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Felty's syndrome

Triad of rheumatoid arthritis (RA), hypersplenism & leukopenia. Epidemiology: 1) occurs in < 1% of patients with RA 2) 2/3 of patients are women 3) mean duration of RA prior to onset of Felty's syndrome is 15 years Clinical manifestations: 1) Felty's triad of rheumatoid arthritis, splenomegaly, neutropenia a) erosive polyarthritis (RA) b) leukopenia (neutropenia) c) hypersplenism, splenomegaly 2) other manifestations may be present a) recurrent fever with & without apparent infection b) rheumatoid nodules are common c) weight loss d) lymphadenopathy e) skin hyperpigmentation f) cutaneous ulcers in lower extremities g) vasculitis h) peripheral neuropathy i) keratoconjunctivitis sicca j) xerostomia k) pleuritis Laboratory: 1) complete blood count (CBC) a) leukopenia 1] profound neutropenia < 500/mm3 is rare 2] lymphopenia in 1/3 of patients b) anemia c) thrombocytopenia (40%) 2) peripheral blood smear - large granular lymphocytes in 40% 3) serology a) high titers of rheumatoid factor (RF) b) antinuclear antibody (ANA) in 2/3 of patients c) decreased complement components (CH50) occurs with vasculitis d) HLA-DR4(+) 4) bone marrow biopsy a) myeloid hyperplasia with maturation arrest b) marrow is rarely hypocellular Complications: 1) bacterial infections, especially of the skin & lungs 2) patients commonly die of sepsis, despite aggressive therapy 3) large granular lymphocyte syndrome Management: 1) corticosteroids 2) methotrexate 3) lithium carbonate 4) granulocyte colony-stimulating factor (G-CSF) 5) splenectomy (except LGLS) a) mitigates hematologic abnormalities, recurrent bacterial infections & leg ulcerations b) does NOT affect overall course of rheumatoid arthritis

Specific

large granular lymphocyte syndrome (LGLS, LGL)

General

syndrome rheumatoid arthritis (RA)

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 76
  2. DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 885
  3. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 829
  4. Medical Knowledge Self Assessment Program (MKSAP) 11, 17. 18. American College of Physicians, Philadelphia 1998, 2015, 2018.
  5. Owlia MB, Newman K, Akhtari M. Felty's Syndrome, Insights and Updates. Open Rheumatol J. 2014 Dec 31;8:129-36. eCollection 2014. PMID: 25614773 Free PMC Article