain that must be evaluated and considered, such as osteonecrosis and if an underlying chronic pain syndrome may be present, as these may limit the theoretical improvement in pain the patient could receive with a transfusion.

Given the lack of data and the potentially significant risks, transfusion for acute refractory pain in sickle cell disease cannot be routinely recommended. Transfusion, however, should be given to treat symptomatic and acute anemia that may co-exist during the acute pain episode. While treatment modalities for acute pain in SCD are limited, consideration of other pharmacologic treatments, such as ketamine or a nerve block (if the pain location is amenable to this), and non-pharmacologic treatments, may be potential treatment options to consider adding to IV opioid therapy that may have some potential to improve pain, without the same risk-profile as transfusion. Finally, initiating and/or optimizing preventative therapies (i.e., hydroxyurea) to prevent future pain episodes should be prioritized.

The 2020 ASH guidelines provide a nice summary of what is known about this point, all in agreement with the response from Dr. @Dr. First Last. Absent compelling or even suggestive data, and given potential harms, it is difficult to routinely offer transfusion as a response to this situation. However, as pointed out, use of transfusion for other indications that complicate this specific presentation is not contraindicated and may contribute to overall improvement.