Autoimmune hemolytic anemia surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2]; Associate Editor(s)-in-Chief:  Irfan Dotani

## Overview

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Splenectomy is the only surgical management option for patients with autoimmune hemolytic anemia. The response rate is moderately high. Assessment for candidacy for splenectomy involves evaluation of the surgical risk and the risk of sepsis from encapsulated organisms. Proper vaccinations must thus be given prior to splenectomy.

## Surgery

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### Splenectomy

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* Splenectomy, or removal of the spleen, is a second-line option for autoimmune hemolytic anemia.
* Splenectomy is frequently considered for patients who have steroid-refractory or relapsed disease.
* The response rate for splenectomy is typically 66%, and nearly 20% of patients will experience a cure.[1]
* This response rate is similar to the response rate observed in immune thrombocytopenic purpura.
* Splenectomy is considered for patients requiring a daily prednisone dose of 10mg or greater or having multiple relapses.
* Splenectomy can also reduce the dose of steroids required to maintain control of the disease.
* The ideal candidate for splenectomy is one who has adequate functional status and cardiopulmonary reserve to undergo surgery.
* The decision to proceed with splenectomy is typically made jointly between the patient and physician, as surgical intervention carries inherent risks.
* Given the infectious risk for splenectomy, patients should undergo vaccination for Hemophilus influenza, Neisseria meningitides, and Streptococcus pneumonia. The three organisms are encapsulated bacteria which are normally eliminated by the spleen via complement-mediated opsonization.

### Adverse effects:

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* The adverse effects of splenectomy include:
* The inherent surgical risk
* Bleeding
* Abdominal wall abscess
* Hematoma
* Post-operative thrombosis including pulmonary embolism
* Post-operative pain
* Systemic infection (asplenic sepsis) with encapsulated organisms
* Reactive thrombocytosis

* The risk of sepsis is 3.3-5%.
* The mortality rate of asplenic sepsis is 50%.
* The inherent surgical risk of open splenectomy can be reduced by performing a laparoscopic splenectomy.

### Splenectomy vaccinations:

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* Pre-splenectomy vaccinations should be provided at least 14 days prior to splenectomy. These vaccinations include:
* Pneumococcal vaccination with PCV13 then PPSV23 at least 8 weeks later
* Meningococcal vaccination with two doses of Menactra or Menveo 2 months apart
* Meningococcal vaccination with either Trumenba (3 doses administered at 0, 1 to 2, and 6 months) or Bexsero (2 doses administered at least one month apart)
* Revaccination with PPSV every 5 years
* Revaccination with either Menactra or Menveo booster every 5 years

## References

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1. ↑ Zanella A, Barcellini W (2014). "Treatment of autoimmune hemolytic anemias". Haematologica. 99 (10): 1547–54. doi:10.3324/haematol.2014.114561. PMC 4181250. PMID 25271314.

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