Splenomegaly

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Splenomegaly
Massively enlarged spleen, the result of extramedullary hematopoiesis, is outlined above. This patient's left upper quadrant appears more full than the corresponding area on the right.
Image courtesy of Charlie Goldberg, M.D., UCSD School of Medicine and VA Medical Center, San Diego, California
ICD-10 Q89.0, R16.1
ICD-9 759.0, 789.2
DiseasesDB 12375
MedlinePlus 003276
eMedicine ped/2139  med/2156
MeSH D013163

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Overview

Splenomegaly is an enlargement of the spleen, which usually lies in the left upper quadrant (LUQ) of the human abdomen. It is one of the four cardinal signs of hypersplenism, the other three being cytopenia(s), normal or hyperplastic bone marrow, and a response to 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.

Complete Differential Diagnosis Splenomegaly

In alphabetical order. [1] [2]

Symptoms and signs

Symptoms may include abdominal 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.[4]

Causes

Splenomegaly grouped on the basis of the pathogenic mechanism

Increased function Abnormal blood flow Infitration
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
SLE
Serum sickness
Autoimmune hemolytic anemia
Immune thrombocytopenia
sarcoidosis
drug reactions
Extramedullary hematopoiesis
Myelofibrosis
Marrow infiltration by tumors, leukemias
marrow damage by radiation, toxins

Organ Failure

Cirrhosis
congestive heart failure
Vascular
hepatic vein obstruction
portal vein obstruction
Budd-Chiari syndrome
splenic vein obstruction
Infections

hepatic schistosomiasis
hepatic echinococcosis

Metabolic diseases
Gauchers disease
Niemann-Pick disease
Hurler syndrome and other Mucopolysaccharidoses
Amyloidosis
Tangier disease
Benign and malignant infiltrations
Leukemias(acute,chronic,lymphoid and myeloid)
lymphomas(Hodgkins and non-hodgkins)
myeloproliferative disorders
metastatic tumors(commonly melanoma)
histiocytosis X
Hemangioma,lymphangioma
splenic cysts
hamartomas
eosinophilic granuloma


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

Thalassemia
Kala-Azar (Leishmaniasis)
Portal hypertension of Bilharziasis
Chronic myelogenous leukemia
lymphomas
hairy cell leukemia
myelofibrosis
polycythemia vera
Gauchers disease
chronic lymphocytic leukemia
sarcoidosis
autoimmune hemolytic anemia
Malaria

Treatment

If the splenomegaly underlies hypersplenism, a splenectomy is indicated and will correct the problem. After splenectomy, however, patients have an increased risk for infectious diseases.

After splenectomy, patients should be vaccinated against Haemophilus influenzae and Streptococcus pneumoniae. They should receive annual influenza vaccinations. Long-term prophylactic antibiotics should be given.

See also

References

  1. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:77 ISBN 1591032016
  2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:68 ISBN 140510368X
  3. Kahan, Scott, Smith, Ellen G. In a page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:157
  4. Grover SA, Barkun AN, Sackett DL (1993). "The rational clinical examination. Does this patient have splenomegaly?". JAMA. 270 (18): 2218–21. PMID 8411607. Ovid full text

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