The American journal of pediatric hematology/oncology最新文献

Purpose: After the harvesting of bone marrow for use in transplantation, pain control needs are greatest in the first 12 to 24 hours. This is also the time during which systemic side effects of parenterally administered narcotics are greatest as a result of the recent general anesthetic. We examined the role of regional anesthesia as an adjunct to pain management in pediatric patients undergoing bone marrow harvest.

Patients and methods: Ten pediatric bone marrow donors were treated with intraoperative caudal blocks using bupivacaine or a combination of bupivacaine and morphine.

Results: Donors who received regional anesthesia had a significant decrease in postoperative parenteral narcotic requirement and fewer systemic complications when compared with a control group of 10 age-matched children who did not receive the caudal block.

Conclusions: Regional anesthesia with caudal block can decrease the morbidity associated with bone marrow harvest in donors.

Purpose: Hepatitis C virus (HCV) transmission is a well-documented complication of blood transfusions, although data on transfused children with cancer is sparse. Using a newer assay for anti-HCV antibodies, the prevalence of HCV infection was determined in a population of children with cancer in the United States.

Patients and methods: Forty-five transfused children with cancer were studied for evidence of HCV infection. Patients had not received chemotherapy for a mean of 2.3 years or transfusions for a mean of 3.1 years before being evaluated. Levels of serum aminotransferases [aspartate aminotransferase and alanine aminotransferase (ALT)], hepatitis B surface antigen (HBsAg), hepatitis B surface antibody (HBsAb), and hepatitis B core antibody (HBcAb) were assessed. A second-generation enzyme immunoassay (EIA) was used to screen for anti-HCV antibodies. Positive EIAs were supplemented by a radioimmunoblot assay (RIBA-2).

Results: No patient tested positively for HBsAg, HBsAb, or HBcAb; four of 45 (8.9%) were positive for HCV antibodies by EIA. Three of the four (6.7% of the total) were also positive by RIBA-2 testing. The mean number of donor exposures was not significantly different between HCV-negative versus RIBA-2-positive patients (23.1 vs. 61.7, p = 0.16). ALT levels off therapy and peak ALT levels during therapy were significantly higher in the RIBA-2-positive group versus the HCV-negative group, although 36% of all patients (16 of 45) had at least one elevation in ALT greater than twice the upper limit of normal. All three RIBA-2-positive patients were transfused before institution of universal screening of blood donors for HCV in 1990 and had hepatomegaly noted at least once.

Conclusions: We have identified a small group of children who may be at high risk for developing chronic active hepatitis and cirrhosis. Testing for HCV should be a routine part of long-term follow-up in children treated for cancer.

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.

Patients and methods: A 3-year-old girl with acute lymphoblastic leukemia developed typhlitis immediately after diagnosis and before the institution of chemotherapy.

Purpose: Typhlitis is a necrotizing colitis that develops in immunodeficient patients with severe neutropenia. Most patients are leukemic children who are receiving or have received chemotherapy. Typhlitis in the absence of chemotherapy is rare. We report a fatal case of typhlitis in an untreated leukemic patient.

Results: This case emphasizes the multifactorial pathogenesis of typhlitis, which may occur in the absence of chemotherapy.

Conclusions: Typhlitis should always be considered as a potential cause of acute sepsis and abdominal pain in a leukemic patient.

Purpose: We describe a newborn baby boy with anemia in whom human parvovirus (HPV) antibodies were detected in his serum.

Patients and methods: Severe reticulocytopenia and anemia lasted until day 11 after his birth. Thereafter reticulocytosis was observed. Work-ups for immune hemolytic anemia, fetomaternal hemorrhage, placental hemorrhage, intrauterine infections were negative by clinical and laboratory analysis. HPV-specific IgM and IgG were positive on both the baby's and the maternal serum.

Results: Despite severe anemia, the baby remained clinically stable. His hemoglobin levels steadily increased along with ongoing reticulocytosis. He is now 14 months old with normal hemoglobin.

Conclusions: Detection of HPV antibodies indicated its causal relation to the development of congenital pure red cell aplasia. The possibility of HPV infection should be considered in the assessment of anemia of the newborn.

Purpose: Despite the excellent prognosis for 90% of patients with Wilms' tumor, survival remains poor among those with recurrent or advanced disease or tumors of unfavorable histology. We sought to identify a chemotherapy regimen for this subset of patients that offers potential efficacy with minimal nephrotoxicity.

Patients and methods: Through a review of patients' medical records, we compared the efficacy and nephrotoxicity of ifosfamide, cisplatin, cisplatin/etoposide, and ifosfamide/carboplatin/etoposide (ICE) regimens in 32 patients with recurrent (n = 23), refractory (n = 1), or metastatic (n = 8) Wilms' tumor, including six with tumors having unfavorable histologic features.

Results: Single-agent ifosfamide was minimally nephrotoxic and induced responses in three of 11 patients, but none have survived. Cisplatin with or without etoposide induced responses in six of 18 patients with recurrent Wilms' tumor (there is one long-term survivor). Seven of eight patients with newly diagnosed extensive metastatic disease responded to cisplatin/etoposide plus vincristine, dactinomycin, adriamycin, and radiotherapy. This regimen produced three long-term survivors, but was associated with significant nephrotoxicity. The ifosfamide, carboplatin, and etoposide regimen induced responses in four of five patients treated, and had minimal nephrotoxicity. Two remain free of disease progression 22 months after recurrence.

Conclusions: Although long-term survival remains to be determined, the ICE combination appears to be effective against recurrent Wilms' tumor without endangering the patients' single remaining kidney. Myelotoxicity can be ameliorated by administering growth factors. We suggest that ICE chemotherapy be considered for the primary treatment of high-risk patients with Wilms' tumor.

Purpose: Cancer in pregnancy is not uncommon, but cases of maternal malignancy involving the placenta or the fetus are much more rare. We report two additional cases.

Patients and methods: We report one case of malignant melanoma discovered during pregnancy and found to have metastasized to the placenta. We also describe a case of maternal medulloblastoma involving the placenta at delivery.

Conclusions: Although maternal malignancy during pregnancy occurs in as many as one in 1,000 pregnancies, involvement of the products of conception is rare. Subsequent malignancy in the fetus is even more rare. We report two cases, one of melanoma and another of medulloblastoma (the first such case described). Both infants are alive and well.

Purpose: We present a report of a child with subclinical immunodeficiency who became chronically infected with parvovirus resulting in pancytopenia and morphologic abnormalities in the bone marrow mimicking myelodysplastic syndrome (MDS).

Patients: An 8-year-old boy presented with severe anemia, moderate thrombocytopenia and granulocytopenia. The patient showed hyper-immunoglobulin M (IgM) immunodeficiency but no increased susceptibility to infections. The bone marrow was hypercellular with dysplastic granulocytopoiesis and erythroblastopenia.

Results: Treatment with cyclosporine and i.v. Ig resulted in temporary normalization of the hemoglobin level. For several years it was assumed that the patient had MDS. A diagnosis of parvovirus infection was initially rejected due to the lack of specific antibodies and the absence of giant pronormoblasts in the bone marrow. When the polymerase chain reaction technique became available, parvovirus DNA was detected from the entire disease course.

Conclusions: This case report expands our conception of the clinical spectrum of parvovirus infection and emphasizes that parvovirus must be considered as a differential diagnosis in MDS. We recommend performing a parvovirus DNA test despite negative serologic findings in patients with MDS, especially when associated with immunologic abnormalities.