Felty’s Syndrom – What is it? Medical Review

Last Updated: October 14, 2014 at 6:48 am

Felty’s Syndrom

Felty's Syndrom

What is Felty’s Syndrom?

Felty’s syndrome is defined by the clinical triad of neutropenia, splenomegaly, and nodular Rheumatoid Arthritis and is seen in less than 1% of patients, although its incidence appears to be declining in the face of more aggressive treatment of the joint disease. It typically occurs in white patients in the late stages of severe Rheumatoid Arthritis. T cell large granular lymphocyte leukemia (T-LGL) may have a similar clinical presentation and often occurs in association with Rheumatoid Arthritis. – Reference  Harrison’s Principles of Internal Medicine: Volumes 1 and 2, 18th Edition

In Felty’s syndrome—the triad of

Rheumatoid Arthritis Splenomegaly and Neutropenia Rheumatoid Hand Spleen produced antibodies can shorten neutrophil life span, while LGLs can attack marrow neutrophil precursors. Splenectomy may increase the neutrophil count in Felty’s syndrome and lower serum neutrophil binding IgG. Some Felty’s syndrome patients also have neutropenia associated with an increased number of LGLs. Splenomegaly with peripheral trapping and destruction of neutrophils is also seen in lysosomal storage diseases and in portal hypertension. – Reference  Harrison’s Principles of Internal Medicine: Volumes 1 and 2, 18th Edition

Felty’s Syndrome Risk factors

Age of onset 50-70 yrs Female > male Caucasians > blacks Long-standing Rheumatoid Arthritis Deforming but inactive disease Seropositive for Rheumatoid Fever – Reference Davidson’s Principles and Practice of Medicine

Felty’s Syndrom Common clinical features

• Splenomegaly • Lymphadenopathy • Weight loss • Skin pigmentation • Keratoconjunctivitis sicca • Vasculitis, leg ulcers • Recurrent infections • Nodules – Reference Davidson’s Principles and Practice of Medicine  So why you need to know the disease which is rare? Because you can find it in Neutropenia

Causes of Neutropenia:

 Bone marrow disorders

Aplastic anemia

Pure white cell aplasia

Congenital

Cyclic neutropenia

Drugs: sulfonamides, chlorpromazine, procainamide, penicillin, cephalosporins, cimetidine, methimazole, phenytoin, chlorpropamide, antiretroviral medications

Benign chronic

Large granular lymphocytic leukemia

Peripheral disorders

Hypersplenism

Sepsis

Immune

Felty’s syndrome

HIV infection

Reference – CURRENT Medical Diagnosis and Treatment 2012, Fifty-First Edition (LANGE CURRENT Series)

Bottom Line Of Felty’s Syndrom:

A small subset of patients with rheumatoid arthritis have Felty syndrome, the occurrence of splenomegaly and neutropenia, usually in the setting of severe, destructive arthritis. Felty syndrome must be distinguished from the large granular lymphocyte syndrome, with which it shares many features. – Reference Kumar and Clark’s Clinical Medicine

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