Case 59 – update 1

Welcome back. The learning points from our first section are outlined below. If you want to skip to the bottom to find out what is now happening with the patient and respond to the next question, please do!

The case so far

Mr Q is a 75 year old man with a 16cm spleen found incidentally on ultrasound. He has a history of diabetes, stroke and hypertension. We asked you to think about the questions you may ask if you saw him in clinic. The suggestions included:

  • ‘B’ symptoms – night sweats, fevers, weight loss.
  • Bleeding history
  • Visual disturbances
  • Early satiety/discomfort on eating
  • Bone pain
  • infections
  • jaundice
  • headache
  • mental fogging
  • aquagenic pruritis
  • erythromelalgia

The diagnoses you suggested as a cause for splenomegaly included:

  • Lymphoma
  • Portal hypertension due to liver disease
  • Chronic Myeloid Leukaemia
  • Primary polycythaemia
  • Myelofibrosis

Other differentials that should be considered include:

  • Acute infections – EBV infection, viral hepatitis, CMV, toxoplasmosis, typhoid.
  • Chronic infections- TB, bacterial endocarditis, syphilis, HIV, brucellosis.
  • Tropical conditions – malaria, leishmaniasis, schistomiasis.
  • Acute Leukaemias
  • Hairy Cell Leukaemia
  • Chronic lymphocytic leukaemia
  • Prolymphocytic leukaemia
  • Haemolytic diseases – Hereditary Spherocytosis, thalassaemia, HbSC disease
  • Megaloblastic Anaemia
  • Rheumatoid Arthritis (Felty’s syndrome)
  • SLE
  • Portal/splenic/hepatic vein thrombosis
  • Congestive cardiac failure
  • Sarcoidosis
  • Metastatic Carcinoma and melanoma
  • Gaucher’s Disease
  • Amyloidosis
  • Benign (i.e. no sequelae on long-term follow up)

How big is big?

A good starting point is, of course, to define splenomegaly. As a haematologist it often feels like everyone who has an ultrasound performed gets slapped with that label!

Radiological diagnosis of splenomegaly is not as straightforward as one might assume, with various parameters that can be assessed. However 11-14 cm as the maximum diameter is well accepted.

What is the most common cause of splenomegaly?

Haematological disorders are, of course, the most common causes of splenomegaly, causing up to 2/3 of all cases in previous studies. Within this bracket the following, by order of frequency, were implicated:

  1. Lymphoma
  2. CML
  3. Haemoglobinopathy
  4. CLL
  5. Myelofibrosis.

It should be noted that these studies were performed in the USA, some dating from the 1960s, and thus may not relate to current patient cohorts (where, in the UK at least,  haemoglobinopathies are usually diagnosed at birth due to antenatal screening) or disease patterns (symptomatic CML being rarer, as incidental FBC abnormalities lead to earlier diagnosis and treatment).

The various investigatations that should be considered will be outlined in the next update.

The case continues

Mr Q is offered the opportunity to undergo bone marrow biopsy, but declines. He is followed up for 2 years, when he begins to become more cytopenic. In his most recent clinic visit his FBC is as follows:

  • Hb          101 g/l
  • WCC       2 x10^9/l
  • Neuts     1.1 x10^9/l
  • Plts         85 x10^9/l.

What conversation would you have with Mr Q now?

What mightyou suggest doing next and why?

As ever – please respond on Twitter including the #teamhaem hashtag. We cannot respond to comments on the blog page.

We will update the case later in the week.


Robertson F et al, Radiology of the Spleen. Eur Radiol 2001, 11:80-95.

O’Reilly RA. Splenomegaly in 2505 patients in a  large university medical centre from 1913 to 1995. 1963-1995: 449 patients. West J Med 1998: 169: 88-97.

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