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Splenomegaly
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<h2 id="definition">Definition</h2> <p>The standard system for classifying splenomegaly on <a href="/facts/Radiography/ab1bvq3q">radiography</a> is:<a class="footnote-ref" id="fnref:3" href="#fn:3"><sup>3</sup></a><a class="footnote-ref" id="fnref:4" href="#fn:4"><sup>4</sup></a> </p> <ul><li>Normal (not splenomegaly): the largest dimension is less than 11 cm</li> <li>Moderate splenomegaly: the largest dimension is between 11 and 20 cm</li> <li>Severe splenomegaly: the largest dimension is greater than 20 cm</li></ul> <p>Also, a <a href="/facts/Cutoff_(reference_value)/IqNt7ELQ">cutoff</a> of a craniocaudal height of 13 cm is also used to define splenomegaly.<a class="footnote-ref" id="fnref:5" href="#fn:5"><sup>5</sup></a> In addition, individual intervals have been established: </p> 90% <a href="/facts/Confidence_interval/NS8mG5UE">confidence interval</a> of maximum spleen length by <a href="/facts/Abdominal_ultrasonography/tLjP82VP">abdominal ultrasonography</a> by height of the person<a class="footnote-ref" id="fnref:6" href="#fn:6"><sup>6</sup></a><table><tbody><tr><th rowspan="2">Height</th><th colspan="2">Spleen length</th></tr><tr><th>Women</th><th>Men</th></tr><tr><td>155 – 159 cm</td><td>6.4 – 12 cm</td><td></td></tr><tr><td>160 – 164 cm</td><td>7.4 - 12.2 cm</td><td>8.9 - 11.3 cm</td></tr><tr><td>165 – 169 cm</td><td>7.5 – 11.9 cm</td><td>8.5 – 12.5 cm</td></tr><tr><td>170 – 174 cm</td><td>8.3 – 13.0 cm</td><td>8.6 – 13.1 cm</td></tr><tr><td>175 – 179 cm</td><td>8.1 – 12.3 cm</td><td>8.6 – 13.4 cm</td></tr><tr><td>180 – 184 cm</td><td></td><td>9.3 – 13.4 cm</td></tr><tr><td>185 – 189 cm</td><td></td><td>9.3 – 13.6 cm</td></tr><tr><td>190 – 194 cm</td><td></td><td>9.7 – 14.3 cm</td></tr><tr><td>195 – 199 cm</td><td></td><td>10.2 – 14.4 cm</td></tr></tbody></table> <table><tbody><tr><th>Age</th><th>Cutoff<a class="footnote-ref" id="fnref:7" href="#fn:7"><sup>7</sup></a></th></tr><tr><td>3 months</td><td>6.0 cm</td></tr><tr><td>6 months</td><td>6.5 cm</td></tr><tr><td>12 months</td><td>7.0 cm</td></tr><tr><td>2 years</td><td>8.0 cm</td></tr><tr><td>4 years</td><td>9.0</td></tr><tr><td>6 years</td><td>9.5 cm</td></tr><tr><td>8 years</td><td>10.0 cm</td></tr><tr><td>10 years</td><td>11.0 cm</td></tr><tr><td>12 years</td><td>11.5 cm</td></tr><tr><td>15 years</td><td><ul><li>12.0 cm for girls</li><li>13.0 cm for boys</li></ul></td></tr></tbody></table> <p>For children, the cutoffs for splenomegaly are given in this table, when measuring the greatest length of the spleen between its dome and its tip, in the <a href="/facts/Coronal_plane/JeB18qFE">coronal plane</a> through its hilum while breathing quietly.<a class="footnote-ref" id="fnref:8" href="#fn:8"><sup>8</sup></a> </p><p>At <a href="/facts/Autopsy/GzSXuCH2">autopsy</a>, splenomegaly can be defined as a spleen weight above the upper limit of the standard <a href="/facts/Reference_range/IqNt7ELQ">reference range</a> of 230 g (8.1 oz).<a class="footnote-ref" id="fnref:9" href="#fn:9"><sup>9</sup></a><a class="footnote-ref" id="fnref:10" href="#fn:10"><sup>10</sup></a> </p><p>Splenomegaly refers strictly to spleen enlargement, and is distinct from hypersplenism, which connotes overactive function by a spleen of any size. Splenomegaly and hypersplenism should not be confused. Each may be found separately, or they may coexist. Clinically, if a spleen is palpable (felt via <a href="/facts/Abdominal_examination/yJUoxlqP">external examination</a>), it means it is enlarged as it has to undergo at least twofold enlargement to become palpable. However, the tip of the spleen may be palpable in a newborn baby up to three months of age.<a class="footnote-ref" id="fnref:11" href="#fn:11"><sup>11</sup></a> </p> <h2 id="signs-and-symptoms">Signs and symptoms</h2> <p><a href="/facts/Symptoms/BzP6MrTN">Symptoms</a> may include <a href="/facts/Abdominal_pain/aF6gPB0G">abdominal pain</a>, chest pain, chest pain similar to <a href="/facts/Pleuritic/JEHqK0wu">pleuritic</a> pain when stomach, bladder or bowels are full, back pain, early satiety due to splenic encroachment, or the symptoms of <a href="/facts/Anemia/AgEPPmg8">anemia</a> due to accompanying <a href="/facts/Cytopenia/FN9gYLPE">cytopenia</a>. </p><p><a href="/facts/Medical_sign/BzP6MrTN">Signs</a> of splenomegaly may include a palpable left upper quadrant <a href="/facts/Abdominal_mass/tvy39oGT">abdominal mass</a> or splenic rub. It can be detected on <a href="/facts/Physical_examination/qzI0BK5P">physical examination</a> by using <a href="/facts/Castell%27s_sign/IJ4UFSsF">Castell's sign</a>, <a href="/facts/Traube%27s_space/Ktva0lEd">Traube's space</a> percussion or <a href="/facts/Nixon%27s_sign/BQwPoBjx">Nixon's sign</a>, but an <a href="/facts/Ultrasound/31Pa5IH9">ultrasound</a> can be used to confirm diagnosis. In patients where the likelihood of splenomegaly is high, the physical exam is not sufficiently sensitive to detect it; abdominal imaging is indicated in such patients.<a class="footnote-ref" id="fnref:12" href="#fn:12"><sup>12</sup></a> </p><p>In cases of <a href="/facts/Infectious_mononucleosis/pTwDw4VF">infectious mononucleosis</a> splenomegaly is a common symptom and health care providers may consider using <a href="/facts/Abdominal_ultrasonography/tLjP82VP">abdominal ultrasonography</a> to get insight into a person's condition.<a class="footnote-ref" id="fnref:13" href="#fn:13"><sup>13</sup></a> However, because spleen size varies greatly, ultrasonography is not a valid technique for assessing spleen enlargement and should not be used in typical circumstances or to make routine decisions about fitness for playing sports.<a class="footnote-ref" id="fnref:14" href="#fn:14"><sup>14</sup></a> </p> <h2 id="causes">Causes</h2> <p>The most common causes of splenomegaly in developed countries are <a href="/facts/Infectious_mononucleosis/pTwDw4VF">infectious mononucleosis</a>, splenic infiltration with <a href="/facts/Cancer/PVW6n1D0">cancer</a> cells from a <a href="/facts/Hematological_malignancy/tzyyShka">hematological malignancy</a> and <a href="/facts/Portal_hypertension/REmqnpER">portal hypertension</a> (most commonly secondary to <a href="/facts/Liver_disease/NHtuS0gU">liver disease</a>, and sarcoidosis). Splenomegaly may also come from bacterial infections, such as <a href="/facts/Syphilis/fdnGLyLT">syphilis</a> or an infection of the heart's inner lining (<a href="/facts/Endocarditis/SlaH14C3">endocarditis</a>).<a class="footnote-ref" id="fnref:15" href="#fn:15"><sup>15</sup></a> Splenomegaly also occurs in mammals parasitized by <i><a href="/facts/Cuterebra_fontinella/3U7S34Ag">Cuterebra fontinella</a></i>.<a class="footnote-ref" id="fnref:16" href="#fn:16"><sup>16</sup></a> </p><p>The possible causes of moderate splenomegaly (spleen <1000 g) are many, and include: </p> Splenomegaly grouped on the basis of the pathogenic mechanism<table><tbody><tr><th>Increased function</th><th>Abnormal blood flow</th><th>Infiltration</th></tr><tr><td><i>Removal of defective RBCs</i><ul><li><a href="/facts/Spherocytosis/9ckd4xyv">spherocytosis</a></li><li><a href="/facts/Thalassemia/NF7GIJsJ">thalassemia</a></li><li><a href="/facts/Hemoglobinopathies/ztfHkna3">hemoglobinopathies</a></li><li>nutritional <a href="/facts/Anemias/AgEPPmg8">anemias</a></li><li>early <a href="/facts/Sickle_cell_anemia/93LT7ATq">sickle cell anemia</a></li></ul><p><i>Immune hyperplasia</i></p><p><i>Response to infection (viral, bacterial, fungal, parasitic)</i></p><ul><li><a href="/facts/Mononucleosis/pTwDw4VF">mononucleosis</a>, <a href="/facts/AIDS/RqLIEMuJ">AIDS</a>,<a class="footnote-ref" id="fnref:17" href="#fn:17"><sup>17</sup></a> <a href="/facts/Viral_hepatitis/DjT90Yhf">viral hepatitis</a></li><li>subacute <a href="/facts/Bacterial_endocarditis/vLgg8K1V">bacterial endocarditis</a>, bacterial <a href="/facts/Sepsis/n52bkffJ">sepsis</a></li><li>splenic <a href="/facts/Abscess/BW3TH8kb">abscess</a>, <a href="/facts/Typhoid_fever/bKPTIYLl">typhoid fever</a></li><li><a href="/facts/Brucellosis/Cq445r85">brucellosis</a>, <a href="/facts/Leptospirosis/4MSPDpnY">leptospirosis</a>, <a href="/facts/Tuberculosis/ggPyovAl">tuberculosis</a></li><li><a href="/facts/Histoplasmosis/FfMdb5m8">histoplasmosis</a></li><li><a href="/facts/Malaria/rN3g58CX">malaria</a>, <a href="/facts/Leishmaniasis/L5v8Thy1">leishmaniasis</a>, <a href="/facts/Trypanosomiasis/nqcKaxpF">trypanosomiasis</a></li><li><a href="/facts/Ehrlichiosis/ut0RXn3r">ehrlichiosis</a><a class="footnote-ref" id="fnref:18" href="#fn:18"><sup>18</sup></a></li></ul><p><i>Disordered immunoregulation</i></p><ul><li><a href="/facts/Rheumatoid_arthritis/qFK4Xwv7">rheumatoid arthritis</a>, including cases of <a href="/facts/Felty%27s_syndrome/GdQ4gAUY">Felty's syndrome</a></li><li><a href="/facts/Systemic_lupus_erythematosus/kfHYawre">systemic lupus erythematosus</a></li><li><a href="/facts/Serum_sickness/8TrV2bLx">serum sickness</a></li><li><a href="/facts/Familial_hemophagocytic_lymphohistiocytosis/JuRzt0u5">familial hemophagocytic lymphohistiocytosis</a></li><li><a href="/facts/Autoimmune_hemolytic_anemia/cQeKSWIK">autoimmune hemolytic anemia</a></li><li><a href="/facts/Autoimmune_lymphoproliferative_syndrome/sw2u3Evx">autoimmune lymphoproliferative syndrome</a>, an autosomal dominant disorder</li><li><a href="/facts/Sarcoidosis/qgV7Uoe8">sarcoidosis</a></li><li>drug reactions</li></ul><p><i>Extramedullary hematopoiesis</i></p><ul><li><a href="/facts/Myelofibrosis/2UHM1T5R">myelofibrosis</a></li><li>marrow infiltration by <a href="/facts/Tumors/K0ytzmPz">tumors</a>, <a href="/facts/Leukemias/WpEfW0Qh">leukemias</a></li><li>marrow damage by <a href="/facts/Radiation/RwMocyeq">radiation</a>, <a href="/facts/Toxins/11oUNk7n">toxins</a></li></ul></td><td><i>Organ Failure</i><ul><li><a href="/facts/Cirrhosis/bkLuLvCD">cirrhosis</a></li></ul><p><i>Vascular</i></p><ul><li><a href="/facts/Hepatic_vein_obstruction/MDh0NQVn">hepatic vein obstruction</a></li><li><a href="/facts/Portal_vein_thrombosis/PPFPF4bw">portal vein obstruction</a></li><li><a href="/facts/Budd%E2%80%93Chiari_syndrome/MDh0NQVn">Budd–Chiari syndrome</a></li><li><a href="/facts/Splenic_vein_obstruction/FFmXaggy">splenic vein obstruction</a></li></ul><p><i>Infections</i></p><ul><li>hepatic <a href="/facts/Schistosomiasis/qXtvvvUo">schistosomiasis</a></li><li>hepatic <a href="/facts/Echinococcosis/0FnVxknd">echinococcosis</a></li></ul></td><td><i>Metabolic diseases</i><ul><li><a href="/facts/Gaucher_disease/KbNa4ruF">Gaucher disease</a></li><li><a href="/facts/Niemann%E2%80%93Pick_disease/uTMDX7k1">Niemann–Pick disease</a></li><li><a href="/facts/Alpha-mannosidosis/RGkM8htn">alpha-mannosidosis</a></li><li><a href="/facts/Hurler_syndrome/I4eHNBjD">Hurler syndrome</a> and other <a href="/facts/Mucopolysaccharidoses/lD9ECGHX">mucopolysaccharidoses</a><a class="footnote-ref" id="fnref:19" href="#fn:19"><sup>19</sup></a></li><li><a href="/facts/Amyloidosis/FeuTXVdn">amyloidosis</a></li><li><a href="/facts/Tangier_disease/79sptP5a">Tangier disease</a></li></ul><p><i>Benign and malignant "infiltrations"</i></p><ul><li><a href="/facts/Leukemias/WpEfW0Qh">leukemias</a> (acute, chronic, lymphoid, and myeloid)</li><li>lymphomas (<a href="/facts/Hodgkin%27s_lymphoma/mCeqnGty">Hodgkins</a> and <a href="/facts/Non-Hodgkin_lymphoma/KIFxt74F">non-Hodgkin's</a>)</li><li><a href="/facts/Myeloproliferative_disease/Wt1JuLC6">myeloproliferative disease</a></li><li><a href="/facts/Metastatic/4dadbm6z">metastatic</a> tumors (commonly <a href="/facts/Melanoma/qdT7E97Y">melanoma</a>)</li><li><a href="/facts/Histiocytosis_X/1Lwp7u1u">histiocytosis X</a></li><li><a href="/facts/Hemangioma/tX9PUGES">hemangioma</a>, <a href="/facts/Lymphangioma/IrjI7l5c">lymphangioma</a></li><li>splenic <a href="/facts/Cysts/N1n2ogU9">cysts</a></li><li><a href="/facts/Hamartomas/0QRzgc9N">hamartomas</a></li><li><a href="/facts/Eosinophilic_granuloma/vQ11e5Nf">eosinophilic granuloma</a></li><li><a href="/facts/Littoral_cell_angioma/9K3kJrdW">littoral cell angioma</a><a class="footnote-ref" id="fnref:20" href="#fn:20"><sup>20</sup></a><a class="footnote-ref" id="fnref:21" href="#fn:21"><sup>21</sup></a><a class="footnote-ref" id="fnref:22" href="#fn:22"><sup>22</sup></a></li></ul></td></tr></tbody></table> <p>The causes of massive splenomegaly (spleen >1000 g) are </p> <ul><li><a href="/facts/Chronic_myelogenous_leukemia/FA9TdgQn">chronic myelogenous leukemia</a></li> <li><a href="/facts/Myelofibrosis/2UHM1T5R">myelofibrosis</a></li> <li><a href="/facts/Malaria/rN3g58CX">malaria</a></li> <li><a href="/facts/Splenic_marginal_zone_lymphoma/PhVKImd0">splenic marginal zone lymphoma</a></li></ul> <h2 id="pathophysiology">Pathophysiology</h2> <p>Splenomegaly can be classified based on its pathophysiologic mechanism: </p> <ul><li>Congestive, by pooled blood (e.g., <a href="/facts/Portal_hypertension/REmqnpER">portal hypertension</a>)</li> <li>Infiltrative, by invasion by cells foreign to the splenic environment (e.g., <a href="/facts/Metastases/4dadbm6z">metastases</a>, myeloid neoplasms, <a href="/facts/Lipid_storage_disease/pebMBYl2">lipid storage diseases</a>)</li> <li><a href="/facts/Immune/vLPpzSNL">Immune</a>, by an increase in immunologic activity and subsequent <a href="/facts/Hyperplasia/gDv6kB4j">hyperplasia</a> (e.g., <a href="/facts/Endocarditis/SlaH14C3">endocarditis</a>, <a href="/facts/Sarcoidosis/qgV7Uoe8">sarcoidosis</a>, <a href="/facts/Rheumatoid_arthritis/qFK4Xwv7">rheumatoid arthritis</a>)</li> <li><a href="/facts/Neoplastic/K0ytzmPz">Neoplastic</a>, when resident immune cells originate a <a href="/facts/Neoplasm/K0ytzmPz">neoplasm</a> (e.g., <a href="/facts/Lymphoma/418wcMEl">lymphoma</a>).</li></ul> <p><a class="footnote-ref" id="fnref:23" href="#fn:23"><sup>23</sup></a> </p> <h2 id="diagnosis">Diagnosis</h2> <p>Abdominal CT is the most accurate. The spleen needs to be 2–3 times larger than normal to be palpable below the costal margin in physical examination. </p> <h2 id="treatment">Treatment</h2> <p>If the splenomegaly underlies hypersplenism, a <a href="/facts/Splenectomy/oFGhehpQ">splenectomy</a> is indicated and will correct the hypersplenism. However, the underlying cause of the hypersplenism will most likely remain; consequently, a thorough diagnostic workup is still indicated, as, leukemia, lymphoma and other serious disorders can cause hypersplenism and splenomegaly. After splenectomy, however, patients have an increased risk for infectious diseases. </p><p>Patients undergoing splenectomy should be <a href="/facts/Vaccine/QcXzLug2">vaccinated</a> against <i><a href="/facts/Haemophilus_influenzae/wqIpoDI1">Haemophilus influenzae</a></i>, <i><a href="/facts/Streptococcus_pneumoniae/B5XxK27j">Streptococcus pneumoniae</a></i>, and <i><a href="/facts/Meningococcus/u4SqwHuc">Meningococcus</a></i>. They should also receive annual <a href="/facts/Influenza_vaccination/6J8BBKN1">influenza vaccinations</a>. Long-term <a href="/facts/Prophylaxis/pWGlacXu">prophylactic</a> <a href="/facts/Antibiotic/sF1kIfxg">antibiotics</a> may be given in certain cases. </p> <h2 id="as-an-adaptation">As an adaptation</h2> <p>An enlarged spleen may be an inherited, adaptive trait selected in populations that need extra oxygen carry capacity such as deep sea divers.<a class="footnote-ref" id="fnref:24" href="#fn:24"><sup>24</sup></a><a class="footnote-ref" id="fnref:25" href="#fn:25"><sup>25</sup></a> The <a href="/facts/Sama-Bajau/eDMPYrEp">Sama-Bajau</a> people, notable for free-diving, have spleens that are 50% larger than those of nearby ethnic groups.<a class="footnote-ref" id="fnref:26" href="#fn:26"><sup>26</sup></a><a class="footnote-ref" id="fnref:27" href="#fn:27"><sup>27</sup></a> </p> <h2 id="see-also">See also</h2> <ul><li><a href="/facts/Asplenia/Roe1jDbz">Asplenia</a></li> <li><a href="/facts/Hepatosplenomegaly/mpVCuqgk">Hepatosplenomegaly</a></li> <li><a href="/facts/Portal_hypertension/REmqnpER">Portal hypertension</a></li> <li><a href="/facts/Sign_(medicine)/BzP6MrTN">Sign (medicine)</a></li> <li><a href="/facts/Splenic_aspiration/2AQRy6Mt">Splenic aspiration</a></li> <li><a href="/facts/Splenic_infarction/3cVIIbWU">Splenic infarction</a></li> <li><a href="/facts/Tropical_splenomegaly_syndrome/TW17KLqa">Tropical splenomegaly syndrome</a></li></ul> <h2 id="external-links">External links</h2> <ul><li><a href="http://www.patient.info/showdoc/40001069/">Splenomegaly and hypersplenism</a> at patient.info</li></ul> <h2 id="references">References</h2> <ol> <li id="fn:1"><p>Chapman, J; Azevedo, AM (2018), "article-29386", Splenomegaly, Treasure Island (FL): StatPearls Publishing, PMID 28613657, retrieved 2019-02-26 <a href="http://www.ncbi.nlm.nih.gov/books/NBK430907/" target="_blank">http://www.ncbi.nlm.nih.gov/books/NBK430907/</a> <a href="#fnref:1" class="footnote-back-ref">↩</a></p></li> <li id="fn:2"><p>Ghazi, Ali (2010). "Hypercalcemia and huge splenomegaly presenting in an elderly patient with B-cell non-Hodgkin's lymphoma: a case report". Journal of Medical Case Reports. 4 (334): 330. doi:10.1186/1752-1947-4-330. PMC 2974746. PMID 20959010. <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2974746" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2974746</a> <a href="#fnref:2" class="footnote-back-ref">↩</a></p></li> <li id="fn:3"><p>Neetu Radhakrishnan. "Splenomegaly". Medscape. Retrieved February 16, 2018. Updated Apr. 2012 (referring the classification system to Poulin et al. <a href="http://emedicine.medscape.com/article/206208-overview" target="_blank">http://emedicine.medscape.com/article/206208-overview</a> <a href="#fnref:3" class="footnote-back-ref">↩</a></p></li> <li id="fn:4"><p>Page 1964 in: Florian Lang (2009). Encyclopedia of Molecular Mechanisms of Disease. Springer Science & Business Media. ISBN 9783540671367. <a href="9783540671367" target="_blank">9783540671367</a> <a href="#fnref:4" class="footnote-back-ref">↩</a></p></li> <li id="fn:5"><p>Saboo, S S; Krajewski, K M; O'Regan, K N; Giardino, A; Brown, J R; Ramaiya, N; Jagannathan, J P (2012). "Spleen in haematological malignancies: spectrum of imaging findings". The British Journal of Radiology. 85 (1009): 81–92. doi:10.1259/bjr/31542964. ISSN 0007-1285. PMC 3473934. PMID 22096219. <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3473934" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3473934</a> <a href="#fnref:5" class="footnote-back-ref">↩</a></p></li> <li id="fn:6"><p>Chow, Kai Uwe; Luxembourg, Beate; Seifried, Erhard; Bonig, Halvard (2016). "Spleen Size Is Significantly Influenced by Body Height and Sex: Establishment of Normal Values for Spleen Size at US with a Cohort of 1200 Healthy Individuals". Radiology. 279 (1): 306–313. doi:10.1148/radiol.2015150887. ISSN 0033-8419. PMID 26509293. <a href="/wiki/Doi_(identifier)" target="_blank">/wiki/Doi_(identifier)</a> <a href="#fnref:6" class="footnote-back-ref">↩</a></p></li> <li id="fn:7"><p>Rosenberg, H K; Markowitz, R I; Kolberg, H; Park, C; Hubbard, A; Bellah, R D (1991). "Normal splenic size in infants and children: sonographic measurements". American Journal of Roentgenology. 157 (1): 119–121. doi:10.2214/ajr.157.1.2048509. ISSN 0361-803X. PMID 2048509. <a href="/wiki/Doi_(identifier)" target="_blank">/wiki/Doi_(identifier)</a> <a href="#fnref:7" class="footnote-back-ref">↩</a></p></li> <li id="fn:8"><p>Rosenberg, H K; Markowitz, R I; Kolberg, H; Park, C; Hubbard, A; Bellah, R D (1991). "Normal splenic size in infants and children: sonographic measurements". American Journal of Roentgenology. 157 (1): 119–121. doi:10.2214/ajr.157.1.2048509. ISSN 0361-803X. 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