Immune complex deposits have been found in the choroid plexus in patients with systemic lupus erythematosus, and it can be assumed that an immune complex injury to the choroid plexus might be related to the neuropsychiatric disorder seen in patients with SLE. Acute serum sickness was experimentally induced in rabbits by intravenous injection of crystalized BSA. Prednisolone in conventionl dosage was administered to study the immunologic injury of the choroid plexus as well as the mechanisms involved in the prednisolone effect. Light, electron microscopic and immunofluorescent studies were made. The host immunoglobulins(IgG, IgA, IgM) and beta 1 C globulin were demonstrated in the choroid plexus. Histopathological findings included mild to moderate interstitial and perivascular lymphocyte and plasma cell infiltrations and edema. Control animals showed no immune deposits and no histopathologic changes. Electron microscopic findings comparing the immunofluorescent and histopathologic changes were minimal, and showed sparse, vague electron dense deposits particularly in the interstitial spaces, knob-like focal thickening of vascular basement membrane, swelling of endothelial cells, and some accentuation of interstitial cells. The morphologic and functional similarities of the choroid plexus and glomerular basement membrane, the findings in morphologic, electron microscopic and immunofluorescent examinations of the experimental rabbits, along with the observed effects of prednisolone, together with similar reports in the recent literature suggest that immunologic injury of the choroid plexus could be considered as a new disease entity. This immunologic injury might play a significant role in neuropsychiatric disorders in the long standing immune complex deposit diseases. The very interesting finding is the nature and function of the interstitial cell between the endothelial (vascular) and epithelial side basement membranes, and speculation as to whether or not the role of this interstitial cell in choroid plexus injury may be in its possible analogy with glomerular mesangial cells.
Acute Disease ; Animal ; Choroid Plexus/drug effects ; Choroid Plexus/immunology* ; Choroid Plexus/pathology ; Lupus Erythematosus, Systemic/etiology ; Prednisolone/pharmacology* ; Rabbits ; Serum Sickness/chemically induced ; Serum Sickness/complications* ; Serum Sickness/immunology

OBJECTIVEImmunsuppressive therapy is a major therapy for severe aplastic anemia, and antithymocyte /antilymphocyte globulin (ATG/ALG) is usually used. This study investigated the therapeutic effect of ATG/ALG on severe aplastic anemia and explored the management of therapy-related complications.

METHODSClinical data of 28 children with severe aplastic anemia who received ATG/ALG treatment from December, 1994 through to September, 2005 were analyzed retrospectively.

RESULTSOf the 28 patients, 2 were nearly cured (7.1%), 4 were relieved (14.3%) and 12 were improved (42.9%) based on a hemoglobin/white blood cell/platelet count. These results represented an overall effective rate of 64.3%. Clinical evidence of serum sickness developed in 19 patients, manifesting as fever (n = 9), cutaneous eruptions (n = 12), arthralgias (n = 7), myalgia (n = 7) and arthrocele (n = 3). Serum sickness occurred 5-17 days after ATG/ALG administration and lasted for 1-15 days (mean 4.4 days). Three children with mild serum sickness symptoms recovered without any treatment. The symptoms of the other 16 patients disappeared after 3-5 days of methylprednisolone treatment (10 mg/kg daily). However, 3 patients had relapses at 2-4 days after termination of methylprednisolone therapy. Another course of methylprednisolone therapy was administered to the 3 patients until the symptoms disappeared. The patients with no serum sickness or with mild serum sickness had a better response to ATG/ALG therapy than those who had severe serum sickness (100% vs 60%; P < 0.05).

CONCLUSIONSATG/ALG therapy for severe aplastic anemia is effective. Serum sickness is a common complication in children with severe aplastic anemia following ATG/ALG therapy, but can be improved by methylprednisolone application.

Anemia, Aplastic ; drug therapy ; Antilymphocyte Serum ; adverse effects ; therapeutic use ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Serum Sickness ; etiology ; T-Lymphocytes ; immunology

Adolescent ; Anemia, Aplastic ; drug therapy ; immunology ; Antilymphocyte Serum ; administration & dosage ; adverse effects ; Child ; Child, Preschool ; Drug Hypersensitivity ; etiology ; Female ; Humans ; Immunosuppressive Agents ; administration & dosage ; adverse effects ; Infant ; Male ; Retrospective Studies ; Serum Sickness ; chemically induced ; T-Lymphocytes ; immunology ; Treatment Outcome