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Biomedical subjects

F G Behm

Publications and source records attributed to F G Behm.

At least 163 records · Page 9Linked to original sources
PubMed1987

Hypodiploidy is associated with a poor prognosis in childhood acute lymphoblastic leukemia.

Leukemic cells from 31 (7.6%) of 409 children with newly diagnosed acute lymphoblastic leukemia (ALL) had a hypodiploid karyotype. The patients' ages ranged from 0.8 to 17 years (median, 5 years) and their initial leukocyte counts from 1.0 to 132 X 10(9)/L (median, 12.7 X 10(9)/L). Modal chromosome numbers for the leukemic stem lines were 45 in 26 cases, 28 in two cases, and 26, 36 and 43 in one case each. Seven cases had one to three additional abnormal lines due to clonal evolution. Chromosome 20 was lost most frequently (nine cases). Structural abnormalities--including chromosomal translocations (21 cases), deletions (ten cases), duplications (two cases), or inversions (one case)--were common findings; the nonrandom translocations consisted of the t(1;19)(q23;p13.3) in two pre-B cases and tdic(9;12)(p1?1;p1?2) in three cases of common ALL. When compared with hyperdiploid cases (greater than 50 chromosomes), ALL with hypodiploidy was found to have a poorer outcome and was more likely to be associated with chromosomal translocations, higher serum lactic dehydrogenase levels, and age less than 2 or greater than or equal to 10 years. Moreover, patients with hypodiploid ALL fared as poorly as those with pseudodiploid karyotypes, even though their leukocyte counts and serum lactic dehydrogenase levels were lower and they had a comparable frequency of leukemic cell translocations. Hypodiploidy is therefore an unfavorable karyotypic feature in childhood ALL.

Acute Disease↗
PubMed1987

7q32-q36 translocations in childhood T cell leukemia: cytogenetic evidence for involvement of the T cell receptor beta-chain gene.

Blast cell chromosomal rearrangements involving the long arm of chromosome 7 were identified in eight of 197 cases of childhood acute lymphoblastic leukemia (ALL). Breakpoints were variable but tended to cluster in either the proximal or the terminal 7q region, depending on the immunophenotype of the cells. The 7q32-q36 region, the locus of the T cell receptor beta-chain gene, was the site of breakpoints in four of 31 cases of T cell ALL but was not involved in any of the 166 cases originating from B cell precursors (P less than .0004). In three of the four T cell cases it was possible to identify the chromosomal segment that had been translocated to the 7q32-q36 region: 1p32, 2p21, and 6p21. The 1p32 and 6p21 bands are particularly interesting, as they contain the sites of two known protooncogenes, c-L-myc and hpim, respectively. Our findings suggest that the locus of the beta-chain gene of the T cell receptor is a preferential site for certain chromosomal rearrangements in leukemic T lymphoblasts, analogous to the T cell receptor alpha-chain gene on human chromosome 14. Translocation of proto-oncogenes to a site near the beta-chain regulatory sequences provides a potential mechanism for oncogene activation.

Adolescent↗
PubMed1987

tdic(9;12): a nonrandom chromosome abnormality in childhood B-cell precursor acute lymphoblastic leukemia: a Pediatric Oncology Group Study.

In a review of 432 children with newly diagnosed acute lymphoblastic leukemia (ALL), we identified a new nonrandom translocation, tdic(9;12)(p1?1;p1?2), in the leukemic marrow cells of eight patients. Seven had hypodiploid karyotypes that lacked chromosomes 9 and 12 and contained a der(12), tdic(9;12); the eighth had a pseudodiploid karyotype with two normal 9 chromosomes, one normal 12 and the der(12), tdic(9;12). Abnormalities involving chromosomes other than 9 and 12 were noted in four of the eight patients. All cells with the tdic(9;12) expressed both the common ALL antigen and HLA-DR. Cytoplasmic immunoglobulin, a marker of pre-B ALL, was detected in one case with the tdic(9;12) but was absent in the other seven. Our results suggest that the tdic(9;12)(p1?1;p1?2) rearrangement is specifically associated with leukemic B cell precursors.

B-Lymphocytes↗
PubMed1986

Somatic rearrangement of the c-myc oncogene in primary human diffuse large-cell lymphoma.

Chromosome translocations involving 8q24, the band to which c-myc has been mapped (Dalla-Favera et al., 1982), are a uniform finding in Burkitt's lymphoma (Bernheim et al., 1981). However, in only a minority of the tumors is the rearrangement of the c-myc locus sufficiently close to the gene to be detected with currently available probes (Dalla-Favera et al., 1983). Approximately 25% of diffuse large-cell lymphomas have also been reported to have translocations involving 8q24 (Mitelman, 1985), but there have been no reports of c-myc rearrangements in this form of non-Hodgkin's lymphoma. We have examined the structure of the c-myc locus in primary tumor tissue of 10 cases of diffuse large-cell lymphoma. In one patient, Southern blot analysis revealed additional c-myc fragments in the tumor DNA but not in the germ-line DNA. Southern blot analysis using probes from both the heavy- and light-chain immunoglobin loci showed that the myc rearrangement was unlikely to involve the immunoglobulin loci in this patient.

Chromosome Aberrations↗
PubMed1986

Unfavorable presenting clinical and laboratory features are associated with CALLA-negative non-T, non-B lymphoblastic leukemia in children.

Twenty-four (5.7%) of 424 children with newly diagnosed acute lymphoblastic leukemia (ALL) were found to have blast cells that expressed HLA-DR antigens but not the common ALL antigen (CALLA), E-rosette receptors, T-cell antigens, or cytoplasmic or surface immunoglobulins. Each of the eight cases tested expressed the B-cell associated antigen B4, but not B1 or B2 antigen. Myeloid-associated antigens were not present in any of the 10 cases tested. By comparison with common (CALLA+ B-cell precursor) ALL, patients having this immunophenotype were more likely to be children less than 2 yr of age (p less than 0.001), to have higher initial leukocyte counts (p less than 0.001), and to have blast cells with a DNA index less than 1.16 (p = 0.05), a pseudodiploid karyotype (p = 0.01) and a chromosomal translocation (p = 0.003). The presence of any chromosomal translocation in these CALLA- ALL was related to measures of increased leukemic cell burden including higher leukocyte counts, larger liver and spleen sizes and higher serum lactic dehydrogenase levels. While the patients were entered into several treatment arms of two protocols, the CALLA- cases appeared to have lower remission rate (p = 0.06) and shorter event-free survival time (p = 0.05) than did those with common ALL. The association with clinical and laboratory features of known adverse prognostic significance provides some explanation for the poor treatment outcome of CALLA- ALL.

Antigens, Neoplasm↗
PubMed1986

Hodgkin's disease: diagnosis by fine-needle aspiration biopsy. Analysis of cytologic criteria from a selected series.

Two hundred thirteen cases in which a lymph node aspirate and subsequent surgical biopsy had been performed were studied independently by four observers. Each observer attempted to discriminate involvement by Hodgkin's disease from involvement by other processes. The material included 18 cases of histologically confirmed Hodgkin's disease. The remaining 195 cases included a wide range of pathologic processes. All four observers were able to distinguish Hodgkin's disease from other processes in the great majority of cases. Two observers reviewed the aspirate material from the 18 cases of Hodgkin's disease to determine the presence and significance of elements known to characterize this disease, i.e., multinuclear and mononuclear Reed-Sternberg cells, polyploidal cells, granulomatous elements, metachromatic material, necrosis, eosinophils, neutrophils, and plasma cells. Reed-Sternberg cells were present in most but not all cases. Reed-Sternberg-like cells also were occasionally encountered in other processes. Polyploidal cells were invariably present and were useful in recognizing the disease. Granulomatous elements, metachromatic material, necrosis, eosinophils, and neutrophils were frequently present and, while not specific for the process, helped to draw attention to or substantiate the diagnosis of Hodgkin's disease.

Biopsy, Needle↗
PubMed1986

Shifts in blast cell phenotype and karyotype at relapse of childhood lymphoblastic leukemia.

Analyses of bone marrow blast cells collected at diagnosis and relapse from 68 children with acute lymphoblastic leukemia (ALL) demonstrated changes in the expression of cell markers in one-fourth of the patients. Loss of the common ALL antigen (CALLA) was a frequent change, occurring in 8 of the 51 cases initially classified as common or pre-B ALL. The HLA-DR antigen was either acquired or lost in 5 of the 68 cases, terminal deoxynucleotidyl transferase was lost in 6 of 25 cases, and reactivity of the T10 antigen with monoclonal antibodies was increased in 6 of 17 cases of non-T cell ALL. Conversion to acute nonlymphoblastic leukemia, so-called lineage switch, was noted in two cases of common ALL and one of pre-B ALL, coinciding with the loss of CALLA. Results of chromosomal analyses in cases with a loss of CALLA implicated several mechanisms in the observed phenotypic changes. In six cases, including each instance of lineage switch, the original karyotype had been replaced by an entirely different abnormal karyotype, suggesting clonal selection or induction of a second malignancy. In another case, the evidence suggested clonal evolution. Our findings demonstrate that sequential phenotypic and cytogenetic studies may yield valuable insights into the mechanisms of leukemic recurrence and may have implications for treatment selection.

Adolescent↗
PubMed1986

Malignant histiocytosis presenting with vasculitis and cutaneous erythrophagocytosis.

We report a case of malignant histiocytosis that presented initially as a cutaneous vasculitis without malignant features. A subsequent biopsy specimen of an erythematous skin lesion showed erythrophagocytosis by normal-appearing histiocytes within the subcutis. The recognition of erythrophagocytosis was followed by systemic symptoms and signs of malignant histiocytosis and a rapidly deteriorating clinical course. Possible mechanisms for erythrophagocytosis are discussed, and other diseases associated with this histologic finding are reviewed briefly.

Adult↗
PubMed1985

Some long-term effects of exchange transfusion with fluorocarbon emulsions in macaque monkeys.

Fluorocarbon emulsions were used as blood substitutes in exchange transfusions of seven macaque monkeys. Some monkeys were tested for electrophoretic abnormalities of serum proteins prior to and at the time of death. At the time of killing 2 1/2 to eight years after transfusion, autopsies were performed on all seven monkeys. Monkeys receiving emulsions of FC-47 (perfluorotributylamine) displayed vacuolated cells representing fluorocarbon throughout the reticuloendothelial system even six years later. Monkeys examined eight years after receiving emulsions of PP-5 (perfluorodecalin) failed to display such changes. However, electrophoresis revealed abnormalities in the blood of all four monkeys in which the test was performed, irrespective of whether FC-47 or PP-5 was given. The electrophoretic abnormality consisted of a polyclonal increase of immunoglobulins, some of which migrated in the beta region. These data suggest that fluorocarbon emulsions that have recently been given to humans may produce a chronic stimulation of the reticuloendothelial system leading to alterations in production of immunoglobulins.

Animals↗
PubMed1985

Malignant lymphoma with primary presentation in the spleen. A study of 20 patients.

We describe 20 adult patients with malignant lymphoma with primary presentation in the spleen. The most common presenting symptoms were fever, malaise, and weight loss. Physical examination revealed prominent splenomegaly without palpable lymphadenopathy. Small lymphocytic lymphoma was the most frequent histologic type (11/20), followed by large cell lymphoma and mixed cell lymphoma (3/20 each). Bone marrow involvement was found in ten of 17 patients. At laparotomy, lymph node involvement, usually retroperitoneal, was found in six of 13 patients. There was liver involvement in seven of 15 patients. Follow-up has been relatively short, with an average of 24 months (range, one to 48 months). Four patients died as a result of progressive disease, one died of sepsis after splenectomy, and one died two years after diagnosis of a stroke. The prognosis in primary splenic lymphoma appears to be similar to that in nodal lymphoma.

Adult↗
PubMed1984

Bone marrow examination in small cell carcinoma of the lung. Comparison of trephine biopsy with aspiration.

Bone marrow examinations in 146 patients with small cell carcinoma of the lung were reviewed. The results of trephine biopsy and aspiration were compared in 128 patients in whom both techniques were performed. Twenty-seven of the 128 examinations (21.2%) showed positive aspirates and 30 biopsies (23.4%) revealed metastatic disease. The overall agreement between the aspirates and biopsies was 96.1%. Thirty-one patients (24.2%) had metastatic tumor in either aspirate or biopsy: 4 of these (12.9%) had a positive biopsy with negative aspirate, and 1 (3.2%) showed a positive aspirate and negative biopsy. The Jamshidi needle was used, yielding an average of 4.1 cm of core material per biopsy. The authors conclude that bone marrow biopsy using the Jamshidi needle is equivalent to aspiration. Although both procedures are complementary, a satisfactory specimen obtained with the Jamshidi needle alone may yield nearly equal detection to the combined procedures. Some guidelines for obtaining an optimal biopsy specimen are suggested.

Biopsy↗
PubMed1984

Fine-needle aspiration effects on benign lymph node histology.

The histology of 28 lymph nodes with benign hyperplasia were assessed for evidence of the effects of fine-needle aspiration prior to biopsy. Only 43% of the lymph nodes showed evidence of prior aspiration. This consisted of needle tracts occupying less than 5% of any one section in ten cases, and 10% in two cases. In none of the 28 lymph nodes did prior fine-needle aspiration interfere with the histologic evaluation. The authors conclude that fine-needle aspiration does not interfere with subsequent histologic evaluation of lymph-adenopathy.

Biopsy, Needle↗
PubMed1982

Ascending herpetic endometritis.

Two cases of herpetic endometritis are reported. In both patients, the disease developed from documented herpetic cervicitis via transcervical ascension. One patient recovered spontaneously. Generalization and death occurred in the other patient, a renal transplant recipient treated with immunosuppressive agents. The histologic features of herpetic endometritis are illustrated. Intranuclear inclusions and ground-glass nuclei were found in endometrial glandular and stromal cells. Focal necrosis was prominent. Herpetic virus particles were demonstrated by electron microscopy in both cases. Ascending herpetic endometritis is a rare complication of a common disease. In patients with deficiencies of the immune system, herpetic endometritis may represent the initial step toward dissemination and warrants particular attention.

Adult↗
PubMed1981

Febrile neutrophilic dermatosis associated with acute leukemia.

Febrile neutrophilic dermatosis (Sweet's Syndrome) is an uncommon syndrome characterized clinically by fever, neutrophilia, and erythematous, non-ulcerating papules or plaques on the face, neck, upper thorax, and extremities. A case associated with acute myelogenous leukemia is reported and nine other reported cases associated with acute non-lymphocytic leukemia are reviewed. The appearance and location of the lesions on the face, neck, upper trunk and extremities, a female predominance, an associated fever, marked dermal infiltrates of mature neutrophils, and rapid clearing of lesions with parenteral corticosteroids were manifestations similar to febrile neutrophilic dermatosis not associated with leukemia. Although the etiology of febrile neutrophilic dermatosis associated with acute leukemia is unknown, the resolution of lesions and symptoms with systemic corticosteroids and recurrence with relapse of leukemia suggests an immune process with the leukemic cell population serving as a possible antigenic source.

Aged↗
PubMedDate not supplied

Acute lymphoblastic leukemia in a developing country: preliminary results of a nonrandomized clinical trial in El Salvador.

PURPOSE: To improve outcome and study biology of childhood acute lymphoblastic leukemia (ALL) in El Salvador. PATIENTS AND METHODS: Between January 1994 and December 1996, 153 children of El Salvador had newly diagnosed ALL treated in a collaborative program between Hospital Benjamin Bloom and St. Jude Children's Research Hospital (SJCRH). Therapy was based on a modified SJCRH protocol, with uniform remission induction (prednisone, vincristine, L-asparaginase) followed-up by consolidation with teniposide/cytarabine and/or high-dose methotrexate. Continuation treatment was risk-stratified: 123 patients assigned to the high-risk group received weekly rotational drug pairs, and 16 assigned to the standard-risk group received daily 6-mercaptopurine, weekly methotrexate, and monthly pulses of vincristine plus dexamethasone. High risk was defined as: DNA index < 1.16, age 12 months or younger, white blood cell count > or = 50 x 10(9)/L, T-cell immunophenotype, anterior mediastinal mass, central nervous system leukemia at diagnosis, or t(4;11), t(1;19), or t(9;22). Duration of the continuation treatment was 2.5 years in both groups. The median age at diagnosis of all patients was 4.8 (range I d-17 yrs), median leukocyte count was 15 (range 1-766) x 10(9)/L, and sex distribution was equal. RESULTS: Immunophenotypes were early beta-progenitor in 79%, T-cell in 3.9%, and inconclusive in 17% of cases. DNA index was <1.16 in 80.5% and was > or = 1.16 in 19.5% of the 123 known cases. For the analyzes, patients who refused therapy (abandoned treatment) were considered to have treatment failure as of their last follow-up dates. Complete remission was achieved in 126 of 151 (82.4%) patients (11 abandoned therapy during induction). The overall 4-year event-free survival (EFS) rate +/- 1 standard error was 48 +/- 6%. The 4-year EFS rates in patients at high-risk and standard-risk were 46 +/- 7% (n = 121) and 69 +/- 15% (n = 16), respectively (P = 0.20). When patients who refused further treatment are censored, the corresponding 4-year estimates of EFS are 51 +/- 8% and 75 +/- 14%, respectively. CONCLUSIONS: These results suggest that the biology of childhood ALL in El Salvador appears to be similar to that seen in the United States. Risk-directed chemotherapy can successfully be used in developing countries, but risk factors must be carefully determined and applied.

Adolescent↗
PubMedDate not supplied

Fine needle aspiration cytology of benign lymph node hyperplasias. Diagnostic significance of lymphohistiocytic aggregates.

The fine needle aspiration cytomorphology and the number of lymphohistiocytic aggregates were correlated with five categories of benign lymph node histologies. Of 187 patients who had benign lymph node aspiration diagnoses made from 1975 to 1982 at the Medical College of Virginia, 26 had a subsequent lymph node biopsy. Excluded from the study were four cases with the biopsy site different from the aspiration site, three cases with evidence of metastatic carcinoma and one case of fistula at the site of biopsy. Of the remaining 18 cases, 13 (72%) had lymphohistiocytic aggregates on the aspiration slides. The mean number of lymphohistiocytic aggregates on the aspiration slides was greater in cases with histologic evidence of follicular hyperplasia (6.1) than in those with some other predominating benign histology (1.6).

Biopsy, Needle↗
PubMedDate not supplied

Preleukemic granulocytic sarcoma of cervix and vagina: initial manifestation by cytology.

Granulocytic sarcoma is an unusual form of tumefaction caused by acute granulocytic leukemia. On rare occasions, the lesion precedes the leukemic phase and presents as a mass with a normal peripheral white cell count. This report describes the initial manifestation of granulocytic sarcoma by vaginal cytology in a 39-year-old female with Down's syndrome. Six days after admission, the patient died of acute peritonitis following spontaneous perforation of the bowel. Autopsy revealed involvement of cervix, vagina, bowel wall and one pelvic lymph node by granulocytic sarcoma. Bone marrow examination confirmed the preleukemic stage of the disease. Cytologically, the malignant cells occurred singly. No nucleoli were seen. The differential diagnosis between malignant lymphoma and granulocytic sarcoma rests upon a positive naphtol AS-D chloroacetate esterase stain in granulocytic sarcoma. This stain may be performed on paraffin-embedded sections or on smears.

Adult↗