Refractoriness to red blood cell transfusion therapy due to hypersplenism.

BACKGROUND

A 55-year-old male presented with myelodysplastic/myeloproliferative neoplasm and severe splenomegaly. The patient is blood group O, D+ with a negative indirect antiglobulin test. Transfusion of 5 units of red blood cells (RBCs) increased the hemoglobin (Hb) level from 6.7 to 7.2 g/dL. No active bleeding or hemolysis was evident. The patient was readmitted 1 week later with a Hb level of 3.3 g/dL. An additional 6 units of RBCs showed only an increase from 3.3 to 3.5 g/dL. Partial splenic embolization was performed, which resulted in a stabilization of the Hb level at approximately 7 g/dL. Because of this, total splenectomy was performed, which resulted in a gradual increase in Hb level to approximately 13 g/dL. The patient remains transfusion independent 160 days postsplenectomy.

RESULTS

RBC transfusion increases Hb concentration by 1 g/dL per unit in a typical adult. This increase is attenuated in the presence of ongoing hemolysis or active blood loss. Occasionally, a low-RBC-volume unit transfused to a recipient with a large intravascular blood volume may show an unexpectedly small increase. In rare situations, however, the etiology of a greatly attenuated response is more perplexing. The pattern of Hb concentration posttransfusion was suggestive of splenic sequestration in our patient.

CONCLUSION

Severe refractoriness to RBC transfusion attributable to severe hypersplenism is a rare event. Our case suggests that splenic artery embolization may be a useful initial approach in individual cases and a potential predictor of the utility of a subsequent surgical splenectomy.