Overview

Splenomegaly generally denotes a palpably enlarged spleen. Splenomegaly can be found in 3% of the normal population. Causes of an enlarged spleen are multiple (hepatic, haemotological, infection or inflammation).

Anatomy of the spleen

• Location: Left hypochondrium
• Rule of odds (1,3,5,7,9-11):
  • 1 inch thick
  • 3 inches broad
  • 5 inches long
  • 7 ounces weight
  • Underlies 9-11 ribs
• Position: obliquely along long axis of 10^th rib; directed downward, forward and laterally
• Arterial supply: Splenic artery from celiac trunk
• Venous drainage: Splenic vein → Portal vein
• Lymphatic drainage: Celiac (Para-aortic) nodes
• Nerve supply: Sympathetic from celiac plexus

Histology of the spleen

• Red pulp: sinuses lined by endothelial macrophages and cords (spaces)
• White pulp: structure similar to lymphoid follicles

Physiology of the spleen

• Red pulp
• White pulp
• Removal of antibody-coated bacteria and antibody coated blood cells from circulation
• Extramedullary haematopoesis 

Major Causes of Splenomegaly

Common Cause Of Splenomegaly

Classification of Splenomegaly

• Mild – just palpable
• Moderate – below left costal margin and umbilicus
• Massive (>8cm) – beyond umbilicus and goes up to pelvic brim
  • Myeloproliferative disorder
  • Chronic malaria
  • Storage disorders
  • Thalassaemia major
  • Sarcoidosis
  • Gaucher disease

Approach in Children

History

• Fever
• Race
• Recent infection
• Recent travel
• Constitutional signs – systemic illness
• Signs and symptoms of splenomegaly – Satiety, abdominal discomfort, dragging sensation, abdominal pain, shoulder tip pain

Examination

Perform GIT examintion

• Lymphadenopathy – infection or malignancy
• Jaudine – haemolytic anaemia or liver disease
• Hepatomegaly  – Liver disease, myeloproliferative disorder
• Spider angiomata – Liver disease
• Rashes and or joint swelling – Systemic autoimmune disease
• Petechiae and ecchymoses – thrombocytopaenia , malignant haemotolgic disease

Difference Between Enlarged Spleen and Kidney Examination

Approach in Adults

History

• Recent trauma
• Race
• Fever
• Recent infection
• Recent travel
• Constitutional signs – systemic illness including malignancy and autoimmune disease
• Pruritis – Myeloproliferative disorder
• Alcohol or hepatitis – portal hypertension secondary to liver disease
• Symptoms of splenomegaly – Satiety, abdominal discomfort, dragging sensation, abdominal pain, shoulder tip pain

Examination

Perform GIT examination

• Murmur- infective endocarditis, amyloidosis, rheumatic fever
• Lymphadenopathy – infection or malignancy
• Jaundice – haemolytic anaemia or liver disease
• Hepatomegaly  – Liver disease, myeloproliferative disorder
• Spider angiomata – Liver disease
• Rashes and or joint swelling – Systemic autoimmune disease
• Petechiae and ecchymoses – thrombocytopaenia , malignant haemotologic disease

Laboratory Investigations

• Full blood count
  • Neutropaenia
  • Anaemia
  • Thrombocytopaenia
  • Neutrophilia – infection
• Blood smear
  • Sepsis or malaria – Invading organisms maybe identified
  • Mycoplasma pnenomiae – Red blood cell agglutination due to presence of cold agglutinins
  • Infectious mononucleosis – Red blood cell agglutination due to presence of cold agglutinins and atypical lymphcytes
  • Bone marrow malignancy – band forms and nucleated red cells, tear drop red blood cells
  • Myelofibrosis – nucleated red cells, tear drop red blood cells
  • Polycythaemia rubra vera
  • Sickle cell anaemia – multple sickle cells
  • Beta thalassaemia trait – hypochromic and microcytic red blood cells
  • Beta thalassaemia intermedia – hypochromic, microcytic red blood cells. Red cell fragements, red cells with bizzare shapes as well
  • Systemic Lupus erythematosus – LE cells (viable neutrophil has ingested nuclear material)
  • Herediatary spherocytosis or autoimmune haemolytic anaemia – spherocytes

• LFT
• Coagulation profile
• Amylase/Lipase
• AMA, Anti CCP, RA factor
• Bone marrow analysis
  • Bone marrow aspiration
  • Bone marrow biopsy

Imaging Investigations

• MRI
• CT
• Ultrasound

Special investigation

• Hemoglobin electrophoresis (↑HbF in B-thalssemia)
• Coomb’s test (+ve in autoimmune hemolysis and -ve in hereditary spherocytosis)
• Red cell enzyme testing (G6PD deficiency)
• Osmotic fragility testing (+ve in Hereditary spherocytosis)
• Flow cytometry for lymphoproliferative profile (CLL, Hairy cell leukemia, lymphomas)
• Erythropoietin level (↓ in Polycythemia vera)
• Coagulation test (Chronic liver disease, DIC in AML, SLE)
• Serum lipase and amylase (Pancreatitis)
• Serum LDH (NHL, AML)
• Serum iron (↑ in Hemochromatosis, Thalassemia)
• Paul-Bunnell test (Infectious Mononucleosis)
• Congo red test (Amyloidosis)
• Serum ACE (Sarcoidosis)
• Napier’s Aldehyde test (Chronic Kala-azar)
• Anti-nuclear antibodies (SLE)
• Rheumatoid factor (RA, Felty’s syndrome)
• HBsAg (Hepatitis)
• Rose-Waaler test (Felty’s syndrome)
• Glucocerbrosidase activity (Gaucher’s disease)
• Sphingomyelinase (Niemann-Pick disease)
• Mantoux skin test (TB)
• Kveim skin test (Sarcoidosis)

Management

Splenectomy

Splenectomy is surgery to remove the entire spleen

Problems after splenectomy:

• Immediate: Increased platelet count may lead to thromboembolic phenomenon
• Long-term: Increased risk of infection with capsulated organisms (like Streptococcus pneumoniae, Nisseria meningitidis, H.influenzae or E.coli), malarial parasites, babesia

Prophylaxis for Post-splenectomy infection:

• Vaccinate 2-3 weeks before elective splenectomy: Pneumococcal vaccine, Hemophilus influenza type B (Hib) vaccine, Meningococcal group C vaccine, Influenza vaccine
• Lifelong Antibiotic prophylaxis: Long-term penicillin V 500mg 12 hourly (erythromycin if allergic to penicillin)
• Revaccination of pneumococcal vaccine: in every 5 years and influenza vaccine anually
• Antimalarial chemoprophylaxis: if needed (travel to endemic area)

Post splenectomy hematological features:

• Thrombocytosis: persists in 30% cases
• WBC count: usually normal but there may be mild lymphocytosis and monocytosis
• Red cell morphology: Howell-Jolly bodies (Nuclear remnants), Pappenheimer bodies (contain sideroblastic granules), Heinz bodies (Denatured hemoglobin), Target cells, Nucleated erythrocyte (occasionally)