Biomedical subjects
Publications and source records attributed to K Konrad.
We present two patients with lymphomatoid granulomatosis (LG) which was diagnosed from the histopathology of cutaneous lesions. Combined cytostatic and corticosteroid treatment induced transient, partial remission in one case, but a lethal outcome could not be prevented in either case. Monoclonal-antibody typing of lesional infiltrates indicated that LG is a malignant T-cell lymphoma.
Oral "hairy" leukoplakia is a new clinical entity and represents an early manifestation of the HTLV-III infection. The white lesions develop rather quickly; they are found principally on the lateral border of the tongue. The lesions are slightly raised and show a corrugated or "hairy" surface. This leukoplakia is associated with both papillomavirus and a herpes-type virus. A case report is presented and the literature discussed.
Fingerprint and palmar dermatoglyphics and creases were investigated in 60 patients (20 males and 40 females) with generalized neurofibromatosis. Like previous investigators, we found a significantly increased frequency of digital central pocket patterns. Furthermore, affected males and females had an increased frequency of monocentric whorls (P = 0.0037), higher quantitative values on digit II of both hands (P = 0.04), more often a reduction of main line C (P less than 0.05) with decreased frequencies of patterns in the 3rd and 4th interdigital area of the left hand (P less than 0.05), and a lower ab ridge count (males P less than 0.005; females P less than 0.001) than control individuals. On the right hand of males the frequency of high endings (5' or 5'') of line A was decreased (P less than 0.05). A significantly increased frequency of Sfl (Sydney line) was found in female patients (P less than 0.001). Male and female patients often showed a high number of secondary creases (P less than 0.001).
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A 60-year-old patient with "Floppy Eyelid" syndrome was treated by wedge-shaped lid resection. The excised tissue was studied by light and electron microscopy. Apart from the signs of chronic inflammation already described no abnormality was found in the ultrastructure or distribution of the collagen and elastic fibers of the tarsus.
Amiodarone, used in the treatment of cardiac arrhythmia, may lead to severe discolouration of sun-exposed skin. The lysosomal storage of a lipid-like material has been shown to be the morphological substrate for this cutaneous hyperpigmentation. Examination of peripheral white blood cells of amiodarone-treated patients disclosed identical lysosomal structures indicating that amiodarone treatment leads to a more generalized lysosomal storage of lipids and of amiodarone and its metabolites.
We present a female patient with a B-immunoblastic lymphoma of the IgM-lambda type arising in angioimmunoblastic lymphadenopathy. An increased ratio of helper/inducer to suppressor/cytotoxic lymphocytes in the lymph node could have triggered the proliferation of B cells. Evolution of IgM cryoglobulinemia was paralleled by malignant transformation in the lymph node. A short-term in vitro chemosensitivity test could predict response to COP combination chemotherapy suggesting that in vitro chemosensitivity testing can be useful for the therapeutic management of angioimmunoblastic lymphadenopathy.
The ultrastructural morphology of both the classical and the AIDS-associated forms of Kaposi's sarcoma was examined electron-microscopically. Tubuloreticular structures were found only in the AIDS-associated form of Kaposi's sarcoma, and not in the classical variant of the tumor. Moreover, the tubuloreticular structures, 20-30 nm in diameter, were present in very high numbers and in two different forms: the loosely intertwined tubuli, which were clearly predominant; and those forming a more compact pattern. These findings suggest that the presence of tubuloreticular structures may well be an ultrastructural marker for diagnosing AIDS and AIDS-associated disorders.
Modern concepts on the classification and prognosis of cutaneous T-cell lymphomas are discussed in this paper. The full spectrum of cutaneous T-cell lymphomas is not yet known. A new classification of histomorphological types of mycosis fungoides and Sézary's syndrome in correlation with prognostic features is proposed.
A case of a tapioca melanoma of the iris is studied by light and electron microscopy. Nodular elevations of the tumour were composed of spindle B- and epitheloid cells and of what appeared to be intermediate types. Tiny structures scattered over the entire tumour consisted of smaller epitheloid cells, many of which were found around superficial tumour vessels. Evidently, tapioca melanoma of the iris does not represent any specific histopathological entity. Tapioca melanoma of the iris is a low-grade malignant, locally aggressive tumour with a chronic clinical course.
We used electron microscopy to examine the ultrastructural morphology of Kaposi's sarcoma (KS) of the oral mucosa in patients with acquired immune deficiency syndrome (AIDS). The tumors manifested endothelial vascular proliferation and neoplastic spindle cell formations. The endothelial tumor cells contained several multivesicular bodies and a large number of tubuloreticular structures within the endoplasmic reticulum. Ultrastructural changes in the other cytoplasmic organelles included defective cell junctions that seemed to facilitate the migration and extravasation of erythrocytes through endothelial gaps, after which erythrophagocytosis occurred. Tumor cells contained viral particles which were 100-120 nm in diameter and contained dense cylindrical cores. We believe that this is the first time these particles have been identified in KS cells of the oral mucosa in patients with AIDS. These viral particles are also ultrastructurally identical to the human T-cell lymphotropic retroviruses subgroup HTLV-III. Our observations are discussed in the light of recent immunological findings.
An immunohistochemical staining procedure using Leu 7 (HNK-1) monoclonal antibody was used to study the distribution of myelin-associated glycoprotein in granular cell tumours. Positive reactions were noted in 10 of the 13 tumours investigated. This observation supports the concept that granular cell tumours are of Schwann cell origin.
Azelaic acid was successfully used in the clinical treatment of 7 cases of lentigo maligna in that remission of the lesions was observed in all our patients. In order to elucidate mechanism(s) of the beneficial clinical effects, we studied the effect of azelaic acid on cultured melanoma cells. Cell numbers recovered from melanoma cell cultures grown for several days in the presence of 10 mM azelaic acid were 50-70% less than those recovered from control cultures or from cultures containing 10 mM adipic acid. This reduction of cell numbers was not due to a simple cytotoxic or cytolytic effect of azelaic acid but rather due to a dose-dependent inhibition of DNA synthesis. Interestingly, nontoxic concentrations of azelaic acid, which significantly reduced DNA synthesis of cultured melanoma cells, had no overt effect on the protein synthesis of these cells. It is conceivable that inhibition of DNA synthesis is one of the mechanisms by which azelaic acid prevents growth and proliferation of abnormal melanocytes.
UNLABELLED: Amiodarone (AM) is one of the most potent antiarrhythmic drugs, the value of which is limited by reversible and irreversible side-effects (SE). 59 patients, 50 male, 9 female (age 33 to 81 years) entered the study with ventricular tachycardia (VT, 68%), WPW-tachycardia (12%), non-sustained VT (12%) or untreatable paroxysmal atrial fibrillation or supraventricular tachycardia (8%). Prior to AM the patients had received 1 to 8 different antiarrhythmic drugs (m 3.5) and maximal 9 different combinations of antiarrhythmics. The drug regimen started with a loading dose of 1200 mg/d for 1 to 2 weeks and was continued with a maintenance dose of 200 to 600 mg/d. The patients were followed up 1 to 41 months (m 14 m). The drug effect was evaluated using clinical criteria (recurrence of arrhythmias, death), computer-assisted analysis of several 24 hr long-term ECGs and programmed electrophysiological stimulation. Three- to six-monthly the patients were seen in our outpatient department for check up and blood-sample analysis (liver, thyroid gland etc.). Also in the majority of the patients frequent ophthalmological and dermatological investigations, as well as lung functions tests were carried out. RESULTS: under AM therapy the initial arrhythmias were no longer detectable in 41% of the patients. In 37% a significant amelioration of the formerly life-threatening arrhythmias was found. 7 patients (12%), predominantly with reduced left ventricular function, died during follow up. No pulmonary, hepatotoxic or neurological SE were found. All patients developed reversible AM keratopathy. In 27% ETR and T4 were elevated, but only one patient developed hyperthyroidism with an increase in TT3. Another patient showed signs of hypothyroidism with an elevated TSH prior to TRH stimulation. Increased phototoxicity was found in 31%, whereas in two patients typical AM hyperpigmentation occurred, especially on the face. In conclusion, AM is a highly effective antiarrhythmic agent, despite a negatively selected collective, but it should only be used in patients with refractory arrhythmia in view of the SE.
In 1975 we started a prospective study on oral methoxsalen photochemotherapy (PUVA) in cutaneous T cell lymphoma (CTCL). The first short-term follow-up of nineteen patients (1978) showed that PUVA may induce long-lasting remission in early stages, and that eventual relapses respond comparably well when PUVA is resumed. We now present the follow-up data of the original nineteen patients, covering a period of up to 7 years, and of an additional twenty-five patients who have entered the trial since April, 1977. Similar to earlier reports, all patients with eczematoid and plaque lesions (stages IA and IB) cleared. Likewise, eczematoid and plaque lesions in patients with early tumors (stage IIB) were cleared. During a mean follow-up of 44 months, 55% of stage IA patients and 39% of stage IB patients remained free of disease. In patients who experienced relapses, the mean disease-free interval was 20 months for stage IA and 17 months for stage IB. All patients with stage IIB experienced multiple relapses and only three of seven were alive after 6 years, despite additional x-ray or cytotoxic therapy. The observation in this study that five of nine stage IA patients and ten of twenty-six stage IB patients have remained in continuous remission after a single PUVA course for up to 79 months indicates that PUVA may induce long-lasting disease-free intervals if used in the early stage of disease. However, the observation period still does not prove whether permanent cure can be achieved in some cases or not.
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The first appearance, the 12-year follow-up, and the autopsy of a patient with a strange soft-tissue tumor in the lower leg region are described. Although it was originally taken for a malignant synovial tumor, its peculiar distribution pattern, together with its histologic picture, confirmed the final diagnosis as an epithelioid sarcoma.
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