Cure of implantable venous port-associated bloodstream infections in pediatric hematology-oncology patients without catheter removal.

The efficacy of antibiotic treatment of port-associated bloodstream infection without device removal has not been systematically studied. We analyzed the outcome of 43 consecutive port-associated bloodstream infections in pediatric hematology-oncology patients. Etiologies included Staphylococcus epidermidis (30) and Staphylococcus aureus (6). Antibiotics were given through the port for a median of 11 days. Four ports were removed within 72 hours. In 36 (92%) of the remaining 39 episodes, there was a response to antibiotic therapy (defervescence and negative blood culture). In 78% of episodes in which there was a response (excluding two in which the catheters were removed because of mechanical problems), the infections were cured without port removal. Two of the four relapses were cured with a second course of antibiotics. The cure rate was 92% for S. epidermidis infections and 67% for S. aureus infections. Thus, the majority of port-associated bloodstream infections in pediatric hematology-oncology patients can be cured without device removal.

Prognosis and treatment of seizures in children with acute lymphoblastic leukemia.

We reviewed the records of 127 consecutive pediatric patients with acute lymphoblastic leukemia (ALL) to determine the incidence, timing, etiologies, and recurrence rate of seizures in this population. Patients with ALL and seizures were identified retrospectively by review of the records of all pediatric ALL patients who were diagnosed and treated during the years 1983 through March 1993 in a large tertiary-care hospital. Seventeen patients (13%) developed one or more seizures. In 16 patients, seizures occurred during antileukemic treatment, and in almost all of them seizures were related to intrathecal methotrexate (IT MTX) or subcutaneous L-asparaginase treatment. One patient who developed a seizure while not receiving chemotherapy had a history of cerebral infarctions. In 8 patients, (47%), the initial seizure episode was associated with a cerebral lesion. One or more seizures recurred in 6 patients. Four of these patients had an isolated recurrence, in 3 patients < or = 3 months and in 1 patient < or = 6 months after the initial event. Two patients (12%) with static encephalopathy and neurological deficits developed a chronic seizure disorder. There is a significant risk of acute symptomatic seizures in pediatric ALL patients. Most seizures in these patients occur during the acute treatment phase and are most frequently related to side effects of chemotherapy. The long-term recurrence risk is low; recurrence occurs most often in patients with evidence of cerebral structural lesions and neurological deficits. Long-term antiepileptic drug (AED) therapy should be restricted to such patients.

Papillary-cystic neoplasm of the pancreas.

Papillary-cystic neoplasm of the pancreas is a rare, nonfunctioning low-grade malignant tumor seen in young patients, most often female. Ultrasound and CT show a circumscribed, solid nonhomogeneous mass with cystic areas, with peripheral but not central enhancement and occasional calcification. Prognosis after excision is usually excellent. We describe a case of the papillary-cystic neoplasm of the pancreas in a 13-year-old girl to illustrate the radiological findings.

Transient erythroblastopenia of childhood. Prospective study of fifty patients.

PURPOSE: To prospectively evaluate 50 patients with transient erythroblastopenia of childhood (TEC) at a single institution in order to compare those patients presenting with reticulocytopenia (group I) with those presenting in the recovery phase with reticulocytosis (group II); to further describe the clinical course of this common pediatric hematological disorder in a large number of patients, particularly the effect on the neutrophils; and to review the available literature regarding this disorder. PATIENTS AND METHODS: Fifty patients presenting to the Children's Hospital from September 1983 to September 1991 were prospectively evaluated. Those patients with a reticulocytosis and in recovery at the time of diagnosis were included and compared with those with reticulocytopenia. All patients were followed through complete recovery. RESULTS: Thirty-six patients were reticulocytopenic (group I) and 14 had a reticulocytosis (group II). There was a high incidence of neutropenia (64%) in both groups and the resolution of this neutropenia was variable in relation to the resolution of the anemia, with 44% having the lowest ANC before, 9% simultaneous with, and 47% after the peak reticulocyte count. CONCLUSIONS: Our experience with a large group of patients with TEC suggests that neutropenia is an integral part of this disorder, and its recovery has no relation to the recovery of the anemia. A significant number of patients are described in the recovery phase for the first time, and this clarifies this group of patients in order to aid in their diagnosis, particularly in the differentiation from a hemolytic process. Some previously described associations of TEC are not supported in this study of a large number of patients.

Transient erythroblastopenia in a child with Kawasaki syndrome: a case report.

PURPOSE: We describe for the first time the case of a child with Kawasaki syndrome and associated transient erythroblastopenia. PATIENTS AND METHODS: A 5 1/2-month-old infant with Kawasaki syndrome as evidenced by lymphadenopathy, fever, rash conjunctival injection, and extremity changes had associated anemia and reticulocytopenia requiring transfusion and thrombocytopenia. Bone marrow aspiration was consistent with a transient erythroblastopenia. She developed cardiac aneurysms despite therapy with i.v. immunoglobulin and aspirin. RESULTS: The anemia and reticulocytopenia resolved with normalization of her hemoglobin. The platelet count increased and she developed a thrombocytosis characteristic of this clinical entity. She completely recovered without recurrence of either the anemia or reticulocytopenia. CONCLUSIONS: We speculate that the cause of the erythroblastopenia and thrombocytopenia is an inflammatory insult of Kawasaki syndrome on the bone marrow and its various lineages causing the myriad hematological abnormalities now associated with Kawasaki syndrome. It is possible that the i.v. immunoglobulin aids in neutralizing the triggering agent and therefore shortening the duration of the marrow insult.

Acute myocardial infarction complicating prothrombin complex concentrate therapy in an 8-year-old boy with hemophilia A and factor VIII inhibitor.

PURPOSE: An 8-year-old boy with mild hemophilia A and inhibitors developed an acute myocardial infarction during treatment with prothrombin complex concentrates. CONCLUSIONS: This rare complication warrants restriction of dosage and length of treatment with these products to the recommended guidelines. It also appears that noninvasive cardiac monitoring of these patients is beneficial.

Comparison of CT and MRI in the evaluation of therapeutic response in thoracic Hodgkin disease.

Current imaging modalities are accurate in establishing the diagnosis and extent of thoracic Hodgkin disease. After treatment, however, it is extremely difficult to differentiate potential residual active neoplastic disease from scar tissue, or identify early recurrence. We evaluated the contribution of MRI in the assessment of the response to treatment of thoracic Hodgkin disease in the assumption that scar formation would be characterized by low signal intensity in all pulse sequences, whereas active tumor should maintain a degree of high signal intensity on T2-weighted images. In 47 occasions (23 patients) both CT and MRI were able to identify correctly active disease, but had low specificity in confirming remission because of residual tissues masses. High signal intensity on T2-weighted MR images often persisted despite remission, probably because of edema, necrosis, granulation or other factors. MRI was somewhat more specific than CT and may be quite valuable to confirm remission in patients with residual masses that no longer appear hyperintense on T2 after treatment.

Acute undifferentiated leukemia (AUL): a case report and a proposed system of classification.

This report describes a case of HLADR+, CD34- acute undifferentiated leukemia (AUL) diagnosed in an 18-year-old male. A definition of AUL and a system for its classification are proposed on the basis of the current state of knowledge about phenotypic features of AUL cells and their clonal counterparts that exist during early stages of normal hematopoiesis.

Therapeutic adherence to oral medication regimens by adolescents with cancer. I. Laboratory assessment.

Outpatient adherence to oral medication regimens was evaluated in 50 adolescents and young adults with cancer: 21 patients with acute lymphoblastic leukemia or Hodgkin disease who were to take prednisone as a component of their chemotherapy regimen, and 29 patients with Hodgkin disease whose chemotherapy had been discontinued and who were to take penicillin for postsplenectomy prophylaxis. Of the 21 adolescent patients with acute lymphoblastic leukemia or Hodgkin disease who were to take prednisone as outpatients, 11 patients were found to be nonadherent to treatment (52%). Among the 29 adolescents for whom penicillin was prescribed for postsplenectomy prophylaxis, nonadherence was detected in 14 patients (48%). If a method is available, laboratory assessment to determine adherence should be performed in adolescents and young adults receiving long-term therapy, particularly if one wishes to draw valid conclusions regarding efficacy of various treatment protocols.

Severe chronic EBV infection associated with specific EBV immunodeficiency and an EBNA+ T-cell lymphoma containing linear, EBV DNA.

A patient with severe chronic Epstein-Barr virus (EBV) infection (CEBVI) of 6 years duration developed an EBV+ T-cell lymphoma. To determine whether the development of the T-cell tumor was linked to EBV, we studied this patient's EBV-specific immune response and her T-cell tumor tissue for evidence of EBV infection. Peripheral blood lymphocytes from this patient were systematically studied for immune function and response to EBV. Tumor tissue was examined for EBV genome and for evidence of EBV replication. This patient failed to develop anti-EBV nuclear antigen (EBNA) antibodies and had decreased mitogen responsiveness. Her T-cells showed a broad suppression of both autologous and allogeneic B-cells, which was coincident with clinical hypoimmunoglobulinemia. A selective cytotoxic T-cell defect toward autologous EBV-infected B lymphoblasts, which could not be corrected by the addition of lymphokine-mediated T-cell help, was also documented. A lymph node biopsy taken 5 years after her clinical presentation revealed lymph node architecture completely effaced by a diffuse CD3+, CD4+, Ia+, CR2+ T-cell lymphoma containing EBNA and linear, replicating EBV DNA. Select CEBVI patients with humoral and combined cellular aberrations in the immune response to EBV may be predisposed to the development of EBV+ T-cell tumors.

Juvenile chronic myelocytic leukemia: experience with intensive combination chemotherapy.

Six children with juvenile chronic myelocytic leukemia (JCML) with adverse prognostic features were treated with intensive combination chemotherapy similar to that utilized in patients with acute nonlymphocytic leukemia (ANLL). Despite obtaining hematologic remissions after induction therapy, clinical findings of extramedullary disease persisted. The use of intensive post-induction chemotherapy did not erradicate persistent extramedullary disease, and all patients developed hematologic relapse and progressive disease at a median of 8 months. The median survival of the treated patients was 15 months. The use of intensive ANLL therapy in poor prognosis JCML does not improve the survival rates reported with less intensive regimens but does have value in producing hematologic remissions that may be useful in preparing patients for bone marrow transplant.

Plasma protein C levels in children with sickle cell disease.

Since thrombotic phenomena have important roles in the vasoocclusive manifestations in sickle cell disease (SCD), protein C (PC) was determined in 32 children with SCD during steady state and vasoocclusive crisis (VOC) and in controls. Children with SCD during steady state had significantly lower PC levels as compared to controls. During VOC there was marked decrease in PC as compared to levels during steady state, and these levels increased to initial levels or higher with clinical improvement. It is postulated that the decreased levels of PC in SCD are probably secondary to increased consumption as well as decreased production because of altered liver function. This data suggests that decreased levels of PC may increase the risk of thrombosis in these patients.

N-myc oncogene RNA expression in neuroblastoma.

Tumor specimens from 33 patients with neuroblastoma were assayed for amplification of the N-myc oncogene and RNA expression to determine whether N-myc RNA expression levels correlated with N-myc gene amplification and clinical outcome. N-myc gene amplification was detected in one stage II tumor, one stage IV-S tumor, and seven stage III or IV tumors. In each case, N-myc RNA expression roughly paralleled N-myc gene amplification. However, enhanced N-myc RNA expression was not confined to tumors with N-myc gene amplification: all of the early (stage I and II) tumors, five stage IV-S tumors, and 12 advanced (stage III and IV) tumors had levels of N-myc RNA that were elevated up to 50-fold. While N-myc gene amplification correlated with prognosis, there was no such correlation with levels of N-myc RNA expression. The precise role of the N-myc gene in the pathogenesis of neuroblastoma remains unclear.

N-myc oncogene expression in histopathologically unrelated bilateral pediatric renal tumors.

Renal tumors of childhood occasionally exhibit histopathologic and clinical features that preclude accurate diagnosis. Molecular and cell culture techniques may be helpful in better characterizing these cases. This approach was used to examine unusual bilateral renal tumors from a young boy. The left kidney tumor was an undifferentiated neoplasm with light microscopic features suggestive of both Wilms' tumor and neuroblastoma, and the right kidney tumor was identified as multilocular cystic nephroma (MLCN). In vitro tissue culture of tumor cells and hybridization experiments with an N-myc oncogene DNA probe contributed to a revised diagnosis of intrarenal neuroblastoma of the left kidney. A cell line established from the left tumor exhibited neurite outgrowth and was positive for neuron-specific enolase and synaptophysin. N-myc was greater than ten-fold amplified in chromosomal DNA from the left kidney tumor. Measurement of N-myc RNA expression enabled distinction between benign and malignant tumor tissue. The detection of N-myc gene amplification predicted a poor prognosis which was confirmed by the patient's subsequent clinical course.

Recurrent sepsis and gastrointestinal ulceration due to child abuse.

An 11 year old girl developed progressive upper gastrointestinal ulceration and recurrent episodes of intravenous catheter associated polymicrobial septicaemia. Evaluation failed to establish a cause. After exclusion of the parents and careful surveillance of the patient she improved, supporting the diagnosis of suspected child abuse (a form of Meadow's syndrome).