Felty's Syndrome

Aka: Felty's Syndrome, Felty Syndrome

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II. Pathophysiology

  1. Hematologic changes associated with longterm seropositive, severe Rheumatoid Arthritis (esp. Nodule forming)
    1. Triad: Rheumatoid Arthritis with Neutropenia and Splenomegaly
    2. Similar findings to the non-RA related Large Granular Lymphocyte Syndrome (LGL Syndrome)
  2. Autoantibodies to Histones (esp. H3) and Neutrophil extracellular chromatin traps (NETS)
  3. Associated with HLA-DR4 Haplotype

III. Findings

  1. Fever
  2. Weight loss
  3. Splenomegaly
  4. Lymphadenopathy
  5. Skin
    1. Pigmented Skin Spots on extremities
    2. Subcutaneous Nodules (rheumatoid Nodules)

IV. Labs: Complete Blood Count

  1. Neutropenia (Absolute Neutrophil Count <2000)
  2. Anemia
  3. Thrombocytopenia

V. Management

  1. Treat underlying severe Rheumatoid Arthritis
    1. Methotrexate and azothioprine have been effective in Felty Syndrome
  2. Splenectomy indications
    1. Persistent, severe Neutropenia ((Absolute Neutrophil Count <2000))
    2. Hypersplenism complicated by severe Anemia or severe Thrombocytopenia with Hemorrhage
    3. Chronic nonhealing leg ulcers
    4. Recurrent or severe Bacterial Infections

VI. Complications

  1. Recurrent infections (associated with increased mortality)
  2. Leg ulcers
  3. Vasculitis
  4. Portal Hypertension
  5. Hodgkin's Lymphoma (increased risk)

VII. References

  1. Anderson in klippel (1997) Primer Rheumatic Diseases, ed. 11, p. 167

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Related Studies

Ontology: Felty Syndrome (C0015773)

Definition (MSH) A rare complication of rheumatoid arthritis with autoimmune NEUTROPENIA; and SPLENOMEGALY.
Definition (NCI) A syndrome characterized by the presence of rheumatoid arthritis, splenomegaly, and granulocytopenia. Patients are at an increased risk of infection because of the low white cell counts.
Concepts Disease or Syndrome (T047)
MSH D005258
ICD9 714.1
ICD10 M05.0 , M05.00, M05.09
SnomedCT 57160007, 156482001
English Feltys Syndrome, Syndrome, Felty's, FELTYS SYNDROME, FELTY SYNDROME, Felty's syndrome (diagnosis), Felty syndrome, Rheumatoid arthritis with splenoadenomegaly and leukopenia, Felty's syndrome, unspecified site, Felty's syndrome, site unspecified, feltys syndrome, felty's syndrome, felty syndrome, Felty's Syndrome, Syndrome, Felty, Felty Syndrome, Felty Syndrome [Disease/Finding], Felty's syndrome, Felty's syndrome (disorder), Rheumatoid arthritis, leucopenia AND splenomegaly, Rheumatoid arthritis, leukopenia AND splenomegaly, Felty, rheumatoid; arthritis, with splenoadenomegaly and leukopenia, arthritis; rheumatoid, with splenoadenomegaly and leukopenia, Rheumatoid arthritis, leukopenia and splenomegaly, Rheumatoid arthritis, leukopenia and splenadenomegaly
Japanese フェルティ症候群, ﾌｪﾙﾃｨｼｮｳｺｳｸﾞﾝ
Swedish Feltys syndrom
Czech Feltyho syndrom
Finnish Feltyn oireyhtymä
Russian FELTI SINDROM, REVMATOIDNYI ARTRIT, SPLENOMEGALIIA I NEITROPENIIA, FELTI SINDROM NASLEDSTVENNYI, ФЕЛТИ СИНДРОМ НАСЛЕДСТВЕННЫЙ, РЕВМАТОИДНЫЙ АРТРИТ, СПЛЕНОМЕГАЛИЯ И НЕЙТРОПЕНИЯ, ФЕЛТИ СИНДРОМ
Korean 펠티 증후군
Croatian FELTYEV SINDROM
Polish Zespół Felty'ego
Hungarian Felty-syndroma
Norwegian Feltys syndrom
Dutch artritis; reumatoïd, met spleno-adenomegalie en leukopenie, reumatoïd; artritis, met spleno-adenomegalie en leukopenie, syndroom van Felty, Felty-syndroom, Syndroom van Felty, Syndroom, Felty-
Spanish artritis reumatoidea, leucopenia Y esplenomegalia, artritis reumatoidea, leucopenia y esplenomegalia, síndrome de Felty (trastorno), síndrome de Felty, Síndrome de Felty
German Felty Syndrom, Felty-Syndrom
French Syndrome de Felty
Italian Sindrome di Felty
Portuguese Síndrome de Felty
Derived from the NIH UMLS (Unified Medical Language System)

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