Nancy L. Dunn

Continuous lidocaine infusion for the relief of refractory malignant pain in a terminally ill pediatric cancer patient

Despite aggressive pain management with opiates, debilitating pain still occurs in a subset of children with terminal cancer. A 5-year-old girl with metastatic retinoblastoma, profound opiate tolerance, and refractory pain was treated. Continuous lidocaine infusion was initiated at a dose of 35 microg/kg per minute and increased over 4 days to 50 microg/kg per minute, at which point the patient was discharged for continued end-of-life comfort care. The patient had excellent pain relief without the associated lethargy of high-dose opiates. No complicating neuroexcitatory symptoms or cardiac conduction abnormalities were experienced. Intravenous lidocaine may be an effective alternative to opioids in the treatment of refractory malignant pain in the pediatric patient with terminal cancer.

Subcutaneous fat necrosis of the newborn.

Abstract We present the MRI findings in a case of subcutaneous fat necrosis of the newborn. To our knowledge, the MRI findings of this entity have not been reported. Subcutaneous fat necrosis of the newborn is an uncommon, benign process in full-term infants. Hypercalcemia may be a potentially life-threatening complication of this otherwise self-limiting process.

Benign Transient Hyperphosphatasemia in Children with Leukemia and Lymphoma

A temporary elevation of serum alkaline phosphatase has been described in young children who have no evidence of liver or bone disease. This phenomenon has been termed benign hyperphosphatasemia of infancy. Its occurrence is described in three children undergoing chemotherapy for acute lymphoblastic leukemia and lymphoma. All three children were in remission and in the consolidation or maintenance phase of their therapy when the hyperphosphatasemia occurred. All children were also receiving methotrexate (IM and IV), oral 6-mercaptopurine, and oral sulfamethoxazole/trimethoprim. Although these agents are associated with hepatotoxicity, other liver transaminases (ALT, AST) remained at normal concentrations, and there was an elevation only in the bone isoenzyme of alkaline phosphatase, thus making hepatic toxicity an unlikely etiology for the hyperphosphatasemia. No alteration in chemotherapy was necessary for resolution of the elevated alkaline phosphatase in these children.

Aplastic anemia following therapy for absence seizures with ethosuximide

We describe a case of aplastic anemia in an 8-year-old girl which was diagnosed 8 months after initiation of ethosuximide as treatment for absence seizures. Blood counts had been previously monitored and were normal. The patient successfully underwent allogeneic bone marrow transplantation. Only 8 cases of ethosuximide-associated aplastic anemia have been reported, and in only one of these reports, was ethosuximide used as a single antiepileptic agent. This rare, but potentially fatal complication of ethosuximide raises the question of whether routine monitoring of blood counts during ethosuximide therapy is useful and should be undertaken.

864 CHILDHOOD ACUTE LYMPHOBLASTIC LEUKEMIA WITH B-CELL PHENOTYPE (B-ALL)

B-ALL is uncommon in children and is associated with a poor prognosis. No distinctive clinical features have been identified. At present B-ALL is defined immunologically by the presence of surface immunoglobulins (slg+) on leukemic lymphoblasts. Since 1976 6/56 (11%) of our newly diagnosed ALL patients have been sIg+ and their data are presented below.Blasts from all six patients expressed Ia-like antigens, a normal B cell alloantigen; 2/6 reacted with rabbit antiserum specific for human thymic lymphocytes; and 1/6 had a common ALL antigen characteristic of null cell ALL. Receptors for the lectin peanut agglutinin (PNA) were present on 3/5 tested and these 3 patients have died. 2/5 did not express PNA receptors and remain in remission at 36+ and 29+ months.We conclude that: (1) B-ALL is immunologically heterogenous with some patients simultaneously expressing a common ALL antigen or certain T cell characteristics and (2) the presence of receptors for PNA appears to have prognostic significance in B-ALL.

Clinical and immunological correlates of leukemia cutis in childhood.

Clinical features of three children with acute lymphoblastic leukemia complicated by leukemia cutis are described. All three patients appeared to have null cell disease, in contrast to adult cutaneous lymphoproliferative diseases which are generally T‐cell disorders. Morphologically normal bone marrow specimens from one of these patients plus an additional four patients with isolated extramedullary relapses were also studied and found to be reactive to antisera defining leukemia associated antigens and/or human Ia‐like antigens during six of eight relapse episodes. Such data may imply the need for systemic as well as local therapy when “isolated” extramedullary relapse occurs.

Unusual Presentation of Ewing Sarcoma with t(21;22) in a 3-Year-Old Boy

Purpose: We describe a 3-year-old boy with widespread, metasta-tie Ewing sarcoma and an unusual translocation. involving chromosomes 21 and 22. Materials and Methods: Cylogenetic studies were performed on a biopsy of the primary tumor. These included GTG banding and fluorescence in situ hybridization. Results: A balanced translocation between chromosomes 21 and 22 was noted with translocation breakpoints at bands 21q22 and 22ql2. Conclusions: The t(21:22) translocation represents a new cyto-genelic abnormality that may be associated with Ewing sarcoma. Its prognostic significance, if any. remains to be determined.

631 NASOPHARYNGEAL LYMPHOEPITHELIOMA

Nine previously unreported cases of lymphoepithelioma of the nasopharynx are presented. Mean age at diagnosis was 13.6 yrs. (range 9-19 yrs). Seven patients were black and 5 were male. Symptoms were present from 6 wks.-4 mos. (X 2.7 mos.) prior to diagnosis and included painful adenopathy, carache or trismus in 7 patients. Only one had metastases beyond the cervical nodes (pulmonary) at diagnosis.Treatment consisted of radiation to the nasopharynx (5290-7000 rad) and neck (4780-6500 rad). Chemotherapy regimens varied, but all included adriamycin and cyclophosphamide. The patient with pulmonary metastases also received lung irradiation.Follow-up has ranged from 2-36 mos. (X 20.3 mos.). Two patients have died, 22 and 14 mos. post-diagnosis. The first developed local recurrence and distant metastases at 20 mos. The second, who presented with distant metastases, died of progressive pulmonary disease despite good local control. One patient had cervical and orbital metastases at 8 mos., received an additional 3000 rads, and is disease-free at 32 mos. Six patients remain disease-free from 2-36 mos. (X 19 mos.).We conclude that: 1. Although a delayed diagnosis is common, the prognosis is favorable when disease is localized. 2. The primary treatment is radiation to the tumor and regional lymphatics bilaterally. The benefit of adjunct chemotherapy is in doubt.

The Role of the Practitioner in the Care of Children with Acute Leukemia

1. Nancy L. Dunn 2. Harold M. Maurer 1. Department of Pediatrics, Childrens Medical Center, Medical College of Virginia, MCV Station Box 121, Richmond, VA 23298 The prognosis for a child with acute leukemia has improved dramatically over the past two decades. There are many factors that account for the improved survival rate including: better supportive care, especially for infectious and hematologic complications; sophisticated diagnostic techniques; and more effective multimodality treatment. The purpose of this article is to provide a guide for handling some of the more common questions and problems that may arise in the course of managing the care of a child with leukemia in practice. The classification of leukemia, previously based simply on the morphology of the bone marrow elements, is now determined by a complex array of morphologic, cytochemical, and immunologic characteristics. The determination of cell surface and cytoplasmic markers by immunologic assays now permits the subclassification of acute lymphocytic leukemia, the most common form of childhood leukemia, into T, B, null, and pre-B subtypes (Table 1). The T and B cell subtypes are associated with a poorer prognosis than the null and pre-B cell subtypes and treatment is tailored accordingly. Antileukemic therapy also has undergone change and refinement. The 1950s and 1960s brought many new cytotoxic drugs, first used singly and then in combinations. The end of this era was marked by the discovery of the value of prophylactic CNS treatment, using cranial irradiation and intrathecal methotrexate, in greatly reducing the incidence of meningeal leukemia.

805A PROGNOSIS AFTER 3-YEAR SURVIVAL IN CHILDHOOD ACUTE LYMPHOBLASTIC LEUKEMIA (ALL)

The appropriate duration of therapy for childhood ALL remains controversial.82 children(M:F, 47:35) with ALL were diagnosed between 1965-1975. Forty (49%)children, including 18 males,remained in complete continuous remission (CCR) at 3 years. Between the third and fifth years of therapy, 3 males and 3 females relapsed. The sites of initial relapse were the CNS in 5(4 had received CNS prophylaxis) and a simultaneous marrow and testicular relapse in the sixth. Five were initially high risk patients by age(<2 or >8 years)or white count(≥30,000/mm3) criterian. One additional child died, in remission, of varicella complications.33 patients (40%) remained in CCR at least 5 years. They had received chemotherapy for a median of 61 mos. Followup times off therapy ranged from 6-178 mos (median 36+ mos). One boy in this group subsequently relapsed in the marrow 16 months after therapy ended.We conclude that the relapse rate of 17.5% (7/40) after 3 years of CCR is comparable to the 20% relapse rate reported by St. Jude Childrens Research Hospital (NEJM 300:269, 1979), although our patients received therapy for an additional 2+ years. Continuing therapy beyond 3 years would therefore seem to subject patients to continued potential morbidity without improving ultimate prognosis.