[A case of acute adult respiratory distress associated with methemoglobinemia].
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Biomedical subjects
Publications and source records attributed to X Troussard.
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The DBA44 monoclonal antibody described by Al Saati recognizes a membrane antigen expressed by a sub-population of B-lymphocytes. It was tested on paraffin-embedded tissues, and it distinguishes Hairy Cell Leukemia (HCL) from the more common B-cell Chronic Lymphocytic Leukemia (CLL). Neither Splenic Lymphoma with Villous Lymphocytes (SLVL) cases nor Prolymphocytic Leukemia (PLL) cases were tested. We have tested 87 B-lymphoproliferative disorders with DBA44 on cytocentrifuge preparations. All five HCL cases were positive (100%). Of 24 cases of SLVL, 19 were positive (79%); of 58 other B-cell malignancies, five cases were positive (8.5%), including 1/8 CD5- CLLs, 1/5 PLLs and 3/24 lymphomas. All CD5+ CLL were negative. DBA44 positivity cannot distinguish HCL from SLVL, which is the disease that may create major diagnostic problems. In contrast, when we compare SLVL to CLL (which is another diagnostic problem), significant differences were found between the incidence of DBA44 positivity in SLVL and both CD5+ B-CLL (p< 10(-5)), and CD5- B-CLL (p< 0.01). The monoclonal antibody DBA44 is positive in HCL and SLVL. It is not helpful in the differential diagnosis of HCL and SLVL. In contrast, when a diagnostic problem arises between SLVL and CLL, the reactivity of DBA44 is of great value.
Three cases of light chain deposition disease are reported. The condition was associated with monoclonal dysglobulinaemia in two cases and with amyloidosis in one case. This, and the different course of the disease in these three patients, illustrates the need for an early histological diagnosis, using immunofluorescence with monospecific anti-light chain sera.
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B-prolymphocytic leukemia (B-PLL) is an infrequent disease with a poor prognosis. We present the clinical and biological features of 41 patients. Median age was 67 years [42-89] and male-female sex ratio was 2.4. The immunophenotyping revealed B-cell phenotype, with a high level expression of surface IgM and/or IgD in all cases, FMC7+ in 76 % of cases and CD5+ in 67%. Marked spontaneous in-vitro apoptosis was observed in most cases tested (n = 12). The median overall survival time was 5 years and the event-free survival time was 37 months. As detected by univariate and multivariate analysis, the only variables associated with a poor prognosis were advanced age and anemia. No significant difference was observed between de novo PLL (n = 27) and prolymphocytoid transformation of chronic lymphocytic leukemia (n = 14). Two groups of patients were individualized according to their clinical course: patients who died within one year of diagnosis (n = 14) and patients who had a prolonged survival (n = 23) without any treatment in some cases. The comparison between the 2 groups showed that they differed in age (p = 0.01) and anemia (p = 0.02). We also observed that the patients with p53 mutations had a worse clinical outcome. Taken together these data confirm that B-PLL should be regarded as a distinct form of chronic lymphoproliferative disorder and suggest the existence of two patterns of clinical evolution.
The appearance of schistocytes in a peripheral blood film is considered to be an important diagnostic marker for thrombotic microangiopathy. However, the morphological analysis of schistocytes remains uneasy. To determine practice patterns in the biological management of schistocytosis, the French Group of Cellular Hematology from the French Society of Hematology conducted a survey on the approach of the diagnosis of microangiopathy. A guideline is proposed in order to cancel the substantial variation among biologists.
Schistocytes are red blood cell fragments observed on a blood smear. They are a mark of mechanical haemolytic anaemias whose group of the thrombotic micro-angiopathies requires an urgent treatment. The detection of the schistocytes and sometimes their quantification are thus of primary importance. To evaluate this search for schistocytes, several surveys of practice were carried out (1999-2003) including pictures of blood fields (identification of schistocytes among abnormal red blood cells) near biologists of variable level of specialization. The aim was to try to lead to a consensus, in particular for the morphological criterias of identification. Our results indicated that: 1) the biologists are badly sensitized with the importance of this research and the consequences of their response for the diagnosis; 2) the morphological identification of the schistocytes is difficult with an important variability of the criteria according to the observers. An investigation overviewed by the French Group of Cellular Hematology (Delphi method) allowed the development of a morphological consensus (fragments of triangular/crescent/helmet forms with rectilinear zone testifying to the zone of break). In order to cancel the observer-dependent identification of the schistocytes, a software of morphometric analysis (Q-WIN, Leica) was developed for sorting, starting from digitalized microscopic fields, the fragmented - among the normal - red blood cells. The results appeared encouraging, but not yet optimized. An automated analyzer (Bayer ADVIA 120) was also evaluated for the measurement of the schizocytes ("fragmented red blood cells" parameter). The moderate over-estimation of the real schizocytes (+ 0,4%) encouraged to observe the clinical value of the fragmented red blood cells detection in a group of patients that undergone a bone-marrow transplantation. The predictive value of the test (98%) was satisfactory.
We present two cases of primary malignant lymphoma of the liver, a very unusual localization of primary malignant lymphoma since only 31 similar cases were reported in the literature. The main symptoms included: hepatomegaly, fever, slight cholestasis and non specific alteration of the liver appearance on ultrasonography or computed tomodensitometry examination. Diagnosis was established in both cases by histopathological demonstration of infiltration by large lymphoid cells. Immunophenotyping in one case confirmed a B cell phenotype. Primary malignant lymphomas of the liver are high-grade malignant large-cell tumours and the immunophenotype, when known, is of the B type. The prognosis of these tumours is not well known due to their extreme rarity. They appear to be highly chemosensitive and cure may be obtained in the long-term in some cases. These data justify careful histological assessment in all cases of hepatomegaly of a tumoral nature.
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