Hemophagocytic syndrome associated with hypercytokinemia in a patients with rheumatoid arthritis

A 65-year old female, who had been suffered from rheumatoid arthritis, was admitted to our hospital because of fever, oral ulcers, perianal skin ulcers, petechiae in the both legs, hepatosplenomegaly and cervical lymphadenopathy. Her laboratory data showed severe anemia, leukocytopenia, and thromboc...

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Bibliographic Details
Published inRyumachi. [Rheumatism] Vol. 43; no. 4; p. 696
Main Authors Sekiuchi, Makiho, Nakabayashi, Kimimasa, Marumo, Tomohumi, Arimura, Yoshihiro, Yamada, Akira
Format Journal Article
LanguageJapanese
Published Japan 01.10.2003
Subjects
Aged
Arthritis, Rheumatoid - complications
Cytomegalovirus Infections - complications
Female
Histiocytosis, Non-Langerhans-Cell - diagnosis
Histiocytosis, Non-Langerhans-Cell - etiology
Histiocytosis, Non-Langerhans-Cell - therapy
Humans
Infusions, Intravenous
Interleukin-6 - blood
Macrophage Colony-Stimulating Factor - blood
Methylprednisolone - administration & dosage
Pulse Therapy, Drug
Receptors, Interleukin-2 - blood
Treatment Outcome
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Summary:A 65-year old female, who had been suffered from rheumatoid arthritis, was admitted to our hospital because of fever, oral ulcers, perianal skin ulcers, petechiae in the both legs, hepatosplenomegaly and cervical lymphadenopathy. Her laboratory data showed severe anemia, leukocytopenia, and thrombocytopenia as well as low PT activity, prolonged APTT, decreased fibrinegen and elevated FDP. In addition to raised values of liver enzymes and triglyceride, marked elevation of several cytokines were found. IgM and IgG class antibodies to cytomegalovirus were demonstrated positive and their titers were 2.60 and 938.0, respectively. The study for the aspiration of bone marrow revealed hemophagocytosis of erythrocytes, leukocytes and thrombocytes. Based upon these findings, she was diagnosed as having hemophagocytic syndrome associated with cytomegalovirus infection. Steroid treatment inducing mini-pulse therapy was introduced to her and bought full recovery from the illness. The association of hemophagocytic syndrome to rheumatoid arthritis was reviewed in the literature and five cases were documented to have good prognosis with steroid treatment.
ISSN:0300-9157