Biomedical subjects
Publications and source records attributed to M Krulik.
A translocation t(1;7) interpreted as t(1;7)(p11;p11) was first reported by Scheres et al. in eight patients with various hematologic disorders. The karyotype of the abnormal cells was trisomic for 1q and monosomic for 7q. Those investigators reported having found four other cases in the literature. We report herein studies of two patients with the same t(1;7).
Three cases of retinal vasculitis in SLE-type diseases are reported. The first was central retinal vein occlusion occurring during clinical remission of SLE in a 55 year old black female. Prednisone maintenance therapy was unchanged and visual loss rapidly regressed with heparin therapy. The second case was a 33 year old black female in whom SLE was discovered following relapsing bilateral optic neuritis. A progressive visual improvement was obtained with high dose of prednisone (1 mg/kg/day). The third cas was a 17 year old white girl with retinal vasculitis. She had an unclassified connective tissue disease inaugurated by optic neuritis at the age of 10. High dose prednisone (1 mg/kg/day) was effective on the visual loss. Retinal vasculitis lesions in SLE and their therapy are reviewed.
2 cases of multiple myeloma, both with IgG kappa, are reported in a man and his daughter. 33 other cases of myeloma involving two or more first degree relatives have been reported in the literature. Reported cases showed no major specific characteristics of the myelomas involved. Monoclonal protein family members were rarely identical. Our observation of identical monoclonal protein in two family members has only been found in 4 other reports. Occurrence of several cases of myeloma in one family is unlikely to be due to chance alone and the possibility of familial myeloma must be considered. Reports of benign monoclonal gammapathy in other members of the family of a patient with myeloma is also in favor of genetic factors. HLA-typing has shown an increase in frequency of the 4c complex and HLA-A9 in myeloma, and the latter was present in our patients. A genetic predisposition is further supported by experimental observations in mice. Finally, the short delay between onsets of myeloma in family members, less than 5 years in 20 families out of 27, and 4 years in our case, suggests a possible role of environmental factors.
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Degree and extent of lytic bone lesions were quantified at diagnosis in 69 patients with myelomatosis (MM). longest survival was found in patients with minimal lytic lesions and shortest survival was found in those either without bone lesions or with severe and extensive lesions. Among the 12 patients without bone lesions only 6 were Durie & Salmon stage I and only 1 later developed a single lesion. Based on these data, bone involvement in MM does not seem to be a reliable predictive factor of survival in all cases. This suggests the existence of 2 distinct types of MM: 1 with tumour and 1 without. Lytic lesions could have a prognostic value only in the 1st type.
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From 1976 to 1981, 335 patients with untreated Hodgkin's disease, clinical stages I, II, and IIIA, have been treated by MOPP (nitrogen mustard, vincristine, procarbazine, prednisone) chemotherapy, three to six cycles according to the prognostic factors, combined with radiotherapy. Irradiation was always performed after the first three cycles of chemotherapy, and was randomized between extensive radiotherapy, ie, mantle and paraaortic areas for supradiaphragmatic presentations, and radiotherapy restricted to the involved areas. No significant difference was observed between the two randomized branches for the disease-free survival (86% after six years in the involved field branch v 90% in the extended field branch), and none for the overall survival. Most of the relapses occurred in nonirradiated areas in the first group, and in irradiated areas in the second. Relapses were especially frequent in the IIE stages with pulmonary extension; extranodal relapses occurred with osseous and cutaneous localizations. Two cases of secondary leukemia were observed after three- or six-cycle MOPP plus radiotherapy limited to the involved areas.
A case of extraskeletal mesenchymal chondrosarcoma in a 62-year-old woman is reported. This unusual sarcoma was localized in the right thigh (quadriceps) and treated with a combination of surgery, chemotherapy and radiotherapy. Sixty-three cases were found in the literature, with neurologic localization in 29 cases and muscular localization in 34 cases. Among clinical features, a significant difference in age at the time of diagnosis was found between the two localizations, which strongly suggests the possibility of two different types of extraskeletal mesenchymal chondrosarcoma.
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The histological study of 36 cases of Non-Hodgkin Lymphomas beginning for the E.N.T. area has allowed us to show that the Non-Hodgkin Lymphoma Working Formulation made for the lymph nodes may be used to ENT Localization and give a reliable prognosis. Other elements like: sex, clinical staging, treatment have no significant value concerning the survival rate in our study. This study agrees with the previous studies about the predominance cases to the high power of malignancy of these lymphomas.
The first case of haemolytic anaemia with thrombocytopenia and acute renal failure induced by ingestion of aspirin in a 22-year old woman is reported. An IgM anti-aspirin antibody which agglutinated erythrocytes of the patient and of ABO compatible donors in the presence of aspirin was isolated in the serum. In addition, the allergic nature of the patient's hypersensitivity to aspirin was confirmed by positive lymphocyte transformation and basophil degranulation tests in the presence of the drug.
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Forty patients with stage III or IV ovarian epithelial carcinoma, mean age 61 +/- 12 years, were treated either with cyclophosphamide-methotrexate-5-FU or with cisplatinum-adriamycin-5-FU-hexamethylmelamine. Median survival from initial surgery was 16 months, 18 months in stage III and 2 months in stage IV disease. 15 patients over 70 years had a median survival of 9 months. Median survival was higher with the regimen without cisplatinum (18 months against 13 months). Clinical response rate was 62.5%. Ten patients clinically free of disease underwent second look laparotomy which showed persistent disease in six. Based on these results and on a review of the literature, a new therapeutical approach is discussed.
The authors report three cases of Ph1-positive chronic myelogenous leukemia (CML) with chromosome no 1 abnormalities. Such abnormalities have seldom been reported: three cases out of 42 in blast crisis and none of the 70 patients in the chronic phase in our series. In case no 1 a translocation: (t(1q;14q) was noted. In case no 2 the rearrangement was more complex: partial duplication of the long arm of chromosome no 1 and presence of a 21q+ corresponding to chromosome no 21 on which a part of the long arm of chromosome no 1 was transferred. In case no 3 a double translocation was noted between chromosomes no 1 and no 11, involving either the p or q arm. A review of the literature shows that abnormalities of chromosome no 1 are more frequent during the blast crisis that in the chronic phase of CML. Chromosome no 1 abnormalities are found in a number of myeloproliferative syndromes but even more frequently in solid tumors. The rearrangement sites are reviewed by the authors. Such rearrangements of chromosome no 1 may indicate an increased potential of malignancy.
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Two female patients, 42 and 30 years old, respectively, died of acute nonlymphocytic leukemia 43 and 38 months, respectively, after a subsequent treatment: chemotherapy for one and irradiation and chemotherapy for the other, following excision of a malignant glioma. At the time of death, both seemed to be in complete remission of their brain tumor. Both had been treated with procarbazine and nitrosoureas. The latter were responsible for severe myelosuppressive episodes and seem to have played an essential role in the induction of the leukemia. In one case, a myelodysplasia was observed before the onset of the AL and the diagnosis of refractory anemia with excess of blasts seemed warranted. Secondary acute leukemias are rare in the evolution of malignant gliomas and the usefulness of subsequent radiochemotherapy cannot be questioned at the present time. The risks involved in this therapy are minor when compared to the short-term fatal prognosis of this type of tumor.
The authors present 7 cases of preleukemic state and/or acute leukemia following the treatment of a first malignancy (3 malignant lymphomas, 3 epithelial cancers and one polycythemia vera). A preleukemic state was found in all patients. In 6 cases it was followed by an acute non-lymphoblastic leukemia which was rapidly fatal; in one case, death occurred during the preleukemic phase. A karyotype was prepared during the preleukemic phase in 6 out of 7 patients and in 4 out of 6 during the leukemia. Karyotypes prepared during the preleukemic state presented at least one of the abnormalities which are characteristic of secondary hematopoietic disorders: -5/5q; -7/7q; involvement of 17. The same major clone was found in 3 of the 6 patients who were karyotyped during the acute leukemic phase. These karyotype abnormalities are not only characteristic of secondary acute leukemias but also of secondary dysmyelopoietic syndromes.