Isochromosome i(3q) and premature chromosome condensation are recurrent findings in chronic B-cell lymphocytosis with binucleated lymphocytes.

Chronic B lymphocytosis with binucleated lymphocytes (LWBL) is a recently described entity. The lymphocytosis is stable over years and atypical binucleated lymphocytes are detected on peripheral blood smears. Further investigation has shown a predominance in females, a polyclonal increase in serum IgM and HLA-DR7 expression in most cases. In almost all cases cytogenetic studies with classical culture conditions have not shown any abnormality. We describe 7 patients with LWBL associated with an abnormal karyotype. An additional i(3q) was found in 6 cases. Premature chromosome condensation (PCC) was detected in all 7 cases. As both abnormalities are rarely present in other benign or malignant proliferations, we suggest a strong correlation between LWBL and i(3q) and/or PCC.

[Auto-dialysis: an 11-year experience of a hemodialysis center in France]. / L'autodialyse: une expérience de 11 ans d'un service d'hémodialyse de province.

OBJECTIVES: Report 11 years of experience with self-managed hemodialysis in patients medically apt for extra-hospital dialysis and living close enough to small outpatient hemodialysis units to become totally self-sufficient. RESULTS: Among the 276 patients with chronic renal failure managed at the hemodialysis center at the Troyes hospital during the 11-year study period from 1984 through 1994, self-managed hemodialysis at small outpatient units was initiated in 127 (46%). None of these 127 patients required medical assistance or specific care during dialysis sessions. At initial hospital admission, only 60/127 (47%) were totally self-sufficient: 52 (41%) were later graft recipients; and 21 (16.5%) had to return to the hospital for a medical or surgical condition incompatible with extra-hospital care but all of these 21 patients remained self-sufficient. CONCLUSION: Extra-hospital hemodialysis in units close to the patients residence offers patients a better quality of life, even when medical assistance is required. All patients who require hemodialysis can thus be treated at lower cost without compromising quality of treatment. Perfect self-sufficiency may not be a goal in itself, but self-managed hemodialysis can be a very useful technique for patients without major medical problems. Continuing contact with the organizing hemodialysis center guarantees the safety of the system.

[IgG kappa myeloma with Fanconi's syndrome and crystalline inclusions. Immunohistochemical and ultrastructural study]. / Myélome IgG kappa avec syndrome de Fanconi et inclusions cristallines. Etude immuno-histochimique et ultrastructurale.

An IgG kappa light-chain myeloma, remarkable for the presence of inclusions in plasmocytes and proximal renal tubular cells, was discovered during investigations for renal failure associated with partial Fanconi's syndrome. An immunohistochemical study showed that the crystalline inclusions were positive for IgG and kappa light-chain in plasmocytes and exclusively for kappa light-chain in the renal tubular cells. An ultrastructural study showed that the inclusions were localized in lysosomes and had a 55 A periodicity. This case is similar to the forty odd myelomas or light-chain diseases reported in the literature and revealed by Fanconi's syndrome. Their prognosis is good, with a slow progression towards late renal failure. In our patient the preference of lesions for proximal tubular cells, the site of protein catabolism, was particular since in myelomas they are usually located in the distal tubular cells.

[Repeated studies of the prevalence of Staphylococcus aureus in the nasal cavity in hemodialysed patients]. / Etude itérative de la prévalence du portage nasal de Staphylococcus aureus chez l'hémodialysé.

OBJECTIVES: In order to better establish a prevention strategy based on mupirocin, we evaluated nasal carriage of Staphylococcus aureus in haemodialysis patients over a 15 month period. METHODS: Search for Staphylococcus aureus in the nasal cavities was made every 2 months in 92 chronic dialysis patients. These patients were divided into 3 groups according to the nature of the carriage: non-permanent, intermittent or permanent. RESULTS: Among the 80 patients retained for analysis, there were 27.5% with intermittent carriage and 11.25% with permanent carriage. Factors which appeared to protect against carriage were rural residence and home self-dialysis. CONCLUSION: Repeated long-term search for nasal carriage of Staphylococcus aureus has provided reliable data for each patient and gave information on the effects of epidemiological conditions and health care structures.

[Nephrotic syndrome revealing malignant thymoma]. / Syndrome néphrotique révélant un thymome malin.

BACKGROUND: A well-established manifestation of neoplastic disease, nephrotic syndrome is infrequently associated with thymoma. Only 18 cases have been reported in the literature. CASE REPORTS: A 65-year-old man and a 60-year-old woman were seen for nephrotic syndrome. Minimal change renal disease was observed in the first patient whose nephrotic syndrome was steroid resistant. The second patient had membranous glomerulopathy and pure red cell aplasia. In both cases, nephrotic syndrome revealed thymoma. DISCUSSION: The histological lesions in 17 of the 18 biopsied cases reported in the literature were minimal change in 10, focal segmental glomerulonephritis in 4, proliferative glomerulonephritis in 2, and membranous glomerulopathy in only one. The outcome of the nephrotic syndrome was dependent on the success of the thymoma treatment. Some patients responded to steroid and immunosuppressive agents. Pure red cell aplasia is uncommon and prognosis is poor. It can be successfully treated with cyclosporin A as in our second case.

[Extramembranous glomerulonephritis of acquired syphilis in a patient recently infected by the hepatitis B virus. Demonstration of the treponemal antigen in the kidney by indirect immunofluorescence]. / Glomérulonéphrite extra-membraneuse de la syphilis acquise chez un malade récemment infecté par le virus de l'hépatite B. Mise en évidence au niveau du rein de l'antigène tréponémique par immunofluorescence indirecte.

A 38 years old male homosexual with active secondary syphilis presented with pure nephrotic syndrome while HBs and HBe tests were positive without clinical hepatitis. He had circulating immune complexes, IgG--IgM cryoglobulinemia and high IgA, IgM and IgE levels; the C3 and C4 complement constituents were normal. Examination of renal biopsy sections under light, fluorescent and electronic microscopy showed stage I membranous glomerulonephritis the syphilitic origin of which was confirmed by indirect immunofluorescence and by rapid cure under penicillin treatment. This case calls for the following comments: (1) glomerular deposits are extramembranous rather than subendothelial in syphilitic nephrosis, a disease now classified among circulating immune complexes diseases; (2) in the kidney, the treponema antigen can be demonstrated by indirect immunofluorescence and the anti-treponema antibody, by elution; (3) the outcome of the nephrotic syndrome is always favourable, either spontaneously or after penicillin treatment; (4) syphilis and HBs antigens are frequently associated, particularly in homosexual patients; one should be looked for when the other is discovered.

[Polyclonal lymphocytosis with binucleated lymphocytes. Morphological, immunological, cytogenetic and molecular analysis in 15 cases]. / Lymphocytose polyclonale avec lymphocytes sanguins binucléés. Analyse morphologique, immunologique, cytogénétique et moléculaire de 15 cas.

OBJECTIVES: Describe the clinical, immunological, cytogenetic and molecular aspects of polyclonal lymphocytosis with binucleated peripheral lymphocytes in order to ascertain the therapeutic consequences. PATIENTS AND METHODS: Fifteen patients, 13 women, 2 men, with a total lymphocyte count persistently above 4 x 10(9)/l at successive counts and binucleated lymphocytes on blood smears were studied. RESULTS: The syndrome is easily recognized at careful examination of blood smears which show binucleated lymphocytes and lymphoid cells with heterogeneous morphology. The lymphocytosis is polyclonal. The clinical situation remains stable without treatment despite cytogenetic clonality which contrasts with molecular polyclonality. DISCUSSION: Polyclonal lymphocytosis with binucleated lymphocytes is a clinical entity characterized by 1) nearly exclusive female predominance, mainly in young smokers; 2) lymphocyte counts above 4 x 10(9)/l which persist (> 6 months), are unchanged over time and fortuitously discovered; 3) lymphocytosis with binucleated lymphocytes on blood smears; 4) polyclonal lymphocytosis; 5) increased polyclonal serum IgM; and 6) recurrent cytogenetic anomalies with an isochromosome supenumerary marker, e.g. +i (3q). The etiology remains unknown: the role of smoking genetic predisposition or viral factors remains to be determined.

[Renal sarcoidosis with a pseudotumoral pyelic localization]. / Sarcoïdose rénale avec localisation pyélique pseudo-tumorale.

BACKGROUND: Renal sarcoidosis exceptionally presents as a unilateral pyelic pseudotumor as in this case where it was associated with granulomatous nephropathy. CASE REPORT: A 33-year-old man had a two-year history of systemic sarcoidosis with no renal involvement. He developed renal failure related to interstitial granulomatous nephropathy associated with a pyelic localization leading to unilateral hydronephrosis. Urine drainage associated with corticosteroid therapy provided a favorable course. DISCUSSION: Renal involvement in sarcoidosis is usually the consequence of hypercalcemia and hypercalciuria related to ectopic secretion of calcitriol by the sarcoidosic granulomas, with urinary lithiasis and nephrocalcinosis leading to renal failure and also granluomatous interstitial nephropathy. Glomerulopathy or obstructive nephropathy are rarely reported. Intraluminal localizations such as the pyelic lesion in our case are exceptional but must be detected early since they respond to corticosteroid therapy.

Routine diagnostic procedures of myelodysplastic syndromes: value of a structural blood cell parameter (NEUT-X) determined by the Sysmex XE-2100™.

INTRODUCTION: Diagnostic features of myelodysplastic syndromes (MDS) are often polymorphic and nonspecific including anemia in most cases. Standard parameters provided by an automated analyzer seldom bring any argument for this diagnosis. The aim of this study was to investigate whether some structural parameters, not routinely provided by Sysmex™ XE 2100 analyzer, could help diagnose MDS in a simple way, adapted to routine practice. METHODS: Blood samples from 184 MDS fully annotated cases and 3545 normal blood count controls were performed with XE 2100 Sysmex™ analyzer. Quantitative and structural parameters were considered. RESULTS: We found that the structural neutrophil parameter, NEUT-X, converted into a semi-quantitative parameter, the granularity index (GI), could be used as a flag for MDS in front of anemia. Negative GI and anemia were able to make otherwise unrecognized MDS stand out in routine practice, increasing the number of slides addressed to review from 67% to 96%, without leading to a large excess of unfounded slide review among non-MDS. CONCLUSION: Including the GI index in the routine parameters provided by the Sysmex analyzer could be of major help for nonspecialized routine laboratories in detecting MDS.