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MR Vol.12 No.1 indexに戻る
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MODERN RHEUMATOLOGY
Vol.12 No.1 |
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A case report: a pediatric patient with
acute lupus hemophagocytic syndrome; differences from reactive
hemophagocytosis
caused by hypercytokinemia |
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| Y. Inamo1, N. Ryou1, O. Abe1, S. Fuchigami1,
K. Hashimoto1, T. Fuchigami1, K. Hayashi2 |
(1)Department of General Pediatrics and Pediatric
Rheumatology, Nihon University Nerima-Hikarigaoka Hospital, 2-11-1Hikarigaoka,
Nerima-ku, Tokyo 179-0072, Japan e-mail: y-inamo@pb3.so-net.ne.jp
(2)Department of Clinicopathology, Nihon University Nerima Hikarigaoka Hospital,
Tokyo, Japan |
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| Abstract |
| Abstract We report the youngest known girl
with acute lupus hemophagocytic syndrome (ALHS) at the onset of
her illness. We investigated the pathogenesis of ALHS by assessment
of factors thought to influence the onset, such as cytokines, Th1/Th2
balance, immune complexes, and autoantibodies. A girl 8 years and
10 months old with systemic lupus erythematosus (SLE) had high
fever, pancytopenia, and hemophagocytosis in the bone marrow. We
diagnosed SLE complicated by ALHS. Treatment with predonisolone
(2 mg/kg/day) was started, and her clinical features improved.
Th2 dominance of the Th1/Th2 balance, hypocomplementemia, and high
levels of anti-ds-DNA antibody, PAIgG, and immune complexes were
seen, but no hypercytokinenemia, hyperferritinemia, or hypertriglyceridemia.
ALHS at the onset of SLE, excluding that caused by infections,
could be a form of reactive hemophagocytosis caused by excessive
production of autoantibodies and immune complexes. High-dose steroid
therapy is effective without need for immunosuppressive drugs.
Our patient showed hypocomplementemia along with high levels of
anti-dsDNA antibody, anticardiolipin antibody, platelet-associated
IgG (PAIgG), and immune complexes. The autoimmune-associated hemophagocytic
syndrome (AAHS) is thought to involve an autoantibody-mediated
mechanism or an immune complex-mediated mechanism. On the basis
of our data, ALHS could simultaneously involve both these mechanisms.
We demonstrated that there was no hypercytokinemia and no hyperferritinemia
in ALHS associated with Th2 dominance. Autoantibodies and immune
complexes may cause histiocytic hemophagocytosis in ALHS. High-dose
steroids and high-dose immunoglobulin are effective but immunosuppressive
drugs are not needed. |
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| Key words |
| Key words Acute lupus hemophagocytic syndrome
(ALHS) ・ Hypercytokinemia ・ Immune complex ・ Th1/Th2 balance |
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