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Biomedical subjects

O Reman

Publications and source records attributed to O Reman.

At least 55 records · Page 3Linked to original sources

[Primary adrenal lymphoma. Report of a case].

The adrenal gland is affected in about 25% of patients with diffuse lymphoma. Conversely, primary lymphoma is a rare cause of non-secreting adrenal tumour. We observed a recent case with exclusive right adrenal localization. An 84-year-old woman was hospitalized for asthenia, anorexia and a 3-kg weight loss. The past history included coronary heart disease. Physical examination was normal and the hemogram revealed pancytopenia. No lymphomatous infiltration was seen on the myelogram or the bone marrow biopsy. Echography of the abdomen showed a well-limited 10 cm mass above the right adrenal gland, distinct from the liver; the left gland had a normal aspect. Hormone assays were within normal limits. The right adrenal gland was removed surgically and histology revealed diffuse proliferation of medium to large sized lymphoid cells invading the entire gland. Immunohistochemistry suggested lymphoid B origin. No curative treatment was begun, but 2 months after surgery local invasion required polychemotherapy. The patient died of septic shock 5 days later. Serum cortisol measured during the septic shock eliminated adrenal gland failure. Malignant primitive non-Hodgkinien lymphomas are extremely rare and usually discovered incidentally. The tumour is always large and sometimes accompanied by adrenal insufficiency when the lesion is bilateral. In spite of suggestive imagery the diagnosis is based on the pathology examination. The lesion is usually a high-grade malignant phenotype B lymphoma. Treatment relies on chemotherapy but is disappointing.

Adrenal Gland Neoplasms↗

[Clostridium perfringens septicemia in drug-induced aplasia].

Septicemia due to Clostridium perfringens during the course of acute leukemia is rare and often lethal particularly in childhood. Antibiotherapy is necessary but polymorphonuclear activity recovery is helpful. This can be done through transfusion or administration of colony stimulating factors. Here is a new case of such a septicemia in a 12 year-old female treated for acute lymphoblastic leukemia. Of particular interest is the favourable outcome despite a high risk situation.

Anemia, Aplastic↗

Double hemibody irradiation with GM-CSF as salvage therapy for refractory chronic lymphocytic leukemia.

We describe a new and original therapy with total body irradiation in two separate 4 gy single courses (double hemibody irradiation) combined with GM-CSF support, 5ug/day on days 1-15 after each hemibody irradiation, for refractory patients with B-chronic lymphocytic leukemia (CLL). A complete response was observed in a patient with a B-CLL resistant to CAP and FAMP therapy. Overall tolerance was good. The major points of interest in this technique are the combination of the antitumor effect of irradiation, limited bone marrow toxicity and a potential specific anti-leukemia effect of GM-CSF.

Aged↗

Unstable alpha-chain hemoglobin variants with factitious beta-thalassemia biosynthetic ratio: Hb Questembert (alpha 131[H14]Ser-->Pro) and Hb Caen (alpha 132[H15]Val-->Gly).

Hb Questembert [alpha 131(H14)Ser-->Pro] was found in several members of a French family suffering from congenital Heinz body anemia. The unstable hemoglobin was expressed in the peripheral red blood cells at a very low level. Globin biosynthetic studies revealed a high specific activity of the abnormal chain and an alpha-/beta-labeling ratio similar to that of beta-thalassemia trait. Hb Caen [alpha 132(H15) Val-->Gly] is another unstable variant with the same globin biosynthesis abnormality. In both cases the structural modification is localized at the end of the H helix, a region encoded by the third exon. The mechanism for the unbalanced globin synthesis is not yet clear. It may be related 1) to a defect in chain assembly, 2) to an increased rate of degradation of the variant chain followed by the release of unlabeled beta-chains from the abnormal hemoglobin, thus leading to an apparent suppression of beta-chain synthesis, or 3) to a modified stability of the abnormal alpha-globin mRNA.

Adult↗

Childhood leukemia incidence in the vicinity of La Hague nuclear-waste reprocessing facility (France).

The incidence of leukemia is examined in young people (aged under 25 years) living within a 35 km radius of the French nuclear-waste reprocessing plant operating in La Hague, Normandy. During the period 1978-90, a total of 23 cases was diagnosed, giving an incidence rate of 2.99 per 100,000 which is close to the expected rate. In the 'canton' in which the nuclear plant is located, three cases of leukemia were observed compared with 1.2 expected, giving a standardized incidence ratio of 2.5. This nonsignificant finding is compatible with no increased risk and also with a sevenfold excess risk. Therefore, it reinforces the necessity of conducting a case-control survey, which is now planned, to assess the part played by occupational radiation exposure in leukemia incidence for this French area.

Acute Disease↗

CD2 + CD19 + acute lymphoblastic leukaemia in 16 children and adults: clinical and biological features. Groupe d'Etude Immunologique des Leucémies (G.E.I.L.).

A small population of CD2 + CD19 + lymphoid cells have been suggested to be common lymphoid progenitors. CD2 + CD19 + biphenotypic ALL account for less than 2% of ALL. We analysed the clinical and laboratory features of a series of 16 patients with CD2 + CD19 + ALL. The incidence of tumoural syndrome was comparable to a previously published series of pre B-ALL but significantly different from that of T-ALL. The mean age of the 11 children of this series was 101 +/- 46 months, and differed significantly from that of children with pre B-ALL (P < 0.01). Complete remission was obtained for all patients except two adults. Only three relapses have been observed. Regardless of the presence of CD2 +, the 16 ALL could be classified as pre B-ALL, according to the nomenclature used by the GEIL. Nine samples could be analysed by Southern blotting. Seven had rearranged IGH genes, usually on both chromosomes. IGK rearrangement was observed in three cases. Only one case had rearranged both TCRG and TCR beta. The patterns observed here and those reported previously follow that of the pre B-ALL which confirms the engagement of most CD2 + CD19 + biphenotypic ALL in the B-lineage.

Adult↗

Virus recovery from stools of patients undergoing bone marrow transplantation.

Diarrhea in marrow transplant recipients is a frequent complication attributable to non-infectious events such as acute GVHD or infectious events such as viral gastroenteritis. Rotavirus and enteric adenovirus are the most frequent viral pathogens. To determine the frequency of these infections, we prospectively examined the stool specimens of 94 patients who underwent autologous BMT (34 cases) or allogeneic BMT (60 cases). Stool specimens were examined from patients twice weekly. Nineteen of the 94 patients were infected with viral pathogens. This study showed: (1) an incidence of viral gastroenteritis identical in autologous and allogeneic BMT (20%), (2) a persistent risk despite treatment in laminar air flow rooms, (3) a significant association with severe acute GVHD, and (4) a significant risk of multiple viral infections in autologous BMT recipients. Rotavirus and adenovirus are a cause of enteritis involvement in patients undergoing BMT and they may be underdiagnosed and confused with GVHD. Screening of stool specimens after BMT should be directed to prevention and treatment of these viral infections to decrease the morbidity and mortality associated with BMT.

Adenovirus Infections, Human↗

Polycythemia and steroid overproduction in a gonadotropin-secreting seminoma of the testis.

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.

Adult↗

Sweet syndrome associated with acute myelogenous leukemia. Atypical form simulating facial erysipelas.

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.

Adult↗

[Acute megakaryoblastic leukemia. Relation to trisomy 21].

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.

Adolescent↗