Hairy cell leukaemia: evidence for the existence of a spectrum of functional characteristics.
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Biomedical subjects
Publications and source records attributed to D Variakojis.
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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.
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.
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.
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.
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.
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.
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Explore the source record for details and available documents.
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In patients with histologically proven Hodgkin's disease, knowledge of the extent of involvement of lymph nodes and other organs has proven valuable in the determination of treatment and prognosis. One of the most common sites of involvement outside the hematopoietic system has been shown to be the lung parenchyma; in this study, six patients with a tissue-proven diagnosis of Hogdkin's disease and positive cytologic findings in the sputum were reviewed. Three cell types not found in normal sputum specimens were identified in these patients and were correlated with the histologic patterns of the tumors as seen in lung biopsies. Our results suggest the usefulness of sputum examination as an adjunctive or possibly a substitute diagnostic procedure in the evaluation of patients with Hodgkin's disease and possible lung involvement. They also suggest that in some cases the cytologic diagnosis can be quite specific in the identification of neoplastic cells as consistent with a diagnosis of Hodgkin's disease.