Is splenectomy a good option for a patient with hemoglobin H disease?

Here’s how readers responded to a You Make the Call question about performing a splenectomy on a patient with hemoglobin H disease who does not require transfusions and is taking deferasirox to control ferretin levels.


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A 35-year-old woman with hemoglobin (Hb) H disease has average long-term numbers (Hb about 7.5 g/dL, reticulocytes about 7%, bilirubin about 2.5 mg/dL). She does not need transfusions but accumulates iron and needs long-term deferasirox to prevent her ferritin from rising above 1,000 μg/mL. Would a splenectomy be a good option to improve her Hb, reduce iron overload, and improve quality of life?

Usually splenectomy does not help with the hemolysis. It marginally improves anemia at best. You have to deal with the increased risk of pneumococcal infection, possible pulmonary hypertension, and thromboembolism.

Kulumani Sivarajan, MD
Joliet Oncology-Hematology Associates
Joliet, IL

Yes, I would recommend a splenectomy in this patient to prevent the ongoing hemolysis that is resulting in increasing iron deposition in her body.

Marion Koerper, MD
UCSF School of Medicine
San Francisco, CA

My opinion is that she does not require a splenectomy. It is not indicated in the scenario whether the patient does have troublesome splenomegaly. That she is transfusion independent means that her erythropoiesis can compensate for the ongoing ineffective erythropoiesis. Therefore, splenectomy would not be very helpful to improve the degree of anemia to which the patient is now very well accustomed.

Her iron overload is from the increased absorption of iron rather than from ongoing transfusional hemosiderosis, which splenectomy would not halt. In addition, splenectomy might increase the risk of overwhelming pneumococcal sepsis.

Taking into account that this patient is well chelated with deferasirox, I would continue to use it rather than going for a splenectomy.

Thamudika Pushpakumari, MBBS
Colombo, Sri Lanka

In her case, a splenectomy might be a good option. You might consider a partial splenectomy if your surgeons can do it. In my experience, most patients with HbH disease run an Hb around 10 to 12 g/dL when not in crisis.

Carole Hurvitz, MD
Cedars-Sinai Medical Center
Los Angeles, CA

I would not do a splenectomy. It will significantly increase the risk of venothrombosis problems. I doubt that it will decrease iron absorption to any significant extent.

Edwin Forman, MD
Icahn School of Medicine at Mount Sinai
New York, NY

I don’t see any indication for splenectomy.

Geoffrey K. Sherwood, MD
Boston, MA

Nope. Well, maybe yes. I don’t like the temptation that most physicians have toward higher levels of Hb. Higher Hb does not necessarily mean better quality of life (QoL). HB H is a pro coagulant. A higher HB level may lead to higher incidence of thrombotic episodes, like portal vein thrombosis, and more pulmonary hypertension. These two are definitely not associated with better QoL.

So, when to remove the spleen in patients with HbH? My usual answer is when it’s becoming harmful, when it causes unbearable pain, when it is large enough to affect stomach size and cause cachexia.

Splenectomy certainly is not on my list when tackling iron overload. Keep in mind, in thalassemia intermedia, an example of which is HbH, serum ferritin underestimates total body iron. I would consider a lower ferritin level as target to my deferasirox therapy, or I would rely on MRI assessment of liver iron concentration.

Ahmed Hamandi, MD, MRCP
Al Kafeel Specialty Hospital
Karbala, Iraq

Splenectomy in HbH disease usually does not improve the clinical status. I do not suggest splenectomy.

Maria Cappellini, MD
University of Milan
Milan, Italy

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