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This article needs additional citations for verification. Please help improve this article by adding citations to reliable sources. Unsourced material may be challenged and removed. (February 2010) |
| Splenomegaly |
| Classification and external resources |
CT scan showing splenomegaly in a patient with chronic lymphoid leukemia |
| ICD-10 |
Q89.0, R16.1 |
| ICD-9 |
759.0, 789.2 |
| DiseasesDB |
12375 |
| MedlinePlus |
003276 |
| eMedicine |
ped/2139 med/2156 |
| MeSH |
D013163 |
Splenomegaly is an enlargement of the spleen. 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. Splenomegaly is usually associated with increased workload (such as in hemolytic anemias), which suggests that it is a response to hyperfunction. It is therefore not surprising that splenomegaly is associated with any disease process that involves abnormal red blood cells being destroyed in the spleen. Other common causes include congestion due to portal hypertension and infiltration by leukemias and lymphomas. Thus, the finding of an enlarged spleen; along with caput medusa; is an important sign of portal hypertension.
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Contents
- 1 Definition
- 2 Symptoms and signs
- 3 Causes
- 4 Treatment
- 5 See also
- 6 References
- 7 External links
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Definition
Alotaibi G 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, it means it is enlarged as it has to undergo enlargement by at least two folds to become palpable. However, the tip of the spleen may be palpable in a newborn baby up to 3 months of age.
Symptoms and signs
Symptoms may include abdominal pain, 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 due to accompanying cytopenia.
Signs of splenomegaly may include a palpable left upper quadrant abdominal mass or splenic rub. It can be detected on physical examination by using Castell's sign or Traube's space, but an ultrasound 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.[2]
Causes
The most common causes of splenomegaly in developed countries are infectious mononucleosis, splenic infiltration with cancer cells from a hematological malignancy and portal hypertension (most commonly secondary to liver disease). Splenomegaly may also come from bacterial infections, such as syphilis or an infection of the heart's inner lining (endocarditis).[3]
Splenomegaly grouped on the basis of the pathogenic mechanism
| Increased function |
Abnormal blood flow |
Infiltration |
Removal of defective RBCs
- spherocytosis
- thalassemia
- hemoglobinopathies
- nutritional anemias
- early sickle cell anemia
Immune hyperplasia
Response to infection (viral, bacterial, fungal, parasitic)
- mononucleosis, AIDS, viral hepatitis
- subacute bacterial endocarditis, bacterial septicemia
- splenic abscess, typhoid fever
- brucellosis, leptospirosis, tuberculosis
- histoplasmosis
- malaria, leishmaniasis, trypanosomiasis
- ehrlichiosis
Disordered immunoregulation
- rheumatoid arthritis
- Systemic lupus erythematosus
- serum sickness
- autoimmune hemolytic anemia
- sarcoidosis
- drug reactions
Extramedullary hematopoiesis
- myelofibrosis
- marrow infiltration by tumors, leukemias
- marrow damage by radiation, toxins
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Organ Failure
Vascular
- hepatic vein obstruction
- portal vein obstruction
- Budd–Chiari syndrome
- splenic vein obstruction
Infections
- hepatic schistosomiasis
- hepatic echinococcosis
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Metabolic diseases
- Gauchers disease
- Niemann–Pick disease
- alpha-mannosidosis
- Hurler syndrome and other mucopolysaccharidoses
- amyloidosis
- Tangier disease
Benign and malignant “infiltrations”
- leukemias (acute, chronic, lymphoid, and myeloid)
- lymphomas (Hodgkins and non-Hodgkins)
- myeloproliferative disease
- metastatic tumors (commonly melanoma)
- histiocytosis X
- hemangioma, lymphangioma
- splenic cysts
- hamartomas
- eosinophilic granuloma
- littoral cell angioma
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The causes of massive splenomegaly (>1000 g) are much fewer and include:
- thalassemia
- visceral leishmaniasis (kala-azar)
- schistosomiasis
- chronic myelogenous leukemia
- chronic lymphocytic leukemia
- lymphomas
- hairy cell leukemia
- myelofibrosis
- polycythemia vera
- Gauchers disease
- Niemann–Pick disease
- sarcoidosis
- autoimmune hemolytic anemia
- malaria
Treatment
If the splenomegaly underlies hypersplenism, a splenectomy 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.
Patients undergoing splenectomy should be vaccinated against Haemophilus influenzae, Streptococcus pneumoniae, and Meningococcus. They should also receive annual influenza vaccinations. Long-term prophylactic antibiotics may be given in certain cases.
See also
- Sign (medicine)
- Hepatosplenomegaly
- Splenic infarction
- Asplenia
References
- ^ 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). http://www.jmedicalcasereports.com/content/4/1/330.
- ^ Grover SA, Barkun AN, Sackett DL (1993). "The rational clinical examination. Does this patient have splenomegaly?". JAMA 270 (18): 2218–21. doi:10.1001/jama.270.18.2218. PMID 8411607. Ovid full text
- ^ Kaiser, Larry R.; Pavan Atluri; Giorgos C Karakousis; Paige M Porrett (2006). The surgical review: an integrated basic and clinical science study guide. Hagerstwon, MD: Lippincott Williams & Wilkins. ISBN 0-7817-5641-3.
External links
- PatientPlus Splenomegaly and hypersplenism
- Medical dictionary
- 11-141b. at Merck Manual of Diagnosis and Therapy Professional Edition (Hypersplenism)
- /Splenomegaly and Hypersplenism
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Lymphatic disease: Lymphatic organ disease (D73/E32/I88–I89, 254/289.4–289.5/457)
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| Thymus |
- Abscess of thymus
- Thymus hyperplasia
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| Spleen |
- Acquired asplenia/hyposplenism
- Wandering spleen
- Splenomegaly (Banti's syndrome)
- Splenic infarction
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| Tonsil |
- see Template:Respiratory pathology
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| Lymph node |
- Lymphadenopathy/lymphadenitis
- Generalized lymphadenopathy
- Castleman's disease
- Intranodal palisaded myofibroblastoma
- Kikuchi disease
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anat(h, u, t, a, l)/phys/devp
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Symptoms and signs: digestive system and abdomen (R10–R19, 787,789)
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| GI tract |
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Upper GI tract
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Nausea/Vomiting · Heartburn · Dysphagia (Oropharyngeal, Esophageal)
Halitosis
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Lower GI tract
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Gas: Flatulence · Abdominal distension · Bloating · Belching (Wet burp) · Tympanites
Stool: Fecal incontinence (Encopresis) · Rectal tenesmus · Obstructed defecation
Blood: Fecal occult blood
Diarrhea
Rectal discharge
Football sign
Psoas sign · Obturator sign · Rovsing's sign
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| Accessory |
Hepatosplenomegaly/Hepatomegaly
Jaundice
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| Abdominopelvic |
Ascites
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| Abdominal – general |
Abdominal pain (Acute abdomen, Colic, Baby colic)
Splenomegaly
Abdominal guarding · Abdominal mass · Rebound tenderness
Shifting dullness · Bulging flanks · Puddle sign · Fluid wave test
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anat(t, g, p)/phys/devp/enzy
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noco/cong/tumr, sysi/epon
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proc, drug(A2A/2B/3/4/5/6/7/14/16), blte
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