Hematologic emergencies.
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Biomedical subjects
Publications and source records attributed to P Lanzkowsky.
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Zinc status was studied in 47 children with homozygous sickle cell disease (SCD). Decreased hair and plasma zinc was demonstrated in these children and hyperzincuria was found in the older patients. This study indicates that zinc deficiency in patients with SCD is probably due to hyperzincuria.
Seventeen children with sickle cell anemia received 40 partial exchange transfusions for serious complications of sickle cell anemia, and preoperatively to reduce the risk of anesthesia. Each patient received two partial exchange transfusions at 24-hour intervals and all patients tolerated the exchange transfusions well. The mean hematocrit level rose from 22.9% before the exchange transfusion to 40.6% at the end of the second exchange. The mean sickle hemoglobin level decreased from 93.8% before the exchange to 28.0% after the second exchange. Patients with acute lung syndrome had remarkable improvement in clinical symptoms and PaO2 levels, and those with acute liver crisis had substantial reduction in serum bilirubin levels. All the other patients showed marked clinical improvement following the exchange transfusions.
Two hundred and twenty-seven children with recurrent acute lymphoblastic leukemia were treated with various combinations of vincristine, prednisone, cyclophosphamide and L-asparaginase in an approach to the induction of remission. The combination of L-asparaginase 1,000 mu/kg iv q.d. x 10, vincristine 2.0 mg/m2iv q.w. x 4 and prednisone 40 mg/m2 p.o.q.d. x 28 days was found to be highly effective. The incidence of remission was 73%. No significant improvement was achieved when cyclophosphamide was added to this regimen. Various combinations of cytosine arabinoside, cyclophosphamide, vincristine, prednisone, BCNU or CCNU failed to maintain remission duration for more than two or three months. Neither BCNU nor CCNU prevented the development of CNS leukemia.
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Pulmonary blastoma is a rare tumor of the lung. Although it has been stated that this is predominantly a tumor of adults, a review of the 42 cases published to date reveals that it is a disease which occurs with some frequency in children. The tumor is relatively benign compared to pulmonary carcinosarcoma. Of the eight cases previously described in children, three are known to have survived and to be free of disease 10 months, 3 and 8 years after surgical resection. We report two additional cases of pulmonary blastoma in children followed for 34 and 26 months without any evidence of recurrence or metastases.
We evaluated hematological findings, with special reference to the monocyte count, in ten infants with congenital syphilis. One patient had a monocytic leukemoid reaction and eight had absolute monocyte counts considerably higher than normal control infants. Monocytes appear to be an essential participant in the cellular reaction to treponema pallidum. To our knowledge, this is the first report of a monocytic leukemoid reaction in congenital syphilis.
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The association of pleural effusion and Wilms' tumor is uncommon. We report three patients who developed pleural effusion as a result of different mechanisms, all of which were related to Wilms' tumor or to its treatment.
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Torsion of the pedicle of a visceroptosed spleen, a rare condition, was diagnosed preoperatively in a 4-year-old girl, with the aid of history, physical examination, blood smear findings, splenic scans with technetium Tc 99m sulfur colloid, and selective angiography. A splenectomy was performed, and the child made an uneventful recovery. This case report illustrates some of the diagnostic and therapeutic considerations pertaining to torsion of the spleen.
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Twenty-five cases of hodgkin's Disease (15 males and 10 females) aged 5 to 17 years were studied from April 1970 to July 1976 (75 month period). Histology revealed that 2 had lymphocytic predominance, 12 had nodular sclerosis, and 11 had mixed cellularity. Pathologic staging revealed that 3 were IA, 1 IB, 5 IIA, 4IIB, 6IIIA, and 6 IIIB. Laparotomy altered the staging in 12 patients (9 were staging up and 3 down). All but 2 patients received extended field radiation, and 5 had recurrence of disease and were treated with combination chemotherapy. Twenty-three are alive without evidence of disease (21-75 months), and the 2 deaths were not due to Hodgkin's Disease but to hemobilia (postliver biopsy) and penumococcal septicemia, purpura fulminans, and disseminated intravascular coagulation (14 months postsplenectomy). Other complications included 2 patients with intestinal obstruction, 1 with postoperative subphrenic abscess, and 1 with streptococcal septicemia and polyarthritis. Nineteen patients received continuous penicillin prophylaxis postoperatively and the 2 with serious infections were amongst the 6 who had not received penicillin or whose penicillin had been discontinued at the time of infection. It is concluded that laparotomy and splenectomy in children is essential for accurate staging but carries significant risk, and continuous penicillin prophylaxis is recommended.
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