Splenectomy for ITP and blood disorders: how safe is it, and which vaccines do you need?

Why is splenectomy performed less often now?

Removing the spleen was once a standard treatment for many blood disorders. Over the past 20 years, medicines have taken over much of that role: thrombopoietin receptor agonists such as romiplostim, eltrombopag and avatrombopag for immune thrombocytopenia (ITP), rituximab for autoimmune blood disorders and lymphoma, imatinib for chronic myeloid leukaemia and ruxolitinib for myelofibrosis. Splenic artery embolisation and radiotherapy have also developed as alternatives.

The number of splenectomies performed each year at Royal North Shore Hospital fell significantly over the study period, and the average patient age fell from 57 to 39 as older patients were increasingly managed with medicines. In the final nine years, only one splenectomy was done for a malignant condition.

Who still benefits from splenectomy?

Splenectomy remains a reasonable choice for ITP that has relapsed or failed medical treatment, for people who prefer to avoid lifelong medication, and for severe ITP that does not respond to medicines. It achieves a response in about 75% of people with warm autoimmune haemolytic anaemia when medical therapy has failed, and it can help selected people with hereditary spherocytosis. In lymphoma it can treat low blood counts from an overactive spleen, relieve symptoms from a large spleen, or confirm a diagnosis, which was the reason in at least six patients here.

How is keyhole splenectomy performed?

The patient lies tilted onto the right side, and the operation uses three small ports. The surgeon first searches for accessory spleens (splenunculi), small pieces of extra splenic tissue that can keep a blood disorder active if left behind, and found and removed them in 11% of patients. The spleen is then freed with a vessel-sealing device, the main blood vessels are divided with a stapler, and the spleen is removed in a bag through the navel incision. Drains are rarely needed.

From 2004 the keyhole approach was used for every patient except those with a massively enlarged spleen, defined as longer than 20 cm, heavier than 1.5 kg or reaching down to the hip bone, where open surgery is often safer.

What are the risks of splenectomy?

Fifteen patients (21.4%) had a complication within 90 days, and complications were far less common after keyhole surgery (15.3%) than open surgery (38.8%). Four patients (5.7%) had a major complication, such as bleeding needing a return to theatre. No patient died within 90 days. One patient developed a pancreatic leak, a known risk because the tail of the pancreas sits against the spleen, and died 110 days after surgery.

Only one patient (1.4%) developed a clot in the splenic vein, compared with 5% to 37% reported in the literature. For comparison, two large international series reported deaths around surgery of 0.4% and 1.5% and complication rates of 8.9% and 12.7%.

Is surgery for a massively enlarged spleen riskier?

Of 54 patients with a recorded spleen size, 14 had massive splenomegaly. They were older, 13 of the 14 had a blood cancer, and 12 had open surgery. They lost more blood (median 200 mL compared with 50 mL) and stayed a day longer in hospital. A third had a complication, all after open operations, and none died within 90 days.

This matters for people with myelofibrosis, where splenectomy is often avoided because of high reported risks. In this series the seven patients with myelofibrosis had minimal complications and no deaths, which is relevant for those preparing for a stem cell transplant, where a very large spleen can delay recovery of blood counts.

How do vaccinations protect you after spleen removal?

The spleen filters certain bacteria from the blood, and without it a person is more vulnerable to overwhelming post-splenectomy infection, a rapid and severe infection with an estimated incidence of 0.23% to 0.42% per year and a lifetime risk of about 5%. Data from the COVID-19 pandemic also showed higher hospitalisation rates in some people without a spleen.

Every patient was vaccinated against pneumococcus, meningococcus and Haemophilus influenzae type B at least two weeks before surgery, and counselled on lifelong boosters coordinated by their GP. No patient died of overwhelming post-splenectomy infection. One patient died of sepsis three years after surgery while heavily immunosuppressed after a stem cell transplant for recurrent lymphoma.

What happens in the long term after splenectomy?

Median follow-up was 9.1 years. At last contact 41 patients (59%) were alive and well. Of 27 deaths, 14 were from the underlying blood cancer, 10 were unrelated to the spleen condition and 3 had an unclear cause.

For ITP the results were durable: 15 of 16 patients needed no ongoing treatment, a higher complete response rate than reported elsewhere. Almost all surviving patients had normal platelet counts and were well in relation to their original condition.

What are the limits of this study?

This was a retrospective series of 70 patients from one surgeon and one hospital, and some details such as spleen size and blood loss were missing for some patients, so some trends lacked statistical power. Its strengths are almost ten years of median follow-up and its size: it is the largest published series of splenectomies in Australasia since 1989.