Biomedical subjects
Publications and source records attributed to P Lanzkowsky.
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.
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.
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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.
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.
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.
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.
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.
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A group of 47 children with homozygous sickle cell disease ranging in age from 2 to 18 years was studied for the prevalence of gallstones. All of these patients had oral cholecystogram and cholecystosonogram. Eight of the 47 patients (17%) had gallstones both on oral cholecystography and on cholecystosonography. These eight patients had a history of recurrent abdominal pain usually localized to the right upper quadrant. All were admitted on several occasions for sickle cell abdominal crises and four of these were admitted for acute hepatic crisis. These patients have undergone elective cholecystectomy and gallstones were found in every patient. The patients have been followed up from seven to 17 months after cholecystectomy and none have had abdominal symptoms or required hospitalization for abdominal crises.
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Two children who presented initially with a lymphoid malignancy were noted to develop recurrences with myeloid features late in the course of their disease. In both cases, evidence of lymphoid differentiation was present in the myelogenous cells that were Ph1 chromosome negative. The first patient had acute myelogenous leukemia and developed a recurrence with morphologic features of acute myelogenous leukemia. Terminal transferase was present in the myelogenous blasts. The second patient initially had a diffuse lymphoblastic non-Hodgkin lymphoma. During the course of her illness she developed a myeloproliferative disorder characterized by basophilic meningitis, splenomegaly, and hypereosinophilia. Lymphocyte T-cell (E-rosette) markers were present on the eosinophils. These observations lend further support to the hypothesis of varying lymphoid and myeloid differentiation in certain cases of leukemia.
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