Hepatosplenomegaly

Notes

## Basic introduction

  • This is a nonspecific medical sign that is characterized by enlarged liver and spleen
  • This is the enlargement of the liver to more than 3cm below the costal margin that is accompanied by the enlargement of the spleen to more than “just being palpable”
  • It has multiple etiologies

## Major causes of hepatosplenomegaly

  • Anemia (contributes the majority of cases):

_Iron-deficiency anemia

_Hemolytic disorders:

||| Sickle cell anemia

||| Thalassemia

||| Autoimmune hemolytic anemia

||| Hereditary spherocytosis

||| Isoimmunization disorders such as ABO and Rh incompatibility

  • Infections and inflammation:

_Hepatitis

||| Viral hepatitis

||| Bacterial

||| Toxic

_Cholangitis (infection of the bile duct, mainly bacterial)

_Epstein-bar virus (EBV) infection or Glandular fever

_Cytomegalovirus infection (CMV) or pseudo-glandular fever

_Amoebic liver abscess

_Pyogenic liver abscess

_Malaria

_Actinomycosis

_Hydatid cyst

_Leptospirosis

_Kala-azar

_Toxoplasmosis

_Salmonellosis

_Brucellosis

_Disseminated TB

_Congenital syphilis

_Schistosomiasis

  • Cancers

_Hepatocellular carcinoma (HCC)

_Hepatoma

_Myeloma

_Leukemia

_Histiocytosis X

_Metastatic disease such as Neuroblastoma

  • Bile duct obstruction (such as biliary atresia)
  • Cardiac disorders
  • Systemic disorders

_Connective tissue diseases

_Cystic fibrosis

_Diabetes mellitus

_Malnutrition

_Obesity

  • Metabolic disorders

_Disorders of carbohydrate metabolism

 ||| Galactosemia

 ||| Glycogen storage disease

 ||| Hereditary fructose intolerance

 ||| Fatty infiltration

_Disorders of amino acid metabolism

 ||| Tyrosinemia

 ||| Urea cycle defects

_Disorders of lysosomal storage

 ||| Mucopolysaccharidoses

 ||| Lipidoses

 ||| Glycoprotein disorders

_Wilsons disease

_Porphyria

  • Drugs

_Statins

_Macrolides

_Amiodarone

  • Trauma
  • Vascular disorders
  • Serum sickness
  • Sarcoidosis
  • SLE
  • Juvenile Rheumatoid Arthritis
  • Amyloidosis
  • Alcoholic liver disease
  • Budd-Chiari
Symptoms
  • Depends on underlying cause(s)
  • Enlarged liver (abdominal mass)
  • Sometimes, jaundice
  • Nausea
  • Pruritus
  • Pyrexia
  • Weight loss
  • Change in bowel habit
  • Pale stool
  • Dark urine
  • Stunting of growth
Diagnosis
  • Clinical review, mainly physical examination

_Palpation

_Percussion

  • Investigations should be directed by the suspected diagnosis
  • Full Blood Count (FBC)
  • Differential Reticulocyte count (high in hemolytic anemia)
  • ESR (elevated in inflammatory diseases)
  • Imaging

_Abdominal ultrasound

_CT scan

_Chest X-ray (especially to detect cardiac cause)

_ECG

  • Liver Function Tests (LFTs)

_SGOT & SGPT (high in hepatitis and hepatic necrosis)

_Albumin

_Alkaline phosphatase (elevated in hepatobiliary obstruction or liver abscess)

_Bilirubin (hemolytic anemia)

_Serum alpha1-antitrypsin, A1A, (it prevents the damage of tissues, including the liver, by trypsin in the body)

_Serum ceruloplasmin (low in Wilson’s disease)

_Total protein (low in Kwashiorkor)

  • Prothrombin time (PT) and activated partial thromboplastin time (APTT) - (high in liver parenchymal dysfunction)
  • Percutaneous liver biopsy
  • Hepatitis B Surface Antigen (HBsAg) detection test
  • Alpha- feto protein (presence of hepatoblastoma)
Differential
  • Abdominal tumor
  • Displacement of liver by the diaphragm
  • Fecal material
  • Spinal deformity
Prevention
  • Prevention of underlying cause(s)
Reference

1. Kenyan Ministry of Health. Clinical guidelines for management and referral of common conditions at levels 4-6. Hospitals. 2009; 3:259-261.http://apps.who.int/medicinedocs/documents/s21000en/s21000en.pdf

2. Wilson, S., Vennervald, B. J., Kadzo, H., Ireri, E., Amaganga, C., Booth, M., ... & Muchiri, E. (2007). Hepatosplenomegaly in Kenyan schoolchildren: exacerbation by concurrent chronic exposure to malaria and Schistosoma mansoni infection. Tropical Medicine & International Health, 12(12), 1442-1449.

3. Bricks, L. F., Cocozza, A. M., Resegue, R., Sucupira, A. C. S., Rodrigues, D., KOBINGER, M. E., ... & BRESOLIN, A. M. B. (1998). Experience in the evaluation of children with hepatosplenomegaly at a teaching ambulatory, Sao Paulo, Brazil. Revista do Instituto de Medicina Tropical de São Paulo, 40(5).

Management
  • Treatment of underlying cause(s)
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