Felty’s syndrome

Published on 02/04/2015 by admin

Filed under Internal Medicine

Last modified 22/04/2025

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120 Felty’s syndrome

Instruction

Examine this patient’s abdomen.

Salient features

History

• Fever, weigh loss

• Rheumatoid arthritis

• Leg ulcers, hyperpigmentation.

Examination

• Mild to moderate splenomegaly

• Rheumatoid arthritis.

Proceed as follows:

• Look for the following signs:

• Anaemia
• Vasculitis
• Diffuse pigmentation
• Leg ulcers.

Diagnosis

This patient has moderate splenomegaly with rheumatoid arthritis (lesions) caused by Felty syndrome.

Advanced-level questions

What is Felty syndrome?

It is a rare complication of rheumatoid arthritis in which there is leucopenia 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’ (Fig. 120.1), a premalignant disorder of the T lymphocyte, occurs in one-third of the patients with Felty syndrome in rheumatoid arthritis.

image

Fig. 120.1 Peripheral blood smear with large granular lymphocytes.

(With permission from Firestein et al. 2008.)

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:

• 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

• For symptoms in massive organomegaly.

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

The presence of Howell–Jolly bodies (in all) (Fig. 120.2), siderocytes and spur cells (in 25% of patients).

image

Fig. 120.2 Howell-Jolly body in an erythrocyte.

(With permission from Goldman L, Ausiello DA 2007.)

What are the causes of asplenia?

• 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 cause significant risk.

• Less common infections are 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?

• Vaccination:

• 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 caused by 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.

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