assessment and investigation of splenomegaly

Last reviewed 07/2021

Assessment of the patient with splenomegaly

  • clinical assessment begins with a thorough history and examination
    • history may elicit symptoms of pressure effects from the enlarged spleen
      • e.g. left hypochondrial discomfort or early satiety
    • may be symptoms of cytopenias due to hypersplenism:
      • a syndrome comprising splenomegaly; anaemia, leucopenia and/or thrombocytopenia; compensatory bone marrow hyperplasia
    • general systemic symptoms
      • such as fever, sweats, weight loss or lymphadenopathy suggest haematological, malignant, infectious or inflammatory disease
      • thorough systemic enquiry is essential to recognise multi-system disorders such as the collagen diseases and sarcoidosis
    • past medical history
      • may suggest the cause of splenomegaly, though further investigations will be indicated if the presentation is unusual (e.g. massive splenomegaly in a patient with mild congestive cardiac failure)
    • a family history
      • should be carefully elicited, such as of malignancy or anaemia; note that individuals with autosomal recessive conditions like Gaucher's disease often have no affected family members
    • risk factors should be identified for liver disease
      • particularly alcohol intake, and for infectious diseases (travel, sexual contacts, intravenous drug use, exposure to animals and predisposition to infective endocarditis)
  • physical examination
    • splenomegaly can assess degree of enlargement (mild, moderate, massive) depending on body habitus
    • general examination may reveal fever, lymphadenopathy, anaemia, signs of hepatic or inflammatory disease, stigmata of endocarditis, or involvement of any other organ system

Initial investigations in the patient with splenomegaly.

In most patients

  • Haematology
    • Full blood count, peripheral blood film, ESR, clotting
  • Biochemistry
    • Urea and electrolytes, Liver function tests, C-reactive protein, Bone biochemistry,Serum LDH,Vitamin B12, red cell folate
  • Microbiology
    • Monospot test, Serology: hepatitis B/C
  • Immunology
    • Auto-antibodies incl. ANA,Rheumatoid factor
  • Radiology
    • Ultrasound/CT abdomen
    • Plain chest radiograph
  • Bedside Urine dipstick (protein blood)

In selected patients (depending on clinical features)

  • Haematology
    • Direct antiglobulin test, Reticulocyte count, Malaria blood film, Haemoglobin electrophoresis/HPLC
  • Biochemistry
    • Serum ACE, Serum protein electrophoresis, Urine Bence Jones protein
  • Microbiology
    • Peripheral blood cultures, sputum microscopy culture and AAFB, Mantoux test, Serology: HIV, CMV, toxoplasmosis, brucella
  • Radiology
    • Ultrasound abdomen with duplex-Doppler studies
    • CT chest abdomen and pelvis
    • Transthoracic/transoesophageal echocardiogram

Notes:

  • clinical finding of a palpable spleen was previously considered to be evidence of splenic enlargement
    • up to 16% of palpable spleens have been found to be of normal size on radiological assessment
      • while clinical examination can be convincing in massive splenic enlargement, radiology is often needed to confirm the diagnosis
  • on ultrasound examination, 'craniocaudal length' is used most often to measure splenic size; this correlates well with splenic volume, particularly when the right lateral decubitus position is adopted
    • the quoted upper limit of normal varies from 11 to 14 cm
    • other ultrasonographic indicators of splenomegaly include an anteroposterior measure greater than two thirds of the distance between the anterior and posterior abdominal wall, or complex formulae can be used to estimate splenic volume
  • with CT examination, splenic length, the 'splenic index' (product of length, depth and width) and the sum of volumes of consecutive scan slices have all been used
  • radiological confirmation of splenomegaly may therefore depend both on the radiologist's preferred method and a degree of subjective judgement
    • a maximum length of 13 cm is a typical limit (1)

  • the frequency and causes of splenomegaly has been studied retrospectively in US hospital inpatients (2)
    • estimated incidence from 1963 to 1995 was 0.3% of admissions and a diagnosis was reached in 98%, but 12% required a diagnostic splenectomy
    • of all patients with splenomegaly, haematological disease was found in 16-66%, hepatic disease in 9-41%, infectious disease in 9-36%, congestive or inflammatory disease in 4-10% and primary splenic disease (e.g. storage disease) in 1-6%
      • within the haematological disorders, the most common diagnoses were lymphoma (16-44% of all splenomegaly), CML (8-29%), haemoglobinopathy (7-25%), CLL (0-20%) and myelofibrosis (9-16%)

Reference:

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splenomegaly