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词条 Splenomegaly
释义

  1. Definition

  2. Signs and symptoms

  3. Causes

  4. Pathophysiology

  5. Diagnosis

  6. Treatment

  7. As an adaptation

  8. See also

  9. References

  10. External links

{{more citations needed|date=February 2010}}{{Infobox medical condition (new)
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| caption = CT scan in a patient with chronic lymphocytic leukemia, showing splenomegaly. Yellow arrows point at the spleen.
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}}Splenomegaly is an enlargement of the spleen[1]. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of hypersplenism which include: some reduction in 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 medusae, is an important sign of portal hypertension.[2]

Definition

The standard system for classifying splenomegaly on radiography is:[3][4]

  • Normal (not splenomegaly): the largest dimension is less than 11 cm
  • Moderate splenomegaly: the largest dimension is between 11–20 cm
  • Severe splenomegaly: the largest dimension is greater than 20 cm

Also, a cutoff of a craniocaudal height of 13 cm is also used to define splenomegaly.[5]

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 external examination), 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.

Age Cutoff[6]
3 months 6.0 cm
6 months 6.5 cm
12 months 7.0 cm
2 years 8.0 cm
4 years 9.0
6 years 9.5 cm
8 years 10.0 cm
10 years 11.0 cm
12 years 11.5 cm
15 years
  • 12.0 cm
    for girls
  • 13.0 cm
    for boys

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 coronal plane through its hilum while breathing quietly.[6]

Signs and symptoms

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, Traube's space percussion or Nixon's sign, 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.[7]

In cases of infectious mononucleosis splenomegaly is a common symptom and health care providers may consider using abdominal ultrasonography to get insight into a person's condition.[8] 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.[8]

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, and sarcoidosis). Splenomegaly may also come from bacterial infections, such as syphilis or an infection of the heart's inner lining (endocarditis).[9]

The possible causes of moderate splenomegaly (spleen <1000 g) are many, and include:

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 hyperplasiaResponse to infection (viral, bacterial, fungal, parasitic)
  • mononucleosis, AIDS,[10] viral hepatitis
  • subacute bacterial endocarditis, bacterial sepsis
  • splenic abscess, typhoid fever
  • brucellosis, leptospirosis, tuberculosis
  • histoplasmosis
  • malaria, leishmaniasis, trypanosomiasis
  • ehrlichiosis[11]
Disordered immunoregulation
  • rheumatoid arthritis, including cases of Felty's syndrome
  • systemic lupus erythematosus
  • serum sickness
  • familial hemophagocytic lymphohistiocytosis
  • autoimmune hemolytic anemia
  • autoimmune lymphoproliferative syndrome, an autosomal dominant disorder
  • sarcoidosis
  • drug reactions
Extramedullary hematopoiesis
  • myelofibrosis
  • marrow infiltration by tumors, leukemias
  • marrow damage by radiation, toxins
Organ Failure
  • cirrhosis
Vascular
  • hepatic vein obstruction
  • portal vein obstruction
  • Budd–Chiari syndrome
  • splenic vein obstruction
Infections
  • hepatic schistosomiasis
  • hepatic echinococcosis
Metabolic diseases
  • Gaucher disease
  • Niemann–Pick disease
  • alpha-mannosidosis
  • Hurler syndrome and other mucopolysaccharidoses[12]
  • 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[13][14][15]

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

  • visceral leishmaniasis (kala-azar)
  • chronic myelogenous leukemia
  • myelofibrosis
  • malaria
  • splenic marginal zone lymphoma

Pathophysiology

Splenomegaly can be classified based on its pathophysiologic mechanism:

  • Congestive, by pooled blood (e.g., portal hypertension)
  • Infiltrative, by invasion by cells foreign to the splenic environment (e.g., metastases, myeloid neoplasms, lipid storage diseases)
  • Immune, by an increase in immunologic activity and subsequent hyperplasia (e.g., endocarditis, sarcoidosis, rheumatoid arthritis)
  • Neoplastic, when resident immune cells originate a neoplasm (e.g., lymphoma).
[1]

Diagnosis

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.

As an adaptation

An enlarged spleen may be an inherited, adaptive trait selected in populations that need extra oxygen carry capacity such as deep sea divers.[16]

See also

{{col div|colwidth=35em}}
  • Asplenia
  • Hepatosplenomegaly
  • Portal hypertension
  • Sign (medicine)
  • Splenic infarction
  • Tropical splenomegaly syndrome
{{colend}}

References

1. ^1 {{cite | last=Chapman | first=J | last2=Azevedo | first2=AM | title=Splenomegaly | chapter=article-29386 | publisher=StatPearls Publishing | publication-place=Treasure Island (FL) | year=2018 | pmid=28613657 | url=http://www.ncbi.nlm.nih.gov/books/NBK430907/ | access-date=2019-02-26}}
2. ^{{cite journal|last=Ghazi|first=Ali|title=Hypercalcemia and huge splenomegaly presenting in an elderly patient with B-cell non-Hodgkin's lymphoma: a case report|journal=Journal of Medical Case Reports|year=2010|volume=4|issue=334|url=http://www.jmedicalcasereports.com/content/4/1/330 | doi = 10.1186/1752-1947-4-330}}
3. ^{{cite web|title=Splenomegaly|url=http://emedicine.medscape.com/article/206208-overview|author=Neetu Radhakrishnan|website=Medscape|access-date=February 16, 2018}} Updated Apr. 2012 (referring the classification system to Poulin et al.
4. ^[https://books.google.com/books?id=DdBJ6jCf8KgC&pg=PA1964 Page 1964] in: {{cite book|title=Encyclopedia of Molecular Mechanisms of Disease|author=Florian Lang|publisher=Springer Science & Business Media|year=2009|isbn=9783540671367}}
5. ^{{cite journal|last1=Saboo|first1=S S|last2=Krajewski|first2=K M|last3=O'Regan|first3=K N|last4=Giardino|first4=A|last5=Brown|first5=J R|last6=Ramaiya|first6=N|last7=Jagannathan|first7=J P|title=Spleen in haematological malignancies: spectrum of imaging findings|journal=The British Journal of Radiology|volume=85|issue=1009|year=2012|pages=81–92|issn=0007-1285|doi=10.1259/bjr/31542964}}
6. ^1 {{cite journal|last1=Rosenberg|first1=H K|last2=Markowitz|first2=R I|last3=Kolberg|first3=H|last4=Park|first4=C|last5=Hubbard|first5=A|last6=Bellah|first6=R D|title=Normal splenic size in infants and children: sonographic measurements|journal=American Journal of Roentgenology|volume=157|issue=1|year=1991|pages=119–121|issn=0361-803X|doi=10.2214/ajr.157.1.2048509}}
7. ^{{cite journal |vauthors=Grover SA, Barkun AN, Sackett DL |title=The rational clinical examination. Does this patient have splenomegaly? |journal=JAMA |volume=270 |issue=18 |pages=2218–21 |year=1993 |pmid=8411607 |doi=10.1001/jama.270.18.2218}} Ovid full text
8. ^1 {{Citation |author1 = American Medical Society for Sports Medicine |author1-link = American Medical Society for Sports Medicine |date = 24 April 2014 |title = Five Things Physicians and Patients Should Question |publisher = American Medical Society for Sports Medicine |work = Choosing Wisely: an initiative of the ABIM Foundation |page = |url = http://www.choosingwisely.org/doctor-patient-lists/american-medical-society-for-sports-medicine/ |accessdate = 29 July 2014}}, which cites*{{cite journal|last1=Putukian|first1=M|last2=O'Connor|first2=FG|last3=Stricker|first3=P|last4=McGrew|first4=C|last5=Hosey|first5=RG|last6=Gordon|first6=SM|last7=Kinderknecht|first7=J|last8=Kriss|first8=V|last9=Landry|first9=G|title=Mononucleosis and athletic participation: an evidence-based subject review|journal=Clinical Journal of Sport Medicine|date=Jul 2008|volume=18|issue=4|pages=309–15|pmid=18614881|doi=10.1097/jsm.0b013e31817e34f8}}*{{cite journal|last1=Spielmann|first1=AL|last2=DeLong|first2=DM|last3=Kliewer|first3=MA|title=Sonographic evaluation of spleen size in tall healthy athletes.|journal=AJR. American Journal of Roentgenology|date=Jan 2005|volume=184|issue=1|pages=45–9|pmid=15615949|doi=10.2214/ajr.184.1.01840045}}
9. ^{{cite book |author1=Kaiser, Larry R. |author2=Pavan Atluri |author3=Giorgos C Karakousis |author4=Paige M Porrett |title=The surgical review: an integrated basic and clinical science study guide |publisher=Lippincott Williams & Wilkins |location=Hagerstwon, MD |year=2006 |pages= |isbn=0-7817-5641-3 |oclc= |doi= |accessdate=}}
10. ^{{Cite journal | last1 = Sproat | first1 = LO. | last2 = Pantanowitz | first2 = L. | last3 = Lu | first3 = CM. | last4 = Dezube | first4 = BJ. | title = Human immunodeficiency virus-associated hemophagocytosis with iron-deficiency anemia and massive splenomegaly | journal = Clin Infect Dis | volume = 37 | issue = 11 | pages = e170–3 |date=Dec 2003 | doi = 10.1086/379613 | pmid = 14614691 }}
11. ^{{Cite journal | last1 = Friedman | first1 = AD. | last2 = Daniel | first2 = GK. | last3 = Qureshi | first3 = WA. | title = Systemic ehrlichiosis presenting as progressive hepatosplenomegaly | journal = South Med J | volume = 90 | issue = 6 | pages = 656–60 |date=Jun 1997 | doi = 10.1097/00007611-199706000-00017| pmid = 9191748 }}
12. ^{{cite book | chapter=The mucopolysaccharidoses |vauthors=Neufeld EF, Muenzer J | title=The metabolic and molecular bases of inherited disease.7th ed. Vol. 2. |veditors=Scriver CR, Beaudet AL, Sly WS, Valle D | year=1995 | publisher=McGraw-Hill, New York | isbn= | pages=2465–94 }}
13. ^{{Cite journal | last1 = Suvajdzić | first1 = N. | last2 = Cemerikić-Martinović | first2 = V. | last3 = Saranović | first3 = D. | last4 = Petrović | first4 = M. | last5 = Popović | first5 = M. | last6 = Artiko | first6 = V. | last7 = Cupić | first7 = M. | last8 = Elezović | first8 = I. | title = Littoral-cell angioma as a rare cause of splenomegaly | journal = Clin Lab Haematol | volume = 28 | issue = 5 | pages = 317–20 |date=Oct 2006 | doi = 10.1111/j.1365-2257.2006.00801.x | pmid = 16999722 }}
14. ^{{Cite journal | last1 = Dascalescu | first1 = CM. | last2 = Wendum | first2 = D. | last3 = Gorin | first3 = NC. | title = Littoral-cell angioma as a cause of splenomegaly | journal = N Engl J Med | volume = 345 | issue = 10 | pages = 772–3 |date=Sep 2001 | doi = 10.1056/NEJM200109063451016 | pmid = 11547761 }}
15. ^{{Cite journal | last1 = Ziske | first1 = C. | last2 = Meybehm | first2 = M. | last3 = Sauerbruch | first3 = T. | last4 = Schmidt-Wolf | first4 = IG. | title = Littoral cell angioma as a rare cause of splenomegaly | journal = Ann Hematol | volume = 80 | issue = 1 | pages = 45–8 |date=Jan 2001 | doi = 10.1007/s002770000223| pmid = 11233776 }}
16. ^{{cite news|last1=Rappaport|first1=Lisa|title=Large spleen helps explain deep-diving skills of Southeast Asian 'sea nomads'|url=https://www.reuters.com/article/us-health-diving-genetics/large-spleen-helps-explain-deep-diving-skills-of-southeast-asian-sea-nomads-idUSKBN1HQ2G9|accessdate=20 April 2018|work=Reuters|date=19 April 2018}}

External links

  • Splenomegaly and hypersplenism at patient.info
{{Medical resources
| DiseasesDB = 12375
| ICD10 = {{ICD10|Q|89|0|q|80}}, {{ICD10|R|16|1|r|10}}
| ICD9 = {{ICD9|759.0}}, {{ICD9|789.2}}
| ICDO =
| OMIM =
| MedlinePlus = 003276
| eMedicineSubj = ped
| eMedicineTopic = 2139
| eMedicine_mult = {{eMedicine2|med|2156}}
| MeshID = D013163
}}{{Lymphatic organ disease}}{{Digestive system and abdomen symptoms and signs}}

2 : Diseases of spleen|Symptoms and signs: Digestive system and abdomen
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