[Papulonodular tuberculids of the lower limbs. An unusual anatomo-clinical form].
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Biomedical subjects
Publications and source records attributed to M Leporrier.
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Two cases of acute megakaryoblastic leukemia in a 4 month-old and a 13 year-old girl are described. In the first case who presented with a large hepatomegaly and portal fibrosis, the diagnosis was made from the surface phenotyping of megakaryoblasts; a trisomy 13, 14 and 19 and an extra chromosome X were present in the bone marrow. An electron microscopy study of megakaryoblasts was necessary to identify the second case. Both children died shortly after treatment (cytosine-arabinoside at low dosage in the first case and polychemotherapy in the second). The 51 other cases reported in the literature are reviewed.
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Selective cardiac involvement by a malignant non-Hodgkin's lymphoma (primary lymphoma of the heart) is extremely rare. Cardiac lesions occurring in the course of a malignant non-Hodgkin's lymphoma (secondary lymphoma of the heart) are found at autopsy in about 10 per cent of the cases. Secondary lymphoma of the heart may involve all cardiac structures, but lesions of the right heart, and particularly the right atrium, are predominant. Clinical manifestations are usually non-specific and appear at a late stage, reflecting diffuse involvement. When the malignant non-Hodgkin's lymphoma is known, systematic two-dimensional echocardiography should easily detect the lymphomatous cardiac infiltration, irrespective of the presence or absence of symptoms. When the malignant non-Hodgkin's lymphoma is unknown, only histology can show that the heart tumour is a lymphoma, evaluate its extension and confirm that it is limited to the cardiac walls, provided the disease is treated at an early stage, prolonged complete remissions are observed after chemotherapy and/or radiotherapy.
One to five percent of human renal cell carcinomas are associated with polycythemia. It is generally assumed that polycythemia results from the secretion of erythropoietin (Epo) by the malignant cells. However, there is no direct proof supporting this hypothesis. Three patients with typical renal adenocarcinoma and polycythemia were studied. All three exhibited high Epo serum levels as measured by radioimmunoassay (RIA). A strong Epo signal was observed on Northern blot analysis of total RNA extracted from the renal tumors. The Epo message seemed to be of normal size and no Epo gene rearrangement was observed with the restriction enzymes tested. Using the in situ hybridization technique, a significant labeling was constantly observed on the tumor cells. Immunohistochemical studies showed that these tumor cells, known to be of tubular origin, were labeled by an anti-cytokeratin antibody and therefore were of epithelial nature. Thus, this study demonstrated that malignant cells of tubular origin were able to produce Epo constitutively, whereas in the mouse hypoxic kidney, peritubular cells (probably capillary endothelial cells) were the major site of Epo synthesis.
In a patient treated for acute myeloblastic leukemia (AML), we saw an angiocentric and angiodestructive lymphoma that resembled lymphomatoid granulomatosis (LG). The lesions tended to involve extranodal sites such as the lung, the parotid gland, and the skin. The immunologic studies showed that the proliferating lymphoid cells were mature T cells. Furthermore, genotypic studies disclosed a clonal rearrangement of the beta T-cell receptor gene. It is concluded that this case of LG is related to a neoplastic T-cell lymphoproliferative disorder. The relations between LG and the previous AML are discussed.
The prognosis of adults with Burkitt's lymphoma is very poor and depends on initial CNS and/or bone marrow involvement. We report results in nine adult patients with CNS (n = 9) and/or bone marrow involvement (n = 7) treated in first complete remission (CR) with allogeneic bone marrow transplantation (BMT). CNS treatment before the conditioning regimen consisted of cranial irradiation at 15 Gy (n = 8) and intrathecal chemotherapy (n = 9). The conditioning regimen included cyclophosphamide and total body irradiation (TBI) in a single dose. No postgraft CNS prophylaxis was administered. At the present time, seven patients are alive and disease-free at 18, 23, 44, 47, 54, 54, and 59 months. Two patients died at 14 and 7 months from transfusion-related acquired immune deficiency syndrome and bacterial septicemia and were disease-free at the time of their death. These preliminary results should encourage the use of BMT. A prospective randomized trial is warranted to further specify and investigate the advantages of allogeneic BMT versus conventional chemotherapy.
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In patients with acute leukaemia, Candida infection may affect exclusively the liver and the spleen. Two such cases were revealed by persistent fever despite correction of bone marrow aplasia, abdominal pain, anicteric cholestasis and hypodense areas at computerized tomography suggesting hepatosplenic abscesses. Surgical liver biopsy confirmed the fungal infection and showed images of granuloma, mycelial filaments and yeasts; cultures were usually negative. The severity of these infections requires an early treatment, but amphotericin B is not very effective. Our two patients were cured after treatment with fluoconazole completed, in one of them by splenectomy.
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A 72-year-old woman presented with rapidly progressive renal failure and multiple myeloma. The patient died 6 months later of severe hepatic insufficiency. The light-microscopic, immunological and ultrastructural findings showed widespread kappa-light-chain deposits including the kidneys, liver, spleen, heart, lungs, tongue, ovary, pancreas and bone marrow associated with massive AL amyloid deposits in the same organs and in the thyroid gland. The concurrent presence of two different deposits is very unusual and the possible mechanisms for such an association are discussed.
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Two cases of Gaucher's disease type I are reported Splenectomy was indicated because of hypersplenism and massive splenomegaly. In one case hypersplenism was treated with pre-operative selective embolization because of the volume of the spleen (20 kg). The embolization corrected the thrombopenia but not the size of the spleen. Four years after operation for case 1 and eight months for case two, there is an improvement in the clinical status.