Table of ContentsView AllTable of ContentsWhat Is the Spleen?Why the Spleen Becomes EnlargedSymptomsTreatment
Table of ContentsView All
View All
Table of Contents
What Is the Spleen?
Why the Spleen Becomes Enlarged
Symptoms
Treatment
Myeloproliferative neoplasmsare a group of diseases where the body produces too many red orwhite blood cellsorplatelets. Often, these diseases result in an enlarged spleen.
If your physician told you that you have this type of blood disorder, you might wonder, “Then why is myspleenso big?“or"What does my spleen have to do with my blood?“or even, “What is the spleen?“These are all excellent questions.
This article explains the function of the spleen, why the spleen enlarges in myeloproliferative neoplasms and describes the treatment options forsplenomegaly(enlargement of the spleen).
Hero Images / Creative RF / Getty Images
The spleen is a relatively small organ (about the size of your fist). It sits on the left side of the abdomen underneath the ribcage. The spleen consists of two types of tissue:
Before birth, the spleen is the primary producer of blood cells (hematopoiesis). However, towards the end of gestation and after birth, the bone marrow takes over this production.
Some types of myeloproliferative neoplasms result in an enlarged spleen. This enlargement occurs because the spleen can’t effectively do its job when the body produces too many blood cells. Thus, it gets overworked and becomes enlarged.
Polycythemia Vera
Inpolycythemia vera, an excessive number of red blood cells are produced, causing the blood to thicken. This increased number of red blood cells then collects in the spleen, leading to splenomegaly.
Primary Myelofibrosis
Inprimary myelofibrosis, abnormal blood cells build up, causing damage to the bone marrow. In this disease, large amounts of blood stem cells become blood cells before they’ve adequately matured.
This process causes the blood inside the marrow to become thick, slowing the ability to make blood cells. Thus, to make up for the lack of blood cells produced in the bone marrow, the liver and spleen begin to make blood cells. The spleen’s extra workload causes it to grow larger.
Essential Thrombocythemia
Inessentialthrombocythemia, too many platelets are made in the bone marrow. With this disease, platelets become sticky and can clump together, making it hard for the blood to flow. This clumping can increase the risk of blood clots, stroke, and heart attack. In addition, when the blood can’t flow adequately, the spleen may become enlarged.
Everything You Need to Know About Hypereosinophilic Syndrome
Symptoms of an Enlarged Spleen
Many people with an enlarged spleen may not know, especially if the spleen is only slightly enlarged.
Symptoms of an enlarged spleen include:
Treatment of Enlarged Spleen
If your spleen is only mildly enlarged, you may not need treatment. So, your physician will likely monitor your blood counts and the size of your spleen closely.
If your doctor recommends treatment, there are three options: medical therapy,splenectomy, and radiotherapy.
Medical Therapy
Other times, doctors use therapies to treat the underlying cause of an enlarged spleen.
These may include:
JAK-2 inhibitors, such as Ruxolitinib, can be used in some circumstances.
Second-line therapies include:
Treatment depends on your specific diagnosis, other medical problems, and the side effects of treatment.
Treatment Options for Polycythemia Vera
Splenectomy
After medication, the second treatment choice issplenectomy, which is the surgical removal of the spleen.
The most significant risk of removing the spleen is the potential for serious life-threatening infections. If you haven’t already, you should receive specific vaccines to protect you from pneumococcal and meningococcal infections before splenectomy. In addition, once the spleen is removed, you will likely be prescribed antibiotics to prevent these infections.
Radiotherapy
A third treatment option is a radiotherapy (also called radiation therapy). Radiation directed towards the spleen can help shrink it in size.
These effects are temporary, so splenic radiotherapy is consideredpalliativetreatment to minimize symptoms to improve quality of life. This therapy may be a good option for people who are not candidates for splenectomy.
Summary
An enlarged spleen doesn’t always require treatment. If you need treatment, it may include medication, surgery to remove the spleen, or radiation to reduce the spleen’s size.
A Word From Verywell
There are many things to consider when deciding if or how to treat an enlarged spleen in polycythemia vera, primary myelofibrosis, and essential thrombocythemia. So, make sure to discuss with your physician the benefits and possible side effects of these treatments.
8 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Jacobs ZG, Kaimila B, Wasswa PM, Bui T.A case of massive splenomegaly due to chronic myeloproliferative neoplasm.Malawi Med J. 2018;30(1):46-48. doi:10.4314/mmj.v30i1.10Mesa RA.How I treat symptomatic splenomegaly in patients with myelofibrosis.Blood. 2009;113(22):5394-5400. doi:10.1182/blood-2009-02-195974Aldulaimi S, Mendez AM.Splenomegaly: diagnosis and management in adults.Am Fam Physician. 2021;104(3):271-276.Vannucchi AM.How I treat polycythemia vera.Blood. 2014;124(22):3212-3220. doi:10.1182/blood-2014-07-551929Wang F, Qiu T, Wang H, Yang Q.State-of-the-art review on myelofibrosis therapies.Clin Lymphoma Myeloma Leuk. 2022;22(5):e350-e362. doi:10.1016/j.clml.2021.11.007Arikan F, Toptas T, Atagunduz IK, et al.Real-life ruxolitinib experience in intermediate-risk myelofibrosis.Blood Res. 2021;56(4):322-331. doi:10.5045/br.2021.2021101Luu S, Spelman D, Woolley IJ.Post-splenectomy sepsis: preventative strategies, challenges, and solutions.Infect Drug Resist. 2019;12:2839-2851. doi:10.2147/IDR.S179902de la Pinta C, Fernández Lizarbe E, Montero Luis Á, Domínguez Rullán JA, Sancho García S.Treatment of symptomatic splenomegaly with low doses of radiotherapy: Retrospective analysis and review of the literature.Tech Innov Patient Support Radiat Oncol. 2017;3-4:23-29. doi:10.1016/j.tipsro.2017.08.002
8 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Jacobs ZG, Kaimila B, Wasswa PM, Bui T.A case of massive splenomegaly due to chronic myeloproliferative neoplasm.Malawi Med J. 2018;30(1):46-48. doi:10.4314/mmj.v30i1.10Mesa RA.How I treat symptomatic splenomegaly in patients with myelofibrosis.Blood. 2009;113(22):5394-5400. doi:10.1182/blood-2009-02-195974Aldulaimi S, Mendez AM.Splenomegaly: diagnosis and management in adults.Am Fam Physician. 2021;104(3):271-276.Vannucchi AM.How I treat polycythemia vera.Blood. 2014;124(22):3212-3220. doi:10.1182/blood-2014-07-551929Wang F, Qiu T, Wang H, Yang Q.State-of-the-art review on myelofibrosis therapies.Clin Lymphoma Myeloma Leuk. 2022;22(5):e350-e362. doi:10.1016/j.clml.2021.11.007Arikan F, Toptas T, Atagunduz IK, et al.Real-life ruxolitinib experience in intermediate-risk myelofibrosis.Blood Res. 2021;56(4):322-331. doi:10.5045/br.2021.2021101Luu S, Spelman D, Woolley IJ.Post-splenectomy sepsis: preventative strategies, challenges, and solutions.Infect Drug Resist. 2019;12:2839-2851. doi:10.2147/IDR.S179902de la Pinta C, Fernández Lizarbe E, Montero Luis Á, Domínguez Rullán JA, Sancho García S.Treatment of symptomatic splenomegaly with low doses of radiotherapy: Retrospective analysis and review of the literature.Tech Innov Patient Support Radiat Oncol. 2017;3-4:23-29. doi:10.1016/j.tipsro.2017.08.002
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Jacobs ZG, Kaimila B, Wasswa PM, Bui T.A case of massive splenomegaly due to chronic myeloproliferative neoplasm.Malawi Med J. 2018;30(1):46-48. doi:10.4314/mmj.v30i1.10Mesa RA.How I treat symptomatic splenomegaly in patients with myelofibrosis.Blood. 2009;113(22):5394-5400. doi:10.1182/blood-2009-02-195974Aldulaimi S, Mendez AM.Splenomegaly: diagnosis and management in adults.Am Fam Physician. 2021;104(3):271-276.Vannucchi AM.How I treat polycythemia vera.Blood. 2014;124(22):3212-3220. doi:10.1182/blood-2014-07-551929Wang F, Qiu T, Wang H, Yang Q.State-of-the-art review on myelofibrosis therapies.Clin Lymphoma Myeloma Leuk. 2022;22(5):e350-e362. doi:10.1016/j.clml.2021.11.007Arikan F, Toptas T, Atagunduz IK, et al.Real-life ruxolitinib experience in intermediate-risk myelofibrosis.Blood Res. 2021;56(4):322-331. doi:10.5045/br.2021.2021101Luu S, Spelman D, Woolley IJ.Post-splenectomy sepsis: preventative strategies, challenges, and solutions.Infect Drug Resist. 2019;12:2839-2851. doi:10.2147/IDR.S179902de la Pinta C, Fernández Lizarbe E, Montero Luis Á, Domínguez Rullán JA, Sancho García S.Treatment of symptomatic splenomegaly with low doses of radiotherapy: Retrospective analysis and review of the literature.Tech Innov Patient Support Radiat Oncol. 2017;3-4:23-29. doi:10.1016/j.tipsro.2017.08.002
Jacobs ZG, Kaimila B, Wasswa PM, Bui T.A case of massive splenomegaly due to chronic myeloproliferative neoplasm.Malawi Med J. 2018;30(1):46-48. doi:10.4314/mmj.v30i1.10
Mesa RA.How I treat symptomatic splenomegaly in patients with myelofibrosis.Blood. 2009;113(22):5394-5400. doi:10.1182/blood-2009-02-195974
Aldulaimi S, Mendez AM.Splenomegaly: diagnosis and management in adults.Am Fam Physician. 2021;104(3):271-276.
Vannucchi AM.How I treat polycythemia vera.Blood. 2014;124(22):3212-3220. doi:10.1182/blood-2014-07-551929
Wang F, Qiu T, Wang H, Yang Q.State-of-the-art review on myelofibrosis therapies.Clin Lymphoma Myeloma Leuk. 2022;22(5):e350-e362. doi:10.1016/j.clml.2021.11.007
Arikan F, Toptas T, Atagunduz IK, et al.Real-life ruxolitinib experience in intermediate-risk myelofibrosis.Blood Res. 2021;56(4):322-331. doi:10.5045/br.2021.2021101
Luu S, Spelman D, Woolley IJ.Post-splenectomy sepsis: preventative strategies, challenges, and solutions.Infect Drug Resist. 2019;12:2839-2851. doi:10.2147/IDR.S179902
de la Pinta C, Fernández Lizarbe E, Montero Luis Á, Domínguez Rullán JA, Sancho García S.Treatment of symptomatic splenomegaly with low doses of radiotherapy: Retrospective analysis and review of the literature.Tech Innov Patient Support Radiat Oncol. 2017;3-4:23-29. doi:10.1016/j.tipsro.2017.08.002
Meet Our Medical Expert Board
Share Feedback
Was this page helpful?Thanks for your feedback!What is your feedback?OtherHelpfulReport an ErrorSubmit
Was this page helpful?
Thanks for your feedback!
What is your feedback?OtherHelpfulReport an ErrorSubmit
What is your feedback?
