Granulocytic Sarcoma in Childhood Acute Myelogenous Leukemia: Clinical Characteristics and Management / 대한소아혈액종양학회지

PURPOSE: Granulocytic sarcoma (GS), an extramedullary tumor consisting of primitive myeloid cells, is a rare manifestation of acute myelogenous leukemia (AML). However, GS can occasionally precede the development of systemic leukemia by weeks to years. The objectives of this study are to describe the frequency, clinical characteristics and survival of AML children with GS from a single Korean institute. METHODS: Retrospective review of all the AML children who presented between January, 1995 and June, 2003 was undertaken. RESULTS: GS developed in 9 children among 118 AML patients (incidence, 7.6%). The median age at diagnosis of AML was 82 months (8 months~13 years) with equal sexual distribution. The sites of GS were scalp (n=4), skull (n=3), paranasal sinuses (n=1), external auditory canal (n=1), spinal epidura (n=1), and spinal intramedulla (n=1). The symptoms related with GS were scalp mass (n=4), paraparesis (n=3), facial nerve palsy (n=3), hearing impairment (n=2), and exophthalmos (n=1). In the case with spinal epidural mass, GS preceded the diagnosis of AML by 15 months. Cytogenetics were available in 8 cases, and t (8; 21) was found in five cases. All cases received systemic chemotherapy, with surgical decompression and radiotherapy for 2 patients involving spine. Seven cases received stem cell transplantations (3, allogeneic bone marrow; 4, autologous peripheral blood). The 5-yr event-free survival was 35.0% by Kaplan-Meier method. All 3 allografted patients are alive (86 mo, 5 mo, 1 mo), while 3 of 4 autografted patients had either died or relapsed. CONCLUSION: GS should be considered in patients with or even without AML who have palpable mass or neurological manifestation. Effective treatment, including allogeneic stem cell transplantation, should be considered to achieve a durable disease control.

A Case of Labial Adhesion

Labial adhesions are postnatal fusion of the labia minora in the midline of varying degrees. They are postulated to be the result of low estrogen levels in the prepubertal child and possibly of a chronic inflammatory process. Topical treatment with conjugated estrogens has been the mainstay of therapy. We experienced one case of labial adhesion in an infant who was treated with estrogen cream for three weeks without any complication. Here we present this case with a brief review of the literature.

A Case of Ramsay Hunt Syndrome Associated with Aseptic Meningitis / 대한소아신경학회지

Ramsay Hunt syndrome(RHS or herpes zoster oticus) is caused by reactivation of latent varicella-zoster virus(VZV) in the geniculate ganglion of the seventh cranial nerve. Unilateral facial paralysis is accompanied by herpetic vesicles on the external auditory canal or in the mouth. The prognosis is not good as that of idiopathic facial palsy. Aggressive treatment with acyclovir, in combination with steroids, is recommended. RHS is thought to affect adults primarily, but a few cases of children with RHS have been reported. We present a case of RHS combined with aseptic meningitis in a previously healthy 10-year-8-month-old boy. He showed a complete peripheral facial palsy on the right side with CSF pleocytosis. Eight days after the onset of the facial palsy, the characteristic herpetic vesicles appeared on the pinna of the right side. The analysis of sera confirmed the clinical diagnosis of RHS with a positive IgG and IgM-ELISA antibody titer of VZV. Although we administered acyclovir and prednisolone adequately to the patient, he has shown an incomplete recovery of the facial palsy on a follow-up visit. The physicians should be prudent in the diagnosis of idiopathic facial palsy or RHS, and must watch for the appearance of vesicles in children with facial palsy.

Long-term Follow-up of an Evans Syndrome with Chronic, Refractory Course: Response to 5 Different Regimens / 대한소아혈액종양학회지

Evans syndrome is the combination of direct Coombs' positive hemolytic anemia and immune thrombocytopenic purpura, in the absence of a known underlying etiology. Being reported rarely in pediatric patients, the syndrome is characterized by periods of remission and exacerbation with viable responses to therapy. Management of the disease remains a challenge despite a variety of therapeutic trials. We experienced a 11-years old male patient of Evans syndrome who was initially presented as having an autoimmune hemolytic anemia 17 months before. Over the 5 years of follow-up, he had a chronic, relapsing courses, showing partial responses to a variety of therapeutic trials, including IVIG, oral prednisolone, methylprednisolone pulse therapy, cyclosporine A and vincristine. A brief review of the literature ensues with the case report.