[Agranulocytosis and connectivitis different from Felty's syndrome: 4 cases. Success of cyclosporin in a case].
Four cases of chronic agranulocytosis in patients with systemic diseases different from Felty's syndrome are reported. Two patients had primary Sjögren's syndrome (confirmed in one, suspected in the other), one had systemic lupus erythematosus and the last patient had an unclassifiable connective tissue disease. Only one severe infection was recorded. The pathophysiological mechanisms involved are discussed. There is a strong suspicion that the cytopenia is of immune origin. Corticosteroid therapy was effective in all four patients. One patient who could not tolerate corticosteroids was successfully treated with cyclosporin A.
Lamy
T
T
Service d'Hématologie Clinique, Hôpital Pontchailou, CHU, Rennes.
Dauriac
C
C
Morice
P
P
Le Prisé
P Y
PY
D000328:Adult; D000368:Aged; D000380:Agranulocytosis / Q000188:drug therapy / Q000209:etiology*; D003240:Connective Tissue Diseases / Q000150:complications* / Q000188:drug therapy; D003524:Cyclosporins / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D005258:Felty Syndrome / Q000175:diagnosis; D005260:Female; D006801:Humans; D008297:Male
D003524:Cyclosporins
Agranulocytose et connectivites distinctes du syndrome de Felty: 4 observations. Succès dans un cas de la ciclosporine.