Splenomegaly

Splenomegaly is an enlargement of the spleen

Spleen

The spleen is an organ found in virtually all vertebrate animals with important roles in regard to red blood cells and the immune system. In humans, it is located in the left upper quadrant of the abdomen. It removes old red blood cells and holds a reserve of blood in case of hemorrhagic shock...

. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. It is one of the four cardinal signs of hypersplenism, some reduction in the number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any combination; a compensatory proliferative response in the bone marrow; and the potential for correction of these abnormalities by splenectomy

Splenectomy

A splenectomy is a surgical procedure that partially or completely removes the spleen.-Indications:The spleen, similar in structure to a large lymph node, acts as a blood filter. Current knowledge of its purpose includes the removal of old red blood cells and platelets, and the detection and fight...

. Splenomegaly is usually associated with increased workload (such as in hemolytic anemia

Hemolytic anemia

Hemolytic anemia is a form of anemia due to hemolysis, the abnormal breakdown of red blood cells , either in the blood vessels or elsewhere in the human body . It has numerous possible causes, ranging from relatively harmless to life-threatening...

s), which suggests that it is a response to hyperfunction. It is therefore not surprising that splenomegaly is associated with any disease

Disease

A disease is an abnormal condition affecting the body of an organism. It is often construed to be a medical condition associated with specific symptoms and signs. It may be caused by external factors, such as infectious disease, or it may be caused by internal dysfunctions, such as autoimmune...

 process that involves abnormal red blood cell

Red blood cell

Red blood cells are the most common type of blood cell and the vertebrate organism's principal means of delivering oxygen to the body tissues via the blood flow through the circulatory system...

s being destroyed in the spleen. Other common causes include congestion due to portal hypertension

Portal hypertension

In medicine, portal hypertension is hypertension in the portal vein and its tributaries.It is often defined as a portal pressure gradient of 10 mmHg or greater.-Causes:Causes can be divided into prehepatic, intrahepatic, and posthepatic...

 and infiltration by leukemias and lymphomas. Thus, the finding of an enlarged spleen; along with caput medusa; is an important sign of portal hypertension.

Definition

Splenomegaly is defined by Dr Abdul Ghaffar as spleen size >12 cm, as measured by ultrasound along its longer dimension.

Poulain et al. classify splenomegaly as:

• Moderate splenomegaly, if the largest dimension is between 11–20 cm
• Severe splenomegaly, if the largest dimension is greater than 20 cm

Splenomegaly should not be confused with hypersplenism. The former is a statement about the size of the spleen, and the latter about the spleen's function: these may coexist, or they may not.
clinically if a spleen is palpable means it is enlarged as it has to undergo enlargement by at least two folds for being palpable. However tip of the spleen may be palpable in a newborn baby upto 3 months of age.

Symptoms and signs

Symptoms may include abdominal pain

Abdominal pain

Abdominal pain can be one of the symptoms associated with transient disorders or serious disease. Making a definitive diagnosis of the cause of abdominal pain can be difficult, because many diseases can result in this symptom. Abdominal pain is a common problem...

, chest pain, chest pain similar to pleuritic pain when stomach, bladder or bowels are full, back pain, early satiety due to splenic encroachment, or the symptoms of anemia

Anemia

Anemia is a decrease in number of red blood cells or less than the normal quantity of hemoglobin in the blood. However, it can include decreased oxygen-binding ability of each hemoglobin molecule due to deformity or lack in numerical development as in some other types of hemoglobin...

 due to accompanying cytopenia.

Sign

Medical sign

A medical sign is an objective indication of some medical fact or characteristic that may be detected by a physician during a physical examination of a patient....

s of splenomegaly may include a palpable left upper quadrant abdominal mass

Abdominal mass

An abdominal mass is any localized enlargement or swelling in the human abdomen. Depending on its location, the abdominal mass may be caused by an enlarged liver , enlarged spleen , protruding kidney, a pancreatic mass, a retroperitoneal mass , an abdominal aortic aneurysm, or various tumours, such...

 or splenic rub. It can be detected on physical examination

Physical examination

Physical examination or clinical examination is the process by which a doctor investigates the body of a patient for signs of disease. It generally follows the taking of the medical history — an account of the symptoms as experienced by the patient...

 by using Castell's sign

Castell's sign

Castell's sign is a medical sign assessed to evaluate splenomegaly and typically part of an abdominal examination. It is an alternative physical examination maneuver to percussion over Traube's space....

 or Traube's space

Traube's space

Traube's space is an anatomic region of some clinical importance. It's a crescent-shaped space, encompassed by the lower edge of the left lung, the anterior border of the spleen, the left costal margin and the inferior margin of the left lobe of the liver...

, but an ultrasound

Ultrasound

Ultrasound is cyclic sound pressure with a frequency greater than the upper limit of human hearing. Ultrasound is thus not separated from "normal" sound based on differences in physical properties, only the fact that humans cannot hear it. Although this limit varies from person to person, it is...

 can be used to confirm diagnosis.

Causes

The most common causes of splenomegaly in developed countries are infectious mononucleosis

Infectious mononucleosis

Infectious mononucleosis is an infectious, widespread viral...

, splenic infiltration with cancer

Cancer

Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...

 cells from a hematological malignancy

Hematological malignancy

Hematological malignancies are the types of cancer that affect blood, bone marrow, and lymph nodes. As the three are intimately connected through the immune system, a disease affecting one of the three will often affect the others as well: although lymphoma is technically a disease of the lymph...

 and portal hypertension

Portal hypertension

In medicine, portal hypertension is hypertension in the portal vein and its tributaries.It is often defined as a portal pressure gradient of 10 mmHg or greater.-Causes:Causes can be divided into prehepatic, intrahepatic, and posthepatic...

 (most commonly secondary to liver disease

Liver disease

Liver disease is a broad term describing any single number of diseases affecting the liver.-Diseases:* Hepatitis, inflammation of the liver, caused mainly by various viruses but also by some poisons , autoimmunity or hereditary conditions...

) Also by Bacterial infections, such as syphilis or an infection of the heart's inner lining (endocarditis).

Splenomegaly grouped on the basis of the pathogenic mechanism

Increased function	Abnormal blood flow	Infiltration
Removal of defective RBCs spherocytosis Spherocytosis Spherocytosis is an auto-hemolytic anemia characterized by the production of red blood cells , or erythrocytes, that are sphere-shaped, rather than bi-concave disk shaped. Spherocytes are found in hereditary spherocytosis and autoimmune hemolytic anemia.It almost always refers to hereditary... thalassemia Thalassemia Thalassemia is an inherited autosomal recessive blood disease that originated in the Mediterranean region. In thalassemia the genetic defect, which could be either mutation or deletion, results in reduced rate of synthesis or no synthesis of one of the globin chains that make up hemoglobin... hemoglobinopathies nutritional anemias early sickle cell anemia Immune hyperplasia Response to infection (viral, bacterial, fungal, parasitic) mononucleosis Mononucleosis Mononucleosis , is a disease most commonly caused by the Epstein-Barr virus . The EBV virus affects the lymphocytes- white blood cells that battle infections by attacking antibodies. Mononucleosis can also be caused by Cytomegalovirus , a herpes virus most commonly found in body fluids... , AIDS AIDS Acquired immune deficiency syndrome or acquired immunodeficiency syndrome is a disease of the human immune system caused by the human immunodeficiency virus... , viral hepatitis Viral hepatitis Viral hepatitis is liver inflammation due to a viral infection. It may present in acute or chronic forms. The most common causes of viral hepatitis are the five unrelated hepatotropic viruses Hepatitis A, Hepatitis B, Hepatitis C, Hepatitis D, and Hepatitis E... subacute bacterial endocarditis, bacterial septicemia splenic abscess Abscess An abscess is a collection of pus that has accumulated in a cavity formed by the tissue in which the pus resides due to an infectious process or other foreign materials... , typhoid fever Typhoid fever Typhoid fever, also known as Typhoid, is a common worldwide bacterial disease, transmitted by the ingestion of food or water contaminated with the feces of an infected person, which contain the bacterium Salmonella enterica, serovar Typhi... brucellosis Brucellosis Brucellosis, also called Bang's disease, Crimean fever, Gibraltar fever, Malta fever, Maltese fever, Mediterranean fever, rock fever, or undulant fever, is a highly contagious zoonosis caused by ingestion of unsterilized milk or meat from infected animals or close contact with their secretions... , leptospirosis Leptospirosis Leptospirosis is caused by infection with bacteria of the genus Leptospira, and affects humans as well as other mammals, birds, amphibians, and reptiles.The... , tuberculosis Tuberculosis Tuberculosis, MTB, or TB is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. Tuberculosis usually attacks the lungs but can also affect other parts of the body... histoplasmosis Histoplasmosis Histoplasmosis is a disease caused by the fungus Histoplasma capsulatum. Symptoms of this infection vary greatly, but the disease primarily affects the lungs... malaria Malaria Malaria is a mosquito-borne infectious disease of humans and other animals caused by eukaryotic protists of the genus Plasmodium. The disease results from the multiplication of Plasmodium parasites within red blood cells, causing symptoms that typically include fever and headache, in severe cases... , leishmaniasis Leishmaniasis Leishmaniasis is a disease caused by protozoan parasites that belong to the genus Leishmania and is transmitted by the bite of certain species of sand fly... , trypanosomiasis Trypanosomiasis Trypanosomiasis or trypanosomosis is the name of several diseases in vertebrates caused by parasitic protozoan trypanosomes of the genus Trypanosoma. Approximately 500,000 men, women and children in 36 countries of sub-Saharan Africa suffer from human African trypanosomiasis which is caused by... ehrlichiosis Ehrlichiosis Ehrlichiosis is a tick-borne disease of dogs usually caused by the organism Ehrlichia canis. Ehrlichia canis is the pathogen of animals. Humans can become infected by E. canis and other species after tick exposure. German Shepherd dogs are thought to be particularly affected by the disease, other... Disordered immunoregulation rheumatoid arthritis Rheumatoid arthritis Rheumatoid arthritis is a chronic, systemic inflammatory disorder that may affect many tissues and organs, but principally attacks synovial joints. The process produces an inflammatory response of the synovium secondary to hyperplasia of synovial cells, excess synovial fluid, and the development... Systemic lupus erythematosus Systemic lupus erythematosus Systemic lupus erythematosus , often abbreviated to SLE or lupus, is a systemic autoimmune disease that can affect any part of the body. As occurs in other autoimmune diseases, the immune system attacks the body's cells and tissue, resulting in inflammation and tissue damage... serum sickness Serum sickness Serum sickness in humans is a reaction to proteins in antiserum derived from an non-human animal source. It is a type of hypersensitivity, specifically immune complex hypersensitivity . The term serum sickness–like reaction is occasionally used to refer to similar illnesses that arise from the... autoimmune hemolytic anemia Autoimmune hemolytic anemia Autoimmune hemolytic anemia occurs when antibodies directed against the person's own red blood cells cause the RBCs to burst , leading to insufficient plasma concentration. The lifetime of the RBCs is reduced from the normal 100–120 days to just a few days in serious cases... sarcoidosis Sarcoidosis Sarcoidosis , also called sarcoid, Besnier-Boeck disease or Besnier-Boeck-Schaumann disease, is a disease in which abnormal collections of chronic inflammatory cells form as nodules in multiple organs. The cause of sarcoidosis is unknown... drug reactions Extramedullary hematopoiesis myelofibrosis Myelofibrosis Myelofibrosis, also known as myeloid metaplasia, chronic idiopathic myelofibrosis, osteomyelofibrosis and primary myelofibrosis is a disorder of the bone marrow... marrow infiltration by tumors, leukemias marrow damage by radiation Radiation In physics, radiation is a process in which energetic particles or energetic waves travel through a medium or space. There are two distinct types of radiation; ionizing and non-ionizing... , toxins	Organ Failure cirrhosis Cirrhosis Cirrhosis is a consequence of chronic liver disease characterized by replacement of liver tissue by fibrosis, scar tissue and regenerative nodules , leading to loss of liver function... Vascular hepatic vein obstruction portal vein obstruction Portal vein thrombosis Portal vein thrombosis is a form of venous thrombosis affecting the hepatic portal vein, which can lead to portal hypertension and reduction in the blood supply to the liver.-Causes:... Budd–Chiari syndrome splenic vein obstruction Infections hepatic schistosomiasis Schistosomiasis Schistosomiasis is a parasitic disease caused by several species of trematodes , a parasitic worm of the genus Schistosoma. Snails often act as an intermediary agent for the infectious diseases until a new human host is found... hepatic echinococcosis Echinococcosis Echinococcosis, which is often referred to as hydatid disease or echinococcal disease, is a parasitic disease that affects both humans and other mammals, such as sheep, dogs, rodents and horses. There are three different forms of echinococcosis found in humans, each of which is caused by the larval...	Metabolic diseases Gauchers disease Niemann–Pick disease alpha-mannosidosis Alpha-mannosidosis Alpha-mannosidosis is a lysosomal storage disorder caused by deficient activity of the enzyme alpha-D-mannosidase . In humans it is known to be caused by an autosomal recessive genetic mutation... Hurler syndrome Hurler syndrome Hurler syndrome, also known as mucopolysaccharidosis type I , Hurler's disease, also gargoylism, is a genetic disorder that results in the buildup of glycosaminoglycans due to a deficiency of alpha-L iduronidase, an enzyme responsible for the degradation of mucopolysaccharides in lysosomes...  and other mucopolysaccharidoses amyloidosis Amyloidosis In medicine, amyloidosis refers to a variety of conditions whereby the body produces "bad proteins", denoted as amyloid proteins, which are abnormally deposited in organs and/or tissues and cause harm. A protein is described as being amyloid if, due to an alteration in its secondary structure, it... Tangier disease Tangier disease Tangier disease is a rare inherited disorder characterized by a severe reduction in the amount of high density lipoprotein , often referred to as "good cholesterol," in the bloodstream.-Diagnosis:... Benign and malignant "infiltrations" leukemias (acute, chronic, lymphoid, and myeloid) lymphomas (Hodgkins Hodgkins Hodgkins may refer to:*Hodgkin's lymphoma, a type of cancer*Frances Hodgkins, an abstract painter*W. M. Hodgkins, New Zealand painter, father of Frances Hodgkins*Hodgkins, Illinois, a village in the United States...  and non-Hodgkins Non-Hodgkin lymphoma The non-Hodgkin lymphomas are a diverse group of blood cancers that include any kind of lymphoma except Hodgkin's lymphomas. Types of NHL vary significantly in their severity, from indolent to very aggressive.... ) myeloproliferative disorders metastatic tumors (commonly melanoma Melanoma Melanoma is a malignant tumor of melanocytes. Melanocytes are cells that produce the dark pigment, melanin, which is responsible for the color of skin. They predominantly occur in skin, but are also found in other parts of the body, including the bowel and the eye... ) histiocytosis X hemangioma Hemangioma A hemangioma of infancy is a benign self-involuting tumor of endothelial cells, the cells that line blood vessels. It usually appears during the first weeks of life and sometimes resolves by age 10. In more severe case hemangioma may have permanency, if not treated by a physician... , lymphangioma Lymphangioma Lymphangiomas are malformations of the lymphatic system, which is the network of vessels responsible for returning to the venous system excess fluid from tissues. These malformations can occur at any age and may involve any part of the body, but 90% occur in children less than 2 years of age and... splenic cysts hamartomas eosinophilic granuloma littoral cell angioma Littoral cell angioma Littoral cell angioma, abbreviated LCA, and formally known as littoral cell angioma of the spleen, is a benign tumour of the spleen that arises the from the cells that line the red pulp.-Diagnosis:...

The causes of massive splenomegaly (>1000 g) are much fewer and include:

• thalassemia
  Thalassemia
  Thalassemia is an inherited autosomal recessive blood disease that originated in the Mediterranean region. In thalassemia the genetic defect, which could be either mutation or deletion, results in reduced rate of synthesis or no synthesis of one of the globin chains that make up hemoglobin...
  
  
• visceral leishmaniasis
  Visceral leishmaniasis
  Visceral leishmaniasis , also known as kala-azar, black fever, and Dumdum fever, is the most severe form of leishmaniasis. Leishmaniasis is a disease caused by protozoan parasites of the Leishmania genus. This disease is the second-largest parasitic killer in the world , responsible for an...
  
   (kala-azar)
• schistosomiasis
  Schistosomiasis
  Schistosomiasis is a parasitic disease caused by several species of trematodes , a parasitic worm of the genus Schistosoma. Snails often act as an intermediary agent for the infectious diseases until a new human host is found...
  
  
• chronic myelogenous leukemia
  Chronic myelogenous leukemia
  Chronic myelogenous leukemia , also known as chronic granulocytic leukemia , is a cancer of the white blood cells. It is a form of leukemia characterized by the increased and unregulated growth of predominantly myeloid cells in the bone marrow and the accumulation of these cells in the blood...
  
  
• chronic lymphocytic leukemia
  Chronic lymphocytic leukemia
  B-cell chronic lymphocytic leukemia , also known as chronic lymphoid leukemia , is the most common type of leukemia. Leukemias are cancers of the white blood cells . CLL affects B cell lymphocytes. B cells originate in the bone marrow, develop in the lymph nodes, and normally fight infection by...
  
  
• lymphomas
• hairy cell leukemia
  Hairy cell leukemia
  Hairy cell leukemia is an uncommon hematological malignancy characterized by an accumulation of abnormal B lymphocytes. It is usually classified as a sub-type of chronic lymphoid leukemia...
  
  
• myelofibrosis
  Myelofibrosis
  Myelofibrosis, also known as myeloid metaplasia, chronic idiopathic myelofibrosis, osteomyelofibrosis and primary myelofibrosis is a disorder of the bone marrow...
  
  
• polycythemia vera
  Polycythemia vera
  Polycythemia vera is a blood disorder in which the bone marrow makes too many red blood cells. It may also result in the overproduction of white blood cells and platelets. Most of the health concerns associated with polycythemia vera are caused by the blood being thicker as a result of the...
  
  
• Gauchers disease
• Niemann–Pick disease
• sarcoidosis
  Sarcoidosis
  Sarcoidosis , also called sarcoid, Besnier-Boeck disease or Besnier-Boeck-Schaumann disease, is a disease in which abnormal collections of chronic inflammatory cells form as nodules in multiple organs. The cause of sarcoidosis is unknown...
  
  
• autoimmune hemolytic anemia
  Autoimmune hemolytic anemia
  Autoimmune hemolytic anemia occurs when antibodies directed against the person's own red blood cells cause the RBCs to burst , leading to insufficient plasma concentration. The lifetime of the RBCs is reduced from the normal 100–120 days to just a few days in serious cases...
  
  
• malaria
  Malaria
  Malaria is a mosquito-borne infectious disease of humans and other animals caused by eukaryotic protists of the genus Plasmodium. The disease results from the multiplication of Plasmodium parasites within red blood cells, causing symptoms that typically include fever and headache, in severe cases...
  
  

Treatment

If the splenomegaly underlies hypersplenism, a splenectomy

Splenectomy

A splenectomy is a surgical procedure that partially or completely removes the spleen.-Indications:The spleen, similar in structure to a large lymph node, acts as a blood filter. Current knowledge of its purpose includes the removal of old red blood cells and platelets, and the detection and fight...

 is indicated and will correct the hypersplenism. However, the underlying cause of the hypersplenism will most likely remain, so 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.

Patients undergoing splenectomy should be vaccinated

Vaccine

A vaccine is a biological preparation that improves immunity to a particular disease. A vaccine typically contains an agent that resembles a disease-causing microorganism, and is often made from weakened or killed forms of the microbe or its toxins...

 against Haemophilus influenzae

Haemophilus influenzae

Haemophilus influenzae, formerly called Pfeiffer's bacillus or Bacillus influenzae, Gram-negative, rod-shaped bacterium first described in 1892 by Richard Pfeiffer during an influenza pandemic. A member of the Pasteurellaceae family, it is generally aerobic, but can grow as a facultative anaerobe. H...

, Streptococcus pneumoniae

Streptococcus pneumoniae

Streptococcus pneumoniae, or pneumococcus, is Gram-positive, alpha-hemolytic, aerotolerant anaerobic member of the genus Streptococcus. A significant human pathogenic bacterium, S...

, and Meningococcus. They should also receive annual influenza vaccinations. Long-term prophylactic antibiotic

Antibiotic

An antibacterial is a compound or substance that kills or slows down the growth of bacteria.The term is often used synonymously with the term antibiotic; today, however, with increased knowledge of the causative agents of various infectious diseases, antibiotic has come to denote a broader range of...

s may be given in certain cases.

External links

• PatientPlus Splenomegaly and hypersplenism
• Medical dictionary (Hypersplenism)