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Biomedical subjects

A Delmer

Publications and source records attributed to A Delmer.

107 records · Page 6Linked to original sources

[Peripheral T-cell malignant lymphomas. Clinical, morphologic and developmental features in 22 cases].

Peripheral T-cell lymphomas (PTCL) represent a new subset of malignant lymphomas, which demonstrates a marked morphological, immunological and clinical diversity. They seem to have a worse prognosis globally than B-cell lymphomas. The main clinical characteristics and outcome of PTCL are analysed in this series of 22 cases. The majority of patients had an advanced disease (stages III and IV; 55 percent) and constitutional symptoms (59 percent) at presentation; extranodal localizations were particularly frequent (41 percent). Three patients presented with isolated or predominant spleen enlargement and fever. According to the updated Kiel classification, there were 7 low-grade PTCL and 15 high-grade PTCL. Phenotypic analysis on fresh frozen tissue was available in 16 cases, showing a predominant helper/inducer phenotype (CD4+, CD8-). The complete remission rate was 76 percent for the whole population, but the median of global survival was only 40 months. The few patients who received radiation therapy and subsequently relapsed did not relapse in the irradiated fields, which suggests that radiotherapy might be included in the therapeutic strategy, the best modalities of which remain to be defined.

Adolescent↗

Recurrent spleen enlargement during cyclic granulocyte-macrophage colony-stimulating factor therapy for myelodysplastic syndrome.

A 65-year-old woman with refractory anemia with excess of blasts received sequential courses of granulocyte-macrophage colony-stimulating factor therapy (GM-CSF) and low-dose cytosine arabinoside. Each course of GM-CSF induced a rapid and tremendous increase in leukocyte count as well as in spleen size, 111indium chloride scanning suggested a myeloid metaplasia of the spleen. This observation suggests that in some patients the granulopoietic response to the myeloid growth factor stimulation may be predominant in the spleen.

Aged↗

[Castleman's disease (giant lymph node hyperplasia): clinical, biological and developing polymorphism. Apropos of 4 cases].

Four cases of plasma cell type Castleman's disease (CD) are described. Two patients had localized forms (one mediastinal and the other mesenteric) and presented systemic manifestations associated with hypergammaglobulinemia and severe anemia. In both cases, the lesions were revealed by computerized tomography scans and cures were obtained by the complete surgical removal of the masses, which led to the rapid disappearance of the systemic manifestations. The other 2 patients had the multicentric form of CD and presented more extensive clinical and biological symptoms. One of these developed severe peripheral neuropathy and endocrine anomalies during the late phase of his disease, which led us to discuss the relationship between multicentric CD and the POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin change) syndrome first described in Japan. Three of our patients presented with hypochromic microcytic anemia too severe to be explained by an inflammatory syndrome alone, and was likely due to several mechanisms. The etiology of CD remains unknown. The histological characteristics of angiofollicular lymph node hyperplasia are among the most important criteria for the diagnosis of localized and multicentric forms of CD, which can easily be made on a lymph node biopsy. However, it must be noted that this lesion can also be observed (but only rarely) in HIV (human immunodeficiency virus) - infected patients. The localized form is always considered to be benign, but, to date, there is no formal argument definitively supporting the malignancy of the multicentric one, in spite of its clinical similarity to a lymphoproliferative syndrome.

Adult↗

Multivariate analysis of prognostic factors in acute myeloid leukemia: value of clonogenic leukemic cell properties.

The initial clinical and biological parameters, including clonogenic leukemic cell (CFU-L) assay, were reviewed for their prognostic significance in a cohort of 188 adult patients with newly diagnosed untreated acute myeloid leukemia (AML). Almost all patients received induction therapy with daunorubicin (DNR) and cytarabine (Ara-C) according to the European Organization for Research and Treatment of Cancer (EORTC) AML 5 to AML 9 trials. Bone marrow samples from 116 representative patients were obtained for CFU-L assay with an efficiency percentage of 89.6%; 76 patients had a measurement of the CFU-L self-renewal capacity (second plating efficiency [PE2]) and 91 patients had CFU-L inhibition test after exposure to DNR and/or Ara-C. The prognostic significance of parameters such as age, hematological antecedent, WBC count, liver enlargement, and Auer rods is confirmed in the present study. Moreover, high platelet and polymorphonuclear counts appeared to be related to resistance to induction course. However, through multivariate analysis, CFU-L sensitivity to drugs and self-renewal capacity appeared to be major independent prognostic factors in AML. A low CFU-L inhibition in the presence of the DNR and Ara-C combination correlates with a poorer complete remission (CR) rate, but not with CR duration. Patients with the lower PE2 values experienced both higher CR rate and longer CR duration. The practical interest of CFU-L study remains to be defined but, at least, PE2 measurement could be considered in the future as a major variable in determining therapeutic aggressiveness.

Adolescent↗

Life-threatening intracranial bleeding associated with the presence of an antifactor VII autoantibody.

This is a report of a 62-year-old male patient who had a bleeding disorder due to the presence of a factor VII (proconvertin) inhibitor. After treatment with a high-dose intravenous (IV) immunoglobulin failed and a life-threatening intracranial hemorrhage occurred, plasma exchanges were performed and immunosuppressive therapy was given. The factor VII inhibitor promptly disappeared, and the patient's parameters of hemostasis became normal. Even though a relapse occurred some months later, the patient responded to immunosuppressive therapy. No underlying disorder was evidenced after 17 months of follow-up.

Autoimmune Diseases↗

[Antibiotic therapy protocol using ceftazidime 3g/day alone or in combination with vancomycin or amikacin. In febrile episodes in neutropenic patients].

In a preliminary study of 21 febrile episodes in neutropenic patients ceftazidime used as empirical treatment in doses of 3 grams per day succeeded in controlling fever in 74 per cent of the cases. Laboratory studies performed in patients with Gram-negative septicaemia showed clinically effective plasma concentrations of the antibiotic. A trial of ceftazidime (3 g/day) administered alone or combined with amikacin or vancomycin is currently in progress in two medical centres. No statistically significant conclusions could be reached from an intermediate study.

Agranulocytosis↗

[Acquired factor VII inhibitor: treatment using high-dose immunoglobulins, corticotherapy and plasma exchange].

A 62 year old man presented a diffuse hemorrhagic syndrome related to the presence of an acquired factor VII inhibitor (proconvertin). Autoantibodies were of the IgG class and no subjacent disease was detected during a 7-month follow up. Treatment with high-dose polyvalent immunoglobulins was ineffective, and the onset of an intracerebral hemorrhage required therapy by a series of plasma exchanges combined with immunosuppressive treatment. The plasma inhibitor was no longer present after the first exchange and clinical and biological remission was rapid.

Cerebral Hemorrhage↗

Prognostic value of clonogenic assay for induction and duration of complete remission in acute myelogenous leukemia.

The validity of an in vitro clonogenic drug sensitivity assay to predict the induction and the duration of complete remission was evaluated in a group of 81 patients with acute myelogenous leukemia treated with chemotherapy including an anthracycline drug (daunorubicin or adriamycin) and cytosine arabinoside (Ara-C). The inhibition of bone marrow clonogenic leukemic cells by in vitro exposure to anthracyclines 10(-5) and 10(-6) M, Ara-C 10(-5) M, and daunorubicin 10(-6) M + Ara-C 10(-7) M was significantly correlated with the achievement of a complete remission, but not with the duration of remission. A high second plating efficiency was correlated with short duration of complete remission, reflecting the poor prognosis of a high self-renewal capacity.

Adolescent↗

Histopathological study of bone marrow biopsies in 30 cases of T-cell lymphoma with clinical, biological and survival correlations.

Forty-five bone marrow (BM) biopsies have been studied in 30 T-cell malignant lymphoma (ML). According to the updated Kiel classification, these ML comprised 12 low grade ML and 18 high grade ML. BM involvement was not significantly more frequent in low grade ML (41.6 per cent) than in high grade (33.3 per cent). This involvement was discovered in 85 per cent of the cases at the time of diagnosis. A correlation was found between BM and other visceral localizations for histological type in all cases. Infiltrates principally showed a nodular pattern in low grade and a diffuse pattern in high grade ML. Hematopoietic hyperplastic reaction was frequent (66.6 per cent) not correlated to involvement. Clinical staging showed extensive spreading. Our patients had an overall median survival of 40 months, worse in high grade ML (median: 19 months) than in low grade ML (41 months) but not statistically different (p = 0.25). Extranodal localizations are a significant criteria for poor prognosis (p = 0.018). Among them, BM involvement appears to be the most significant criteria (Cox model, p = 0.006). Patients with BM localization had a median survival of 9 months contrasting with 40 months in patients without BM localization (p = 0.007). Thus, BM biopsy is useful for the diagnosis of patients with T-ML and essential to establish the prognosis.

Adult↗

[Aleukemic monoblastic leukemia cutis].

BACKGROUND: The diagnostic and prognostic value of specific cutaneous lesions in acute leukemia is well-known. Paradoxically, these lesions may initially develop without peripheral blood or bone marrow involvement. We report the case of a patient with cutaneous lesions of acute monoblastic leukemia whereas peripheral blood was normal and massive infiltration of dermis was demonstrated. OBSERVATION: A 49 year-old man had papules and nodules of the back and upper arms evolving for several months. Histological examination with appropriate immunostaining led to the diagnosis of specific cutaneous lesions of acute monoblastic leukemia. Several hemograms with peripheral blood smears were normal, bone marrow smear demonstrated an important blastic infiltration on one site and a discrete infiltration on another. Cutaneous lesions disappeared with chemotherapy. DISCUSSION: Specific cutaneous lesions may be isolated during acute leukemia, and called aleukemic leukemia cutis. These are a rare form, the underlying mechanism of which relies on the accumulation of small quantities of myeloblasts in bone narrow and with high tropism for the dermis.

Acute Disease↗

Waldenström's macroglobulinemia with prominent splenomegaly and multiple immune disorders.

We report an uncommon case of Waldenström's macroglobulinemia with prominent splenomegaly associated with acquired C1 inhibitor deficiency, lupus anticoagulant and red blood cell autosensitization. Each of these immune abnormalities has been occasionally reported separately in patients with diverse B-cell lymphoid malignancies, but never before have they appeared simultaneously in the same patient. In this case, the pathogeny of the C1 inhibitor deficiency is questionable and may possibly be directly related to the IgM monoclonal gammapathy through protein-protein interactions, as occurs for lupus anticoagulant.

Autoimmune Diseases↗