A 26-month-old boy was admitted because of recurrent fever, generalized erythematous rash, and joint pain. The fever persisted, and the joint pain waxed and waned. The combination of fever, erythematous rash, and joint pain suggested that the patient suffered from a childhood chronic inflammatory illness such as systemic juvenile idiopathic arthritis (sJIA) or chronic infantile neurological cutaneous and articular syndrome (CINCA, alternatively called neonatal-onset multisystem inflammatory disease or NOMID). However, neither of the conditions could explain the unique clinical features and the extraordinary laboratory findings seen in this patient. The patient had characteristic facial features such as flat nasal bridge, frontal bossing, and prominent edema of the eyelids. Marked hepatomegaly was noted, but the spleen was absent. Unlike most of patients with asplenia, this patient did not have any form of congenital heart disease. His brother and sister were healthy. The mother had experienced two intrauterine fetal deaths.
KeywordsHeme oxygenase-1 Oxidative stress Asplenia Hemolytic anemia Endothelial injury
- Mizuno K, Toma T, Tsukiji H, Okamoto H, Yamazaki H, Ohta K, Ohta K, Kasahara Y, Koizumi S, Yachie A (2005) Selective expansion of CD16high CCR2− subpopulation of circulating monocytes with preferential production of haem oxygenase (HO)-1 in response to acute inflammation. Clin Exp Immunol 142:461–470PubMedPubMedCentralGoogle Scholar
- Nakahira K, Kim HP, Geng XH, Nakao A, Wang X, Murase N, Drain PF, Wang X, Sasidhar M, Nabel EG, Takahashi T, Lukacs NW, Ryter SW, Morita K, Choi AM (2006) Carbon monoxide differentially inhibits TLR signaling by regulating ROS-induced trafficking of TLRs to lipid rafts. J Exp Med 203:2377–2389CrossRefGoogle Scholar
- Yachie A, Toma T, Shimura S, Yue L, Morimoto K, Maruhashi K, Niida Y, Ohta K, Kasahara Y, Saikawa Y, Koizumi S (2002) Human HO-1 deficiency and the oxidative injury of vascular endothelial cells. In: Abraham NG (ed) Heme oxygenase in biology and medicine. Kluwer Academic/Plenum Publishers, New YorkGoogle Scholar
- Yamazaki H, Ohta K, Tsukiji H, Toma T, Hashida Y, Ishizaki A, Saito T, Arai S, Koizumi S, Yachie A (2007) Corticosteroid enhances heme oxygenase-1 production by circulating monocytes by up-regulating hemoglobin scavenger receptor and amplifying the receptor-mediated uptake of hemoglobin-haptoglobin complex. Biochem Biophys Res Commun 358:506–512CrossRefGoogle Scholar