[Excessive hyperferritinemia as an indication of a reactive hemophagocytosis syndrome].
METHODS
A 17-year-old girl with a history of a polyarthritis of unknown etiology was admitted because of acute fever and general weakness. There were palpable cervical lymph nodes and her body temperature was 39.5 degrees C.


METHODS
GOT was raised to 282 U/1, GPT to 266 U/l lactate dehydrogenase to 1275 U/I and bilirubin to 0.6 mg/dl. The Quick value was 67%, albumin 28 mg/dl. White cell count was decreased to 1700/microl, with 43% granulocytes, 39% lymphocytes, 17% monocytes. Platelet count was 64,000/microl. Ultrasound revealed splenomegaly. Ferritin was markedly raised to 11,860 ng/ml (normal up to 150 ng/ml). An epstein-barr-virus infection was found.


METHODS
Suspecting a reactive hemophagocytosis syndrome, she was treated with prednisolone (2 mg/kg). The diagnosis was confirmed by a bone marrow aspirate. The patient's condition and laboratory values improved rapidly.


CONCLUSIONS
Markedly increased ferritin levels in a clinically septic patient with an underlying rheumatic disease indicates a hemophagocytotic syndrome. High dosage steroid should be started before there is biopsy confirmation of the disease.
Dinser
R
R
Medizinische Klinik I, Universitätskliniken des Saarlandes, Hamburg. robert.dinser@uniklinik-saarland.de
Brunner
J
J
Engel
A
A
D000293:Adolescent; D000410:Alanine Transaminase / Q000097:blood; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001168:Arthritis / Q000150:complications*; D001219:Aspartate Aminotransferases / Q000097:blood; D001663:Bilirubin / Q000097:blood; D001707:Biopsy, Needle; D001853:Bone Marrow / Q000473:pathology; D003937:Diagnosis, Differential; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000175:diagnosis; D005260:Female; D005293:Ferritins / Q000097:blood*; D005334:Fever; D015616:Histiocytosis, Non-Langerhans-Cell / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood; D007958:Leukocyte Count; D008198:Lymph Nodes / Q000473:pathology; D018908:Muscle Weakness; D010976:Platelet Count; D011239:Prednisolone / Q000627:therapeutic use; D013163:Splenomegaly / Q000000981:diagnostic imaging; D014463:Ultrasonography
D000893:Anti-Inflammatory Agents; D005293:Ferritins; D011239:Prednisolone; D007770:L-Lactate Dehydrogenase; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase; D001663:Bilirubin