Examine this patient's abdomen.



· Fever, weight loss.

· Rheumatoid arthritis.

· Leg ulcers, hyperpigmentation.


· Mild to moderate splenomegaly.

· Rheumatoid arthritis.

Proceed as follows:

Look for the following signs:

· Anaemia.

· Vasculitis.

· Diffuse pigmentation.

· Leg ulcers.


This patient has moderate splenomegaly with rheumatoid arthritis (lesions) due to Felty's syndrome.


What is Felty's syndrome?

It is a rare complication of rheumatoid arthritis in which there is leukopenia with selective neutropenia and splenomegaly. The bone

marrow is typically hyperplastic. The disease typically manifests late in the course of 'burnt-out' joint disease. The prognosis is poor

because of recurrent Gram-positive infections. The 'large granular lymphocyte syndrome', a pre-malignant disorder of the T

lymphocyte, may mimic Felty's syndrome in rheumatoid arthritis.

Note. Splenectomy does not prevent sepsis and may hasten the onset of malignancy.

What do you understand by the term 'hypersplenism'?

It implies removal of erythrocytes, granulocytes or platelets from the circulation by the spleen. Removal of the spleen is indicated

when the underlying disorder cannot be corrected.

Criteria for hypersplenism include the following:

· Enlarged spleen.

· Destruction of one or more cell lines in the spleen.

· Normal bone marrow.

What are the indications for splenectomy?

· Hereditary spherocytosis in children.

· Autoimmune thrombocytopenia or haemolytic anaemia not controlled by steroids.

· To ameliorate hypersplenism in Gaucher's disease, thalassaemia, hairy cell leukaemia.

· Symptoms due to massive organomegaly.

What are the characteristic cells in a peripheral blood smear following splenectomy ?

The presence of Howell-Jolly bodies (in all), siderocytes and spur cells (in 25% of patients).

What are the causes of asplenia?

Causes of asplenia include:

· Diminished function: sickle cell disease, thalassaemia, coeliac disease, SLE, lymphoma, leukaemia, amyloidosis.

· Surgical removal: hereditary spherocytosis, thalassaemia, lymphoma, idiopathic thrombocytopenia, traumatic rupture.

To which infections is the asplenic patient susceptible?

· Streptococcus pneumoniae, Haemophilus influenzae B, Neisseria meningitidis and malaria parasites pose a significant risk.

· Less common organisms include babesiasis, caused by tick-borne protozoa, and infection with Capnocytophaga canimorus

following a dog bite.

What precautions would you advise an asplenic patient in the outpatient clinic?


· Pneumococcal vaccine - a single injection; booster doses at 5-10-year intervals. · Hib vaccine - a single dose at the same time as

pneumococcal immunization.

· Meningococcus groups A and C vaccine (although the majority of infections are due to group B strains for which there is no

vaccine of proven efficacy).

Antibiotic prophylaxis

Phenoxymethylpenicillin or amoxicillin.

Foreign travel

Antimalarial chemoprophylaxis, other precautions (insect repellants, screens at night).

Augustus R. Felty (1895-1964) was a physician at Hartford Hospital, Hartford, Connecticut. He described this syndrome while he

was working at the Johns Hopkins Hospital, Baltimore.