[Hypercalcemia in chronic myeloid leukemia].
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Biomedical subjects
Publications and source records attributed to O Reman.
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While investigating the cause of mild polycythemia in a young man, a testicular seminoma was discovered with unusual and tumor-dependent features: an absolute polycythemia with high plasma erythropoietin (EPO) levels, an overproduction of estradiol and testosterone, and a dramatic Leydig cell hyperplasia surrounding the tumor tissue. The authors attempted to gain insight into the relationship between this testicular tumor and the hormonal overproduction, i.e., of EPO, estradiol, and testosterone. Their results favored the conclusion that the high EPO levels and the polycythemia were an indirect effect secondary to the steroid overproduction rather than a direct EPO-producing activity. Moreover, the steroid overproduction by the testis could be caused by a paracrine mechanism through human chorionic gonadotropin activity on the Leydig cells.
A case of Sweet syndrome in a patient with an acute myelomonocytic leukemia is reported. It is an unusual case simulating a facial erysipela. This case illustrates the difficulty in differentiating infection from Sweet syndrome in an immunocompromised patient. The negative cultures, ineffective antibiotics, and histopathologic examination results allowed the onset of corticosteroid therapy. The phagocytic function of the neutrophils studied by chemoluminescence was normal in the patient. The physiopathology of Sweet syndrome is discussed, in particular the role of cytokines in the accumulation of granulocytes.
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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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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.
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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 13 year-old girl was diagnosed as having acute megakaryoblastic leukemia. A serious infectious syndrome appeared during the chemotherapy, not improved by broad spectrum antibiotic therapy. A pulmonary aspergillosis was diagnosed one month later by a second bronchoalveolar lavage. A treatment with Itraconazole, a new antifungal triazole, was started. Despite this treatment, the child died after 3 days. Death was due to multiple aspergillus abscesses disseminated in the brain leading to coma and transtentorial herniation. Autopsy confirmed the cerebral aspergillus abscesses and showed also the dramatic dissemination of aspergillosis in the body. Diagnosis and treatment to aspergillosis in immunosuppressed patients should be made early to improve prognosis.
Two months after discontinuation of maintenance chemotherapy for acute lymphoblastic leukemia, an eight-year old boy developed anterior uveitis shown by aspiration of the anterior chamber to be caused by a recurrence. At the initial stage of ocular involvement, no evidence of neuromeningeal involvement was present. Remission was achieved by topical corticosteroid therapy, systemic chemotherapy, and irradiation of the eyeballs. The patient then underwent total body irradiation, major chemotherapy, and autogenic bone marrow transplantation. The fact that the eye acts as a "sanctuary" explains why delayed ocular recurrences are occasionally seen in acute lymphoblastic leukemia.
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