Splenomegaly
Updates to Article Attributes
Body
was changed:
Splenomegaly refers to the enlargement of the spleen. The upper limit of the normal adult splenic length is traditionally cited at 12 cm, but lengths upwards of 14 cm can be seen in normal, taller males 7.
Terminology
Massive splenomegaly is variably defined, including when the spleen is 5 standard deviations above the mean normal volume (~943 cm3) 4, heavier than 1.0 kg 5 or 1.5 kg 8, >14.5cm.5 cm true craniocaudal measurement on coronal CT 4, >15 cm on clinical exam 8, or at/below the umbilicus, extending into the pelvis and/or across the midline 4,8.
Pathology
Aetiology
The causes of splenomegaly are protean, and can be thought of under several headings 3,8:
- haematological disease
- haemodynamic
- infectious
- storage diseases/metabolic/infiltrative disorders
- neoplastic (non-haematologic)
- traumatic
- connective tissue disorders
Haematological disease
-
anaemias
-
thalassaemia (including beta-thalassemia major
*) - sickle cell disease with splenic sequestration (in young patients before developing autosplenectomy)
- hereditary spherocytosis
- pyruvate kinase deficiency
- thrombotic thrombocytopenic purpura (TTP)
- Plummer-Vinson syndrome
-
thalassaemia (including beta-thalassemia major
- neoplastic/proliferative/redistribution of haematopoiesis
- myeloproliferative neoplasms
* -
acute leukaemia
* -
lymphoma / chronic lymphoid neoplasms
* - extramedullary haematopoiesis
- osteopetrosis
- idiopathic hypereosinophilic syndrome
- myeloproliferative neoplasms
Haemodynamic
- cirrhosis: portal hypertension (common)
- congestive splenomegaly (Banti syndrome)
- splenic vein obstruction
- portal vein obstruction
- right heart failure
- cystic fibrosis 6
Infection
- viral
- bacterial
- fungal
- histoplasmosis (common)
- candidiasis
- parasitic disease
- malaria* (hyperreactive malarial splenomegaly syndrome or tropical splenomegaly syndrome)
- schistosomiasis
- hydatid disease
-
leishmaniasis (kala-azar)
*
- rickettsial
- typhus
Storage diseases/metabolic/infiltrative disorders
-
Gaucher disease
* - glycogen storage disease
- mucopolysaccharidoses
- Niemann-Pick disease
- haemochromatosis
- amyloidosis
- porphyria
- sarcoidosis
Neoplastic (non-haematologic)
Trauma
Connective tissue disorders
Massive splenomegaly
*It is sometimes helpful to consider those aetiologies that may cause massive splenomegalythe spleen to grow especially large as a separate group 3,8:
- thalassaemia (including beta-thalassemia major)
- myeloproliferative neoplasms
- acute leukaemias
- lymphomas/chronic lymphoid neoplasms
- leishmaniasis (kala-azar)
- Gaucher disease
- AIDS with Mycobacterial avium complex infection
- cirrhosis with portal hypertension4
Radiographic features
The shape and orientation of a spleen make accurate linear measurement difficult. Since single length measurements are relatively inaccurate many volume-based approaches have been proposed. True volumetry is the most accurate but is rarely feasible in routine clinical practice.
Ultrasound
On sonographic assessment, a length of 12 cm is generally considered the upper limit of normal 15.
On ultrasound the prolate ellipsoid formula is used most commonly 13:
- spleen volume (cm3) = 0.52 × length × anteroposterior dimension × width (cm)
A modified formula has been proposed to improve accuracy 14:
- spleen volume (cm3) = 0.524 × width × thickness × (maximum length + craniocaudal length) / 2
CT
Single splenic measurements can be used to identify splenomegaly:
- width (largest AP axial measurement) >10.6 cm 15
- craniocaudal length
- >9.5-10.5 cm for splenomegaly 4,15
- >14.5 cm for massive splenomegaly 4
- coronal oblique length >12 cm
The splenic index was first proposed to express splenic volume 10:
- splenic index = length × width × thickness (cm)
The normal range of the index is 120-480 and this is considered the most reliable measurement for splenomegaly 15. Since the index does not have a unit, other formulas have been proposed for estimating splenic volume on cross-sectional imaging 5:
- spleen volume (cm3) = 30 + 0.58 × craniocaudal length × diameter × thickness (cm)
Where:
- length is defined as the craniocaudal distance between the first and last slices in the axial plane where the spleen is depicted
- diameter is the largest measurable long axis distance in the axial plane
- thickness is the largest perpendicular dimension to the diameter in the axial plane
The normal range of the estimated volume in healthy individuals with this formula is 107.2 – 314.5 cm3 (mean: 214.6 cm3) 5.
Practical points
The normal spleen size for any individual is substantially influenced by demographic factors, gendersex, and body habitus. E; e.g. a study using volumetry found a 236.89 ± 77.58 cm3 average normal spleen size, whilst thresholds of 314.47 and 430.84 cm3 were determined for mild and massive splenomegaly respectively 11. In contrast, a study done by Japanese researchers has found much lower values in a different patient population for average spleen volume (112 cm3) and normal range (32-209 cm3) 12. Furthermore, it has been also shown that normal spleen size is influenced by sex and body habitus, with men, and taller or heavier individuals having longer and larger spleens 13.
See also
-<p><strong>Splenomegaly</strong> refers to the enlargement of the <a href="/articles/spleen-1">spleen</a>. The upper limit of the normal adult splenic length is traditionally cited at 12 cm, but lengths upwards of 14 cm can be seen in normal, taller males <sup>7</sup>.</p><h4>Terminology</h4><p><strong>Massive splenomegaly </strong>is variably defined, including when the spleen is 5 standard deviations above the mean normal volume (~943 cm<sup>3</sup>) <sup>4</sup>, heavier than 1.0 kg <sup>5</sup> or 1.5 kg <sup>8</sup>, >14.5cm on coronal CT <sup>4</sup>, >15 cm on clinical exam <sup>8</sup>, or at/below the umbilicus, extending into the pelvis and/or across the midline <sup>4,8</sup>.</p><h4>Pathology</h4><p>The causes of splenomegaly are <a href="/articles/protean">protean</a>, and can be thought of under several headings <sup>3,8</sup>:</p><ul>- +<p><strong>Splenomegaly</strong> refers to enlargement of the <a href="/articles/spleen-1">spleen</a>. The <a title="upper limit of normal" href="/articles/upper-limit-of-normal">upper limit of the normal</a> adult splenic length is traditionally cited at 12 cm, but lengths upwards of 14 cm can be seen in normal, taller males <sup>7</sup>.</p><h4>Terminology</h4><p><strong>Massive splenomegaly </strong>is variably defined, including when the spleen is 5 standard deviations above the mean normal volume (~943 cm<sup>3</sup>) <sup>4</sup>, heavier than 1.0 kg <sup>5</sup> or 1.5 kg <sup>8</sup>, >14.5 cm true craniocaudal measurement on coronal CT <sup>4</sup>, >15 cm on clinical exam <sup>8</sup>, or at/below the umbilicus, extending into the pelvis and/or across the midline <sup>4,8</sup>.</p><h4>Pathology</h4><h5>Aetiology</h5><p>The causes of splenomegaly are <a href="/articles/protean">protean</a>, and can be thought of under several headings <sup>3,8</sup>:</p><ul>
-</ul><h5>Haematological disease</h5><ul>- +</ul><h6>Haematological disease</h6><ul>
-<a href="/articles/thalassaemia">thalassaemia</a> (including beta-thalassemia major*)</li>- +<a href="/articles/thalassaemia">thalassaemia</a> (including beta-thalassemia major)</li>
-<li>myeloproliferative neoplasms*<ul>- +<li>myeloproliferative neoplasms<ul>
-<a href="/articles/acute-leuk">acute leukaemia</a>*<ul>- +<a href="/articles/acute-leuk">acute leukaemia</a><ul>
-<a href="/articles/lymphoma">lymphoma</a> / chronic lymphoid neoplasms*<ul>- +<a href="/articles/lymphoma">lymphoma</a> / chronic lymphoid neoplasms<ul>
-</ul><h5>Haemodynamic</h5><ul>- +</ul><h6>Haemodynamic</h6><ul>
-</ul><h5>Infection</h5><ul>- +</ul><h6>Infection</h6><ul>
-<a href="/articles/hivaids">AIDS</a> with <a href="/articles/pulmonary-non-tuberculous-mycobacterial-infection-3">Mycobacterial avium complex infection</a>*</li>- +<a href="/articles/hivaids">AIDS</a> with <a href="/articles/pulmonary-non-tuberculous-mycobacterial-infection-3">Mycobacterial avium complex infection</a>
- +</li>
-<li>-<a href="/articles/leishmaniasis">leishmaniasis (kala-azar)</a>*</li>- +<li><a href="/articles/leishmaniasis">leishmaniasis (kala-azar)</a></li>
-</ul><h5>Storage diseases/metabolic/infiltrative disorders</h5><ul>-<li>-<a href="/articles/gaucher-disease">Gaucher disease</a>*</li>- +</ul><h6>Storage diseases/metabolic/infiltrative disorders</h6><ul>
- +<li><a href="/articles/gaucher-disease">Gaucher disease</a></li>
-</ul><h5>Neoplastic (non-haematologic)</h5><ul>- +</ul><h6>Neoplastic (non-haematologic)</h6><ul>
-</ul><h5>Trauma</h5><ul>- +</ul><h6>Trauma</h6><ul>
-</ul><h5>Connective tissue disorders</h5><ul>- +</ul><h6>Connective tissue disorders</h6><ul>
-</ul><p>* may cause massive splenomegaly<sup> 3,8</sup></p><h4>Radiographic features</h4><p>The shape and orientation of a spleen make accurate linear measurement difficult. Since single length measurements are relatively inaccurate many volume-based approaches have been proposed. True volumetry is the most accurate but is rarely feasible in routine clinical practice. </p><h5>Ultrasound</h5><p>On sonographic assessment, a length of 12 cm is generally considered the upper limit of normal <sup>15</sup>. </p><p>On ultrasound the prolate ellipsoid formula is used most commonly <sup>13</sup>: </p><ul><li>spleen volume (cm<sup>3</sup>) = 0.52 × length × anteroposterior dimension × width (cm)</li></ul><p>A modified formula has been proposed to improve accuracy <sup>14</sup>: </p><ul><li>spleen volume (cm<sup>3</sup>) = 0.524 × width × thickness × (maximum length + craniocaudal length) / 2</li></ul><h5>CT</h5><p>Single splenic measurements can be used to identify splenomegaly:</p><ul>- +</ul><h6>Massive splenomegaly</h6><p>It is sometimes helpful to consider those aetiologies that may cause the spleen to grow especially large as a separate group <sup>3,8</sup>:</p><ul>
- +<li>
- +<a href="/articles/thalassaemia">thalassaemia</a> (including beta-thalassemia major)</li>
- +<li>myeloproliferative neoplasms</li>
- +<li><a href="/articles/acute-leuk">acute leukaemias</a></li>
- +<li>
- +<a href="/articles/lymphoma">lymphomas</a>/chronic lymphoid neoplasms</li>
- +<li><a href="/articles/leishmaniasis">leishmaniasis (kala-azar)</a></li>
- +<li><a title="Gaucher disease" href="/articles/gaucher-disease">Gaucher disease</a></li>
- +<li>
- +<a href="/articles/hivaids">AIDS</a> with <a href="/articles/pulmonary-non-tuberculous-mycobacterial-infection-3">Mycobacterial avium complex infection</a>
- +</li>
- +<li>
- +<a title="Cirrhosis" href="/articles/cirrhosis">cirrhosis</a> with <a title="Portal hypertension" href="/articles/portal-hypertension">portal hypertension</a> <sup>4</sup>
- +</li>
- +</ul><h4>Radiographic features</h4><p>The shape and orientation of a spleen make accurate linear measurement difficult. Since single length measurements are relatively inaccurate many volume-based approaches have been proposed. True volumetry is the most accurate but is rarely feasible in routine clinical practice. </p><h5>Ultrasound</h5><p>On sonographic assessment, a length of 12 cm is generally considered the upper limit of normal <sup>15</sup>. </p><p>On ultrasound the prolate ellipsoid formula is used most commonly <sup>13</sup>: </p><ul><li>spleen volume (cm<sup>3</sup>) = 0.52 × length × anteroposterior dimension × width (cm)</li></ul><p>A modified formula has been proposed to improve accuracy <sup>14</sup>: </p><ul><li>spleen volume (cm<sup>3</sup>) = 0.524 × width × thickness × (maximum length + craniocaudal length) / 2</li></ul><h5>CT</h5><p>Single splenic measurements can be used to identify splenomegaly:</p><ul>
-</ul><p>The normal range of the estimated volume in healthy individuals with this formula is 107.2 – 314.5 cm<sup>3</sup> (mean: 214.6 cm<sup>3</sup>) <sup>5</sup>.</p><h4>Practical points</h4><p>The normal spleen size for any individual is substantially influenced by demographic factors, gender, and body habitus. E.g. a study using volumetry found a 236.89 ± 77.58 cm<sup>3</sup> average normal spleen size, whilst thresholds of 314.47 and 430.84 cm<sup>3</sup> were determined for mild and massive splenomegaly respectively <sup>11</sup>. In contrast, a study done by Japanese researchers has found much lower values in a different patient population for average spleen volume (112 cm<sup>3</sup>) and normal range (32-209 cm<sup>3</sup>) <sup>12</sup>. Furthermore, it has been also shown that normal spleen size is influenced by sex and body habitus, with men, and taller or heavier individuals having longer and larger spleens <sup>13</sup>. </p><h4>See also</h4><ul>- +</ul><p>The normal range of the estimated volume in healthy individuals with this formula is 107.2 – 314.5 cm<sup>3</sup> (mean: 214.6 cm<sup>3</sup>) <sup>5</sup>.</p><h4>Practical points</h4><p>The normal spleen size for any individual is substantially influenced by demographic factors, sex, and <a title="Body habitus" href="/articles/body-habitus">body habitus</a>; e.g. a study using <a title="volumetry (general)" href="/articles/volumetry-general">volumetry</a> found a 236.89 ± 77.58 cm<sup>3</sup> average normal spleen size, whilst thresholds of 314.47 and 430.84 cm<sup>3</sup> were determined for mild and massive splenomegaly respectively <sup>11</sup>. In contrast, a study done by Japanese researchers has found much lower values in a different patient population for average spleen volume (112 cm<sup>3</sup>) and normal range (32-209 cm<sup>3</sup>) <sup>12</sup>. Furthermore, it has been also shown that normal spleen size is influenced by sex and body habitus, with men, and taller or heavier individuals having longer and larger spleens <sup>13</sup>. </p><h4>See also</h4><ul>
Unable to process the form. Check for errors and try again.