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.

Cord blood volume reduction using an automated system (Sepax) vs. a semi-automated system (Optipress II) and a manual method (hydroxyethyl starch sedimentation) for routine cord blood banking: a comparative study.

Background With the development of cord blood banking, solutions have to be found to solve the storage space problem, by reducing the volume of cord blood units (CBU). Methods We compared total nucleated cell (TNC) and CD34(+) cell counts before and after processing with three different CBU volume reduction methods used consecutively in our bank: a manual method based on hydroxyethyl starch sedimentation (HES) (n=447), a top-and-bottom (TB) semi-automated method (n=181) using Optipress II, and the Sepax automated method (n=213). Statistical analysis was done using t-tests, linear regression and Spearman correlation coefficients. Adjusted variables included TNC, CD34(+) cell counts, CD34(+) cell percentage and CB volume before processing. Results TNC recovery was higher with Sepax (80.3+/-7.7%) than with HES (76.8+/-9.1%) and TB (60.7+/-13.5%) (P<0.0001, both). It was higher with HES than with TB (P<0.0001). CD34(+) cell recovery was higher with Sepax (86+/-11.6%) than with HES (81.5+/-12.5%) and TB (82.0+/-17.7%) (P<0.008 and <0.0001, respectively) and results with HES and TB were not significantly different (P=0.7). Interestingly, with Sepax, TNC and CD34(+) cell recoveries were not correlated with pre-processing values (P=0.8 and 0.4, respectively). Discussion In conclusion, the Sepax volume reduction method allows higher TNC and CD34(+) cell recoveries.

[Drug induced linear IgA bullous dermatosis]. / Dermatoses bulleuses à IgA linéaire d'origine médicamenteuse.

Linear IgA disease is an autoimmune subepidermal bullous disease in which linear IgA deposits are found at the basement membrane zone. It is classically idiopathic but a drug-induced variant seems to be individualized in which cutaneous lesions resolve spontaneously after cessation of responsible treatment. Among the commonly implicated drugs, vancomycin is the most frequently reported. One should not however ignore other precipitating events sometimes associated, particularly infectious diseases and non-lymphoid or lymphoproliferative malignancies. Authors present here clinical and histological features of this disease as well as drugs that have been implicated.

[Prevention of bacteremia from central venous catheters in hemodialysis: value of application of a mixture of rifampicin and protamine at the insertion site]. / Prévention des bactériémies liées aux cathéters veineux centraux en hémodialyse: intérêt d'un soin du site d'insertion par un mélange de rifampicine et protamine.

During the last 8.5 years, authors observed only 3 bacteremias out of 249 catheters in place in different sites corresponding to a total of 10,063 days with central venous catheterization in hemodialysis (0.30/1000 catheter days). This good result seems to be correlated mainly with the preventive application, on catheter insertion-site, of a rifampin and protamine sulphate mixture. This protocol appears worthy to be known because, at the present time, whatever the new preventive strategies, infection rates reported in the literature are generally more consequent and it is necessary to consider all the possibilities of decreasing them.

[Value of isotretinoin in the treatment of acne in hemodialysis patients: apropos of 1 case]. / Intérêt de l'isotrétinoïne dans le traitement de l'acné de l'hémodialysé(e): à propos d'une observation.

Rare are reports about isoretinoin treatment for acne in dialysis or kidney transplant patients whereas its efficacy and safety make it very interesting. Authors report here an additional observation of a 32 year-old young woman, hemodialyzed after one period of 12 years renal transplantation, and presenting a diffuse and severe acne. Treatment by isotretinoin with a moderate amount of 20 mg (0.38 mg/kg) per day cures this dermatosis in 2 months and half, without severe clinical side effect or significant metabolic disturbance, apart from a transient increase in parathormone rate.

[Colonic necrosis caused by sodium polystyrene (kayexalate) in hemodialysis: myth or reality? Two case reports]. / La nécrose colique due au polystyrène de sodium (Kayexalate) chez le dialysé: mythe ou réalité? A propos de deux observations.

Among gastro-intestinal complications reported in uremic or transplanted patients, colonic necrosis due to Kayexalate and sorbitol is not so unusual for english authors. This iatrogenic event is probably underestimated by physicians and pathologists. In France, neither literature nor pharmacovigilance refer this complication but Vidal dictionary advises against such association. We thought of interest to report 2 representative observations and, after reviewing literature, to discuss diagnostics circumstances and etiopathogenic hypothesis and then to give some advices.

[Pulmonary migration of an axillary stent in a chronic hemodialysis patient]. / Migration pulmonaire d'une endoprothèse axillaire chez une hémodialysée chronique.

The short-lived results of angioplasty for venous stenosis of a dialysis access led to stent placement. Its migration is a rare but dangerous complication, because of its final destination, that is right heart or pulmonary artery. We report on such a case and compare it with three cases found in literature.

[Severe Schonlein-Henoch nephritis in adults. A report of twenty cases]. / Les néphropathies sévères du purpura rhumatoïde de l'adulte. A propos de vingt observations.

Twenty patients with severe Schönlein-Henoch nephritis were selected on a histologically basis of diffuse proliferative endo- and/or extracapillary glomerulonephritis during a period of 12 years in Champagne-Ardenne. There were 15 men and 5 women, mean age 44.1 years. An infectious history was found in 40%, an urinary tract cancer in 15%. In all cases there was purpura, in 80% joint pain and in 50% digestive symptoms. Clinical presentation at diagnosis included, in all cases, hematuria (gross in 50%) and proteinuria (of nephrotic range in 80%); there was hypertension in 60% and renal failure in 80%. Histology found, in all cases, mesangial IgA and often C3 deposits, with a diffuse endocapillary proliferation in 10%, extra-capillary proliferation in 30% and both endo-extracapillary in 60%; 45% of the patients had crescents in greater than 50% of glomeruli. The outcome, after steroid and immunosuppressive treatment, was end-stage renal failure in 25%, moderate renal failure in 20%, or normal renal function in 55% with a mean follow-up period of 4.6 years. These severe nephritis were associated with repetitive and often necrotic purpura, frequent joint pain and severe digestive symptoms. The analysis of initial renal presentation confirmed the bad prognosis of nephrotic syndrome, renal failure and especially hypertension, which were well correlated with the severity and diffusion of proliferative lesions. Despite a worse known prognosis, these nephritis responded to an aggressive and early treatment.

[Midodrine (Gutron) treatment of permanent arterial hypotension in a chronic hemodialysis patient]. / Traitement par midodrine (Gutron) de l'hypotension artérielle permanente d'un patient hémodialysé chronique.

Chronic hypotension, although unfrequent in uremic patients on hemodialysis, accentuates the deterioration of patients physical state and thus, their general well-being. These patients often experience acute intradialytic symptoms and respond very poorly to conventional therapies. Well tolerated, midodrine is a suitable and effective choice as it raises blood pressure significantly through its effect on peripheral alpha-adrenergic receptors. The authors report observing the use of midodrine by a dialysis patient during the longest time period published to date, documented by a pharmacokinetic study, and that confirms the excellent results and proves long term tolerance for that drug.

[Value of an end-stage renal failure registry. Experience of the Champagne-Ardenne region]. / Intérêt d'un registre des insuffisants rénaux terminaux. Expérience de la région Champagne-Ardenne.

Since 1992, a registry gathering all the patients with end-stage renal failure is established in Champagne-Ardenne. It is based upon demographic data and allocation of dialysis treatment. It is supported by regional nephrologists. It is exhaustive and has been validated by the regional health administration. It is the only data base considered for the regional scheme of sanitary organization. In 1999 the incidence of terminal renal failure was 119 pmp and, at 1 January 2000, prevalence of uremic patients treated with supportive therapy, except transplantation, was 426 pmp. Our registry may be used as a regional registry awaiting to participate into built the national program project REIN (Renal Epidemiology and Information Network) and to help better health care policy and costs containment.

[Severe renal forms of leptospirosis. Apropos of 6 cases seen in 15 years at one center]. / Les formes rénales graves de la leptospirose. A propos de six cas recueillis en quinze ans dans un même service.

We report on six cases of leptospirosis observed in fifteen years in our department of nephrology. The contamination is two times from professional origin and four times linked to water leisures, during hot season, from june to september for the majority. Two serotypes are found: L. icterohaemorragiae characterized by marked jaundice, and L. patoc with anicteric form. Acute renal failure, five times oliguro-anuric, necessitates in the six cases hemodialysis (two to six sessions). Meningitis is found in four patients, associated in one case to an encephalitis and a polyradiculoneuritis. In one case, there were interstitial pulmonary lesions, in one another, a myocarditis with conduction disorders, responsible of the only death of this series, and finally, in three cases, a gastrointestinal hemorrhage. Bacteriological diagnosis seems little profitable and microagglutination remains the reference technique, helped by the IgM detecting through ELISA, looking forward to the evaluation of PCR (Polymerase Chain Reaction).

[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.

Persistent polyclonal B lymphocytosis with binucleated lymphocytes: a study of 25 cases. Groupe Français d'Hématologie Cellulaire.

Persistent and polyclonal lymphocytosis of B lymphocytes (PPBL) with binucleated lymphocytes is an entity characterized by a polyclonal lymphocytosis. The lymphocytosis is stable for years and binucleated lymphocytes are detected on peripheral blood smears. We previously described +i(3q) as a recurrent chromosomal abnormality in seven PPBL patients. In this study we report a large series of 25 PPBL patients and demonstrated that PPBL was associated with +i(3q) in 77% of cases, premature chromosome condensation (PCC) in 50% and both abnormalities in 41% of cases. Furthermore, we demonstrated that i(3q) was present in a minority of B cells, restricted to B lymphocytes independently of the kappa or lambda light Ig chain expression, and exclusively observed in non-binucleated cells. The benign clinical course of PPBL and the lack of biological evolution in the majority of cases suggest that recognition of these disorders is so important that aggressive therapy in PPBL has to be avoided. Whether this syndrome represents a premalignant or benign disease remains unclear. The persistence of cytogenetic abnormalities after stopping tobacco use suggests no association with cigarette smoking.

[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.

[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.

Indwelling silicone femoral catheters: experience of three haemodialysis centres.

The aim of this study is to describe the experience of three haemodialysis centres using indwelling femoral silicone catheter (model SSL 1220M, Medcomp, USA) in 55 patients, three with acute renal failure, one requiring plasmapheresis, and 51 with chronic renal failure but no other available vascular access. Sixty-four catheters were in place for a mean duration of 41.5 +/- 30 days. The rate of catheter-related complications, including mechanical problems, thromboses, and infections was low and they were never life-threatening. The results of the study suggest that femoral cannulation with modern flexible devices can be considered as a reliable temporary access, even for extended periods, with advantages exceeding those for subclavian and jugular routes.