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Biomedical subjects

D Variakojis

Publications and source records attributed to D Variakojis.

51 records · Page 3Linked to original sources

Combined therapy for patients with mycosis fungoides.

In a comprehensive mycosis fungoides program, 60 patients have been seen with a pathologic diagnosis of this disease. Forty-four patients with advanced disease were referred for radiation therapy. Three treatment techniques were identified in which 14 patients were treated with localized fields using electrons or whole-body electron-beam therapy with doses of less than 3000 rads, 21 patients were treated using the Stanford technique with tissue doses of between 3000 and 4000 rads, and nine patients were treated with six cycles of mechlorethamine, vincristine, prednisone, and procarbazine or cyclophosphamide, vincristine, prednisone, and procarbazine following the electron-beam therapy. The actuarial survival rate was 45% at 1 year for the 14 patients with localized electron-beam therapy, whereas the actuarial survival rates were 83% for patients treated with whole-body electron-beam therapy and 100% for patients treated with whole-body electron-beam therapy followed by four-drug chemotherapy. The recurrence-free interval for these three groups correlates with these observations. A central nervous system recurrence has been observed in the combined-therapy group.

Adult

B cell acute lymphoblastic leukemia (ALL) with a 14q+ chromosome abnormality.

An adult patient with acute lymphoblastic leukemia associated with a 14q+ marker chromosome is presented. The abnormality resulted from a translocation of material from the long arm of chromosome 11. The leukemic cells were found to be B cells on the basis of surface immunoglobulins, lack of receptors for sheep erythrocytes, and a characteristically low level of adenosine deaminase activity. In other patients with ALL studied by us or reported by others in whom chromosome banding was done, a 14q+ chromosome was present in only one instance, also a case of B cell ALL. These two cases are the only examples of B cell ALL studied with chromosome banding reported to date. The frequent occurrence of a 14q+ chromosome in other malignant lymphoproliferative diseases of B cell origin suggests that a general association may exist between the 14q+ abnormality and B cell neoplasms. Cytogenetic analysis may therefore be useful in defining subtypes of ALL and in relating specific chromosomal abnormalities to lymphoproliferative disorders.

Adenosine Deaminase

Banding studies of chromosomal abnormalities in patients with acute lymphocytic leukemia.

Karyotypes were analyzed by routine Giemsa and quinacrine fluorescence for 16 patients with acute lymphocytic leukemia [ten adults (18 to 51 years) and six children (3 to 15 years)]. Four patients had received previous therapy, but all 16 had active disease when they were first studied. Eight patients (five untreated) had a normal karyotype initially; however, three of these developed a chromosomal abnormality during relapse. Eight patients had a chromosomal abnormality in their initial samples. Each of the 11 patients had different abnormalities. All chromosomes except Nos. 3, 5, 15, 16, and Y were involved in the various aneuploidies. One patient had a Ph1 chromosome due to a translocation with No. 21: t(21;22)(q22;q11). A patient with B-cell acute lymphocytic leukemia had a 14q+ marker in addition to other abnormalities. The median survival of patients with initially normal karyotypes may be longer than that of patients whose karyotypes are abnormal initially.

Acute Disease

Banding studies of chromosomes in a patient with mycosis fungoides.

Chromosomes from a patient with mycosis fungoides were examined in detail with banding techniques. Hyperdiploid cells from a lymph node had common anomalies of certain chromosomes which formed three similar clones. The abnormalities involved chromosomes No. 1, 2, 5, 8, 9, 10, 14, and 18, in addition to an unkwown small metacentric marker (M3). Although there were a number of mitotic cells in peripheral blood cultured both with and without PHA, none of the few cells with abnormal karyotypes was similar to the clonal cells of the lymph node. One of the abnormalities in the lymph node was a 14q rearrangement, which could be the result of a translocation of Nos. 8 and 14 involving a third chromosome, No. 2. An abnormality in the blood resulted from a translocation between the long arms of Nos. 1 and 14. These findings could be useful for studies in which mycosis fungoides is compared with the Sézary syndrome and other lymphoid malignancies.

Bone Marrow Examination

Multicentric giant lymph node hyperplasia.

A patient with retroperitoneal and axillary lymphadenopathy and splenomegaly was demonstrated histologically to have the hyaline vascular type of giant lymph node hyperplasia, with plasma cell infiltrates in each region. The abdominal lesions were not surgically resectable and did not respond to radiotherapy. The clinical findings included polyclonal gammopathy, high cold agglutinin titers, neuropathy, and bilateral papilledema. All of these abnormalities have persisted three years since the initial diagnosis.

Female

Banding studies on chromosomes in diffuse "histiocytic" lymphomas: correlation of 14q+ marker chromosome with cytology.

Chromosomes were studied in cells from tissues primarily involved by diffuse "histiocytic" lymphoma in nine patients. Two of the patients had stage II disease; their tumors were fibrotic and had no mitotic cells. One patient was in stage III, and the remaining six patients had stage IV disease. The modal chromosome number of abnormal cells from these last seven patients was hypodiploid in two, hyperdiploid in four, and near-triploid in one. Complete banding studies of six cases and partial analysis of the seventh indicate that (1) every patient had a distinct cell line with common markers, with a few cells showing minor variants; (2) although certain chromosomes (Nos. 1, 2, 3, 9, 12, and 14) were structurally affected more often than others, no markers with the same banding pattern were noted among them; and (3) the cytologic type of lymphoma could be correlated with the karyotype in all seven patients. When the Lukes and Collins classification was used, three patients whose tumors were composed predominantly of large noncleaved cells showed a 14q translocation leading to the formation of a 14q+ marker chromosome. This marker was not observed in four patients whose tumors had a majority of large cleaved cells. These preliminary results, if confirmed in a larger series of patients, will provide additional evidence that there are consistent chromosome changes associated with specific subtypes of lymphoproliferative disorders analogous to the Ph1 chromosome in chronic myelogenous leukemia.

Chromosome Aberrations

Survival of patients with localized histiocytic lymphoma.

Twenty of 65 patients with diffuse histiocytic lymphoma were identified by staging laparotomy as being in pathologic stages (PS) I, I(E), II, II(E). Six of the 20 patients were treated with total nodal, 10 with extended mantle, and four with involved-field radiotherapy. The survival rate and relapse-free survival at five years were 71% and 78%, respectively. All relapses occurred within the first year and were confined to patients with PS II disease and four or more sites of involvement. Accurate pathologic staging identifies patients who are potentially curable with radiotherapy. Further studies are required to determine the treatment necessary to achieve cure in PS II patients with more than four sites of involvement.

Adolescent

Bronchial web diagnosed by bronchography.

Bronchial webs are rare lesions which often go unrecognized. The authors describe a patient who presented with right-lower-lobe bronchiectasis and at-electasis secondary to a web in the right-lower-lobe bronchus. This is the first known instance where such a lesion has been demonstrated by bronchography. The etiology of this and similar lesions reported in the literature remains obscure.

Bronchi

Papillary clear cell carcinoma of the thyroid gland.

A case of an unusual papillary clear cell carcinoma of the thyroid gland is described. The patient expired 17 days after operative biopsy and thyroxine suppression. Special stains were helpful in differentiating renal cortical carcinoma and parathyroid malignant disease from primary papillary clear cell carcinoma of the thyroid. Ultrastructural features of this tumor may relate to the effects of thyroid stimulating hormone as well as the malignant nature of the tumor.

Adenocarcinoma

The Sézary syndrome lymphoid cell: abnormal surface properties and mitogen responsiveness.

The peripheral blood lymphoid cells of five patients with Sézary syndrome (SS) were examined with respect to their surface membrane characteristics and their response to mitogens. These cells showed markedly defective mitogenic responses to a broad dose range of phytohaemagglutinin (PHA), pokeweed mitogen, concanavalin A, and a rabbit antihuman lymphocyte antiserum (ATS), when compared with normal human lymphocytes. SS lymphoid cells (three patients studied) also displayed diminished or nearly absent capacity to form rosettes with unsensitized sheep erythrocytes (E-rosettes), and lacked surface immunoglobulin determinants. Despite their poor mitogenic response to ATS, they were as susceptible as normal lymphocytes to ATS-induced, complement mediated cytotoxicity. By comparison with lymphocytes from patients with chronic lymphocytic leukaemia, however, SS lymphoid cells showed decreased susceptibility to leukoagglutination by PHA. By way of contrast, three patients with mycosis fungoides having normal-appearing peripheral blood lymphocytes showed normal lymphocyte responses to mitogens, as well as normal proportions of E-rosette forming and surface immunoglobulin-bearing lymphocytes. These studies demonstrate that the SS lymphoid cell may, in some cases, lack surface properties and mitogen response characteristics of both B- and T-lymphocytes.

Adult

The Sézary syndrome cell: surface ultrastructural characteristics.

Peripheral blood mononuclear cells from a patient with Sézary syndrome which lacked E-rosette-forming ability and surface immunoglobulins, and which displayed a markedly depressed response to a variety of mitogens, were studied by scanning electron microscopy (SEM) and transmission electron microscopy (TEM) on 3 occasions. The first peripheral blood sample (smooth) differed significantly from two later samples (moderate numbers of microvilli) when surface characteristics were examined by SEM; these differences were confirmed by TEM. The Sézary syndrome cells in this patient may be related to a T lymphocyte which has lost certain surface markers and mitogen response characteristics through a process of de-differentiation.

Cell Membrane