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Biomedical subjects

L Penchansky

Publications and source records attributed to L Penchansky.

At least 55 records · Page 3Linked to original sources

Heterogeneous acute leukemias.

We have reported two cases of Philadelphia-chromosome-negative acute leukemia with heterogeneous populations of leukemic cells and reviewed six cases from the literature. The cases were identified by several methods, including morphology, cytochemistry, enzymatic analysis, cytogenetic analysis, and electron microscopy. Predisposing factors were probably present in three cases and may partially account for the seemingly increasing incidence, as has been the experience with other secondary leukemias. The prognosis was variable, but most of the complete remissions were seen only when lymphocytic and nonlymphocytic antileukemic chemotherapeutic regimes were used. The occurrence of these unusual acute leukemias strongly supports the concept of a lymphoid-myeloid stem cell.

Acute Disease↗

Acute leukemia following a malignant teratoma in a child with Klinefelter's syndrome: case report and review of secondary leukemias in children following treatment of a primary neoplasm.

A case of Klinefelter's syndrome with the development of a mediastinal teratocarcinoma is reported suggesting that the association of a gonadotropin-secreting tumor with the XXY chromosomal abnormality may be more than coincidental. Whereas this child appeared to survive the effects of the teratocarcinoma, he succumbed to acute leukemia two years later. This prompted a review of secondary leukemias in children following chemotherapy/radiotherapy for another primary malignancy. These patients responded poorly to treatment of the secondary leukemia with a median survival of about four months. The incidence of secondary leukemias might be expected to be on the rise as increasing numbers of pediatric cancer patients are surviving longer after treatment with agents that are potentially leukemogenic or carcinogenic themselves. Children who have survived cancer and its therapy present special problems and it will be necessary for the pediatrician and practitioner to monitor these children.

Adolescent↗

Sex differences in prognosis of childhood T-cell leukemia.

Bone marrows of 41 untreated children and adolescents with acute lymphocytic leukemia were studied by combined immunologic and histochemical methods at the time of diagnosis. Eleven were classified as T-cell lymphoblastic leukemias (27%) on the basis of cytochemical stains and E-rosette assay. The patients in this group has low median age of 8 years, relatively low median WBC of 13.4 x 10(3)/cc, 6/11 were female, and only 2/5 males had a mediastinal mass. The girls had a lower median age than boys (7 vs 9 years), none had mediastinal masses or extramedullary involvement, and their survival was greater than 27 months compared to 14 months for the boys (P less than 0.01). All patients were enrolled and treated on the (then) currently active CCSG protocols for ALL. This study emphasizes the fact that not all patients with T-cell ALL have poor prognosis, that sex could be an important factor affecting survival, and that the difference in survival could not be adequately explained by differences in the initial WBC.

Adolescent↗

Philadelphia chromosome--positive pre-B-lymphocytic leukemia in a child.

A boy with acute lymphocytic leukemia (ALL) associated with a pre-B-cell phenotype and the Ph' chromosome is described. The Ph'-positive blasts with L3 morphology contained intracytoplasmic IgM but lacked surface immunoglobulins. The child's response to intensive chemotherapy was unsatisfactory. The short survival of our patient suggests that childhood leukemias expressing the pre-B-cell phenotype and Ph' chromosome may carry a poor prognosis. For that reason, the usefulness of cytogenetic and immunologic characterization of leukemic blasts at diagnosis is emphasized.

B-Lymphocytes↗

Hodgkin's disease in childhood: therapy results in Argentina.

From 1940 to 1977, a total of 152 children aged 15 years or less with the histologic diagnosis of Hodgkin's disease were treated in Argentina. For analysis, these patients were placed into three periods which displayed the most outstanding changes in diagnostic workup and therapy. The periods are as follows: (1) 1940 to 1966 (43 children), (2) 1967 to 1972 (35 children), and (3) 1973 to 1977 (74 children). The patients were treated with extended field radiation therapy and followed by courses of cyclophosphamide-vinblastine-procarbazine-prednisone, with CCNU added (CCVPP) or not added (CVPP) in a randomized trial. The mean age of the whole group was 7.5 years (range 2 to 15 years) and there was a predominance of males (79%). Crude 6-year survival for the three periods were as follows: 1940 to 1966, 34%; 1967 to 1972, 50%; and 1973 to 1977, 79%. We conclude that (1) survival of Hodgkin's disease of childhood has shown a rather marked improvement during the last decade and this progress is probably due to the use of combined multidrug chemotherapy administered under collaborative controlled clinical trials; (2) preliminary evaluation of the results of CVPP and CCVPP therapy shows that the latter combination (CCVPP) neither increases the percent of patients achieving complete remission nor prolongs relapse-survival in Hodgkin's disease of childhood: and (3) all stages of childhood Hodgkin's disease can be successfully managed with multidrug chemotherapy alone.

Adolescent↗

Rhabdomyosarcoma of the kidney in children.

Among 227 consecutive renal tumors examined from the Hospital de Niños de Buenos Aires, seven examples of a distinctive clinicopathological entity occurred. These renal neoplasms were all unilateral, grossly infiltrative without a definitive capsule. Microscopically, the lesion was characterized by a sarcomatous pattern with a rhabdomyoblastic differentiation. Four of them had regional lymph node involvement at surgery. Four developed bone metastases and none had lung metastases. In three instances, the bone metastases were histologically similar to the original neoplasm. The age of the patients ranged from 11 months to 5 1/2 years. A male predominance was observed. Two patients died; one is alive 5 years after surgery; three are alive with metastases; one has been followed for less than a year. In this study, it is proposed that another group of tumors be removed from the Wilms' tumor classification. The neoplasm has been described previously in the literature under several names. It is not clearly related clinicopathologically to nephroblastoma. The term rhabdomyosarcoma is proposed in view of its entirely different biological behavior.

Bone Neoplasms↗

Identification of cytomegalovirus in bone marrow biopsy.

The bone marrow trephine biopsy has become a common procedure for evaluating hematopoietic abnormalities. Occasionally, the first clue to an infectious process is obtained from this source. A bone marrow biopsy, done to evaluate pancytopenia in a patient who had received a renal transplant, revealed large intranuclear inclusions diagnostic of cytomegalovirus. Antibody titers were initially negative but rose over the next few weeks to confirm the diagnosis. Even though the bone marrow biopsy may not be the best site for finding CMV inclusions, the careful examination of a random bone marrow biopsy, particularly in an immunosuppressed individual, may contribute to the diagnosis.

Adolescent↗

Non-Hodgkin's lymphoma in children: an analysis of 122 cases from Argentina.

One hundred twenty two children with non-Hodgkin's lymphoma were studied from January 1966 to December 1975. The first group (1966-1972) did not receive an uniform treatment. The second group (1973-1975) entered in a G.A.T.L.A. protocol consisting of: vincristine-prednisone plus surgery and/or radiotherapy as induction treatment, craniocervical radiotherapy and intrathecal methotrexate as CNS preventive treatment and anti-leukemia (6-mercaptopurine, methotrexate and vincristine-prednisone pulses) or anti-lymphoma (COPP) treatment as maintenance, in a randomized trial. Comparison of survival of the two groups are as follows: series 1966-1972, 22% and 20% at 12 and 24 months of evolution, respectively, and series 1973-1975, 33% and 26% at 12 and 24 months, respectively. After 2 years of complete remission we have not seen any relapse. We conclude that 1) this disease is highly malignant and must be treated with more intensive chemotherapeutic treatment, and 2) there is no difference between antileukemia or anti-lymphoma maintenance treatment, as yet.

Adolescent↗

Cystic nephroma.

The clinical and pathologic findings of four cases of cystic nephroma (so-called "renal multilocular cyst") in four infants nephrectomized with the diagnosis of Wilms' tumor are presented. Each lesion consisted of an encapsulated mass composed of cysts separated by thin septa. Histologically the cysts were lined by epithelium, and the septa contained foci of immature metanephric blastema (abundant in one case and sparse in another). rhabdomyocytes and mature tubules. Neither embryonic ducts nor cartilage were seen. One cystic lesion was adjacent to a nephroblastoma which formed a separate nodule. No recurrences of metastases developed, and the only death was due to postoperative sepsis. These lesions are considered by some authors to be dysplasia, while others interpret them as benign neoplasms. Our findings and a review of the literature probably represent the differentiated counterpart of nephroblastoma.

Child, Preschool↗

Detection of neuroblastoma in the bone marrow: biopsy versus aspiration.

PURPOSE: Multiple studies have emphasized the higher yield of detection of metastatic neuroblastoma (MNb) by bone marrow biopsy (BMB) than by bone marrow aspiration (BMA). Because the need for BMA has been questioned, the yield of both procedures was investigated at diagnosis and during the course of disease. METHODS: For morphologic and immunohistochemical detection of MNb, 289 specimens obtained by BMA and BMB from 57 children with neuroblastoma were reviewed. RESULTS: In 34% of cases, MNb was present in both the aspirate and biopsy specimen. MNb was present in only the biopsy specimen in 8% and in only the aspirate in 6%. In 52%, neither BMA nor BMB detected MNb. In 15 of 18 cases in which MNb was present in the aspirate only, protein gene product 9.5 (PGP) stain was performed on the biopsy specimen. In one case, this helped to identify MNb that was not evident by routine hematoxylin and eosin stain. Of the 24 cases in which only the BMB was positive, 3 were identified only by means of PGP stain. CONCLUSIONS: Even with the additional use of immunohistochemistry, both BMA and BMB should be performed to have the highest yield of detection of MNb in bone marrow.

Azure Stains↗

Leukemia in a child with a history of medulloblastoma.

Leukemia of mixed lineage, was diagnosed in a 6.5-year-old boy with a history of medulloblastoma, 38 months after his initial cancer diagnosis. Therapy had included craniospinal radiation and nitrosourea-based chemotherapy. In addition, onset of leukemia was preceded by therapy with recombinant growth hormone for short stature. Although rare, leukemia is a treatment-related complication for patients with past brain tumors whose follow-up should therefore include surveillance with complete blood counts.

Brain Neoplasms↗

Idiopathic hypereosinophilic syndrome terminating in acute lymphoblastic leukemia.

Idiopathic hypereosinophilic syndrome (IHES) is a heterogeneous group of disorders characterized by multisystem dysfunction and persistent, extreme eosinophilia of unknown cause. We describe a 9-1/2-year-old boy whose course included several unusual clinical features and terminated 2 years after diagnosis in acute lymphoblastic leukemia (ALL). Serial studies suggest that leukemia was not present earlier in his course. We speculate that this child may have had an evolving lymphoproliferative syndrome with a terminal blast crisis to which the eosinophilia was a nonmalignant leukemoid reaction.

Antigens, Differentiation↗

Myeloproliferative syndrome in childhood.

This report describes an atypical "myeloproliferative disorder" in a child that evolved over a 6-year period. The clinical and laboratory features resembled the adult type of chronic myelogenous leukemia, but a Philadelphia chromosome was lacking. Although the proper classification of this disorder remains uncertain, the case makes a good point for the use of diagnostic procedures such as the bone marrow core biopsy and cytogenetic studies to help unravel and define the characteristics of these unusual "myeloproliferative" disorders.

Bone Marrow Cells↗