Peripheral blood stem cell mobilization in multiple myeloma comparison of two consecutive regimens in a limited resources country.

This study compared retrospectively the effectiveness, toxicity and hematopoietic recovery after autologous peripheral blood stem cell transplantation (ASCT) of two consecutive peripheral blood stem cell mobilization regimens in newly diagnosed MM patients. Patients in group 1 (n=178) were treated with 4 g/m2 of cyclophosphamide (CY) plus G-CSF (5 µg/kg/day). Patients in group 2 (n=117) with 750 mg/m2 of VP16 plus G-CSF (10 µg/kg/day). Optimal mobilization, defined by a target number of 8 × 106 CD34+ cells/kg collected, was achieved in 62.4% and 89.7% of patients in groups 1 and 2, respectively (P<10-4). The median number of aphaeresis sessions was reduced from two in group 1 to one in group 2 (P<10-4). Grade4 neutropenia, febrile neutropenia and IV antibiotic use were significantly more frequent in group 1 than in group 2 (P<10-4). Red blood cell transfusion requirements were significantly greater in group 1 (P=0.007). The switch to VP16-G-CSF10 resulted in a significant reduction of the number of hospitalization days (P<10-4). Neutrophil and platelet recovery after ASCT occurred on days 11 and 12, respectively, in the two groups with no significant differences. VP16+G-CSF10 allowed liberation of resources in the clinical and aphaeresis departments and demonstrated a better effectiveness-safety profile than CY+G-CSF5.

[Significance and contribution to the diagnosis of multiple myeloma according to the IGF1-R gene expression profile]. / Profil d'expression du gène IGF1-R dans le myélome multiple: signification et apport au diagnostic.

Multiple myeloma (MM) is a still incurable adult's severe hematologic malignancy. It is characterized by deregulation of several cytokines and their receptors. Among these cytokines, Insulin growth factor 1 (IGF1) and its receptor (IGF1-R) are well documented as major factor of malignant plasma cells growth and survival in multiple myeloma. The objective of this study was to analyze the expression of IGF1-R in multiple myeloma at diagnosis in correlation with clinical and biological data. IGF1-R gene plasma cells expression was studied in 47 patients and 17 controls by Taqman technology RT-PCR. IGF1-R gene was down expressed in the malignant plasma cells of MM patients at diagnosis compared to normal plasma cells, isolated from healthy donors (p = 0.01). Expression decrease was accentuated in the disease advanced stage IIIB. A negative correlation was found between IGF1-R malignant plasma cells expression and the percentage of bone marrow invasion (p = 0.03). Bone marrow infiltration greater than 30% was significantly associated with a low level of IGF1-R gene expression (p = 0.04). Our results suggest that the decreased expression of IGF1-R by malignant plasma cells is a prognostic factor associated with severe disease. Understanding of mechanisms involved in IGF1-R expression negative regulation may contribute to the discovery of new targets therapy in myeloma. the discovery of new targets therapy in myeloma.

[Identification of Enterocytozoon bieneusi by PCR in stools of Tunisian immunocompromised patients]. / Identification d'Enterocytozoon bieneusi par PCR dans les selles des patients immunodéprimés tunisiens.

INTRODUCTION: Intestinal microsporidiosis is recognised as an important cause of opportunistic parasitosis in immunocompromised patients, especially HIV-infected patients. Enterocytozoon bieneusi is the common causal agent. The diagnosis of intestinal microsporidiosis has usually based on microscopic detection of the spores of microsporidia species in stool samples, requires additional staining techniques as Modified Weber's trichrome stain. However, the detection of the spores can be difficult and species determination, which is important for defining the appropriate treatment, is impossible. Polymerase chain reaction (PCR)-based methods have been successfully used for detection of microsporidian infections. They are more sensitive and are able to identify microsporidia species. The purpose of this study is to identify E. bieneusi to adapt treatment and assess the true prevalence of the intestinal microsporidiosis due to this species in compromised patients in Tunisia. PATIENTS AND METHODS: One hundred and eighteen stools from immunocompromised patients, with a symptomatology in favour of the intestinal microsporidiosis, were analysed using light microscopy after staining with Modified Weber's trichrome stain and PCR. RESULTS: Only four were positive by Modified Weber's trichrome stain whereas eleven stools were positive by PCR, giving a prevalence of 20% in HIV-infected patients and 5,35% in human immunodeficiency virus-negative patients. CONCLUSION: This study confirms the usefulness of PCR in the diagnosis of the intestinal microsporidiosis due to E. bieneusi. Indeed, PCR has greater sensitivity than Modified Weber's trichrome stain and can identify the species of microsporidia in order to adapt the treatment.

[Epidemiological and clinical characteristics of intestinal microsporidiosis]. / Caractéristiques épidémiologiques et cliniques de la microsporidiose intestinale chez les immunodéprimés vus à Tunis.

INTRODUCTION: Intestinal microsporidiosis is an opportunistic parasitological infection affecting mainly immunocompromised patients, particularly those infected with HIV. PURPOSE: The purpose of this study was to analyse the epidemiological and clinical characteristics of intestinal microsporidiosis and the treatments available for it. MATERIAL AND METHODS: This retrospective study examined records collected over a 13-year period (from January 1995 through December 2007). It included 572 immunocompromised patients (279 HIV-infected patients and 293 without HIV infection) with symptoms suggesting intestinal microsporidiosis. All were tested systematically for microsporidia spores by modified (Weber's) Trichrome staining. RESULTS: Fourteen patients (10 men, 4 women) were diagnosed with intestinal microsporidiosis, for a prevalence of 2.4% overall, 3.6% in HIV-infected patients and 1.4% in those without HIV infection. Intestinal microsporidiosis affected 10 HIV-infected patients, 70% of whom had a CD4 count <100 cells/mm3. Their mean age was 30+/-15 years (range: 15 months to 48 years). The average age of HIV-infected patients (36 years) was significantly higher than of those without HIV infection (15 years). Thirteen patients had symptoms, most frequently diarrhea (11 cases), sometimes associated with dehydration (5 cases). Eight patients (57%) received only symptomatic treatment, and 4 (28.6%) received albendazole. No treatment was recommended in 2 cases (14.3%). Clinical course was marked by improvement in 6 cases, death in 5, and persistence of asymptomatic carriage in one. Two patients were lost to follow-up. CONCLUSION: Intestinal microsporidiosis is a parasitological disease that mainly affects AIDS patients with CD4 counts <100 cells/mm3. Its diagnosis requires special techniques. Its symptomatology is dominated by chronic diarrhea that can cause dehydration. Effective treatment requires identification of the species.

Monitorage de l'infection active a CMV par deux methodes : antigenemie PP65 et PCR qualitative sur plasma

Au cours de ce travail nous avons evalue deux methodes diagnostiques de l'infection active a CMV (antigenemie pp65 CMV et PCR qualitative) quant a leur utilite dans le monitorage de cette infection. Notre travail a porte sur 31 patients allogreffes de moelle osseuse dont 22 ont developpe une infection active a CMV. Le pourcentage des antigenemies positives etait de 59contre 28PCR positives. En mediane; les PCR se sont positivees 14 jours apres l'antigenemie. La duree de positivite de l'antigenemie etait de 5 semaines contre une semaine pour la PCR et la positivite de la PCR n'etait jamais isolee. Le taux de concordance de 61;5entre les deux techniques et le coefficient ( = 0.50) modere temoignerait d'un degre moyen de precision des resultats. La PCR sur plasma a presente une association plus importante avec la maladie a CMV (p= 0;014). La GVHD etait le seul facteur favorisant l'infection active a CMV (p=0;02). Cette derniere (p= 0;001) et la maladie a CMV (p= 0;04) ont presente des facteurs de mauvais pronostic chez nos patients. L'antigenemie constitue une methode de choix pour le diagnostic et le monitorage de l'infection active a CMV. Alors que la PCR qualitative sur plasma ne presente pas d'interet dans ce contexte

Allogeneic hematopoietic stem cell transplantation for acquired aplastic anemia using cyclophosphamide and antithymocyte globulin: a single center experience.

Between February 1998 and October 2007, 97 (69 male, 28 female) patients with acquired aplastic anemia and a median age of 18 years (range, 2-39) received related allogeneic hematopoietic stem cell transplantation. Ninety-five patients received bone marrow grafts and two patients G-CSF primed peripheral blood stem cell transplantation. The donors were genotypically HLA-identical siblings in 94 cases, HLA-matched parents in 2 cases and a syngeneic twin in 1 case. Median time from diagnosis to transplantation was 2 months (range, 1-15). Conditioning regimen consisted of cyclophosphamide combined with antithymocyte globulin in all patients. For graft versus host disease (GVHD) prophylaxis, all patients received methotrexate and cyclosporine. Eighty-six patients showed evidence of hematopoietic engraftment. Eight patients died before engraftment. Rejection rate was 14.8% with three primary graft failures and eight secondary graft rejections occurring between 2 and 27 months post transplantation. Of the 11 rejecting patients, 3 died from infection and 8 proceeded to a second transplantation. Among the eight patients re-transplanted, seven are alive with successful second engraftments and one died from acute grade III GVHD. Acute GVHD occurred in 15.5% and extensive chronic GVHD in only 5.3% of patients. The 4-year overall probability of survival was 76.8%. Infection was the cause of 81.1% of deaths. The major factor affecting survival was onset of infection before transplantation. Major ABO donor-recipient incompatibility, disease severity and acute GVHD had also negative impact on survival. These results could be improved by reducing the time to transplant and by a more efficient supportive care policy.Bone Marrow Transplantation advance online publication, 27 July 2009; doi:10.1038/bmt.2009.175.

Allogeneic hematopoietic stem cell transplantation in Tunisia.

In 1998, the Tunisian team of the 'Centre National de Greffe de Moelle Osseuse' initiated allogeneic hematopoietic SCT (AHSCT) in Tunisia. As of June 2007, information was collected about 299 patients with a first AHSCT and 12 additional retransplants. The median age was 19 years (range 2-49 years). The main indications were aplastic anemia (n=106, 36%), leukemia and nonmalignant disorders (n=153, 51%), Fanconi anemia (n=26, 9%) and other nonmalignant disorders (n=14, 4%). Preparative regimens depended on indication. All donors were HLA geno-identical. The stem cell sources were BM (87%) and PBSCs (13%). At the time of analysis, 200 patients (67%) were alive after a median follow-up of 42 months (range 3-112 months). The overall TRM rate was 17%. Outcome depended on indication. According to our results, allogeneic HSCT is potentially curative for hematological diseases, but it is a toxic approach for malignant disorders.

Detection of catheter-related bloodstream infections by the Gram stain-acridine orange leukocyte cytospin test in hematopoietic stem cell transplant recipients.

In patients with central venous catheters (CVCs), catheter-related bloodstream infections (CRBI) are a prominent cause of morbidity, excess hospital costs, and in some cases mortality. The aim of this prospective study was to assess the validity of the Gram stain-acridine orange leukocyte cytospin (AOLC) test for the diagnosis of CRBI in hematopoietic stem cell transplant (HSCT) recipients with nontunnelled CVCs, using the differential-time-to-positivity (DTP)/clinical criteria as the criterion standard to define CRBIs. CVCs were externalized, nontunnelled, polyurethane double lumen catheters (Arrows, Readings, USA). All CVCs were placed in the subclavian vein by the infraclavicular approach, in the operating room. Catheters were inserted percutaneously, using the Seldinger technique. Study catheters were not exchanged over guidewires. Between May 2002 and December 2004, a total of 245 consecutive patients were included. Twenty-six of the 245 patients (10.6%) had CRBI as determined by the DTP method. The Gram stain-AOLC was positive in only two patients (7.6%) with a CRBI. Our results suggest that the Gram stain-AOLC test is not useful for the diagnosis of catheter-related bloodstream infection in HSCT recipients.2006.

Prospective randomised comparison of the COBE spectra version 6 and haemonetics MCS(+) cell separators for hematopoietic progenitor cells leucapheresis in patients with multiple myeloma.

A randomised crossover trial of two separators was undertaken to compare the mononuclear cell, CD34(+) cell and CFU-GM yield, in patients (<61 years) with previously untreated symptomatic multiple myeloma. After first-line therapy, all patients received mobilising chemotherapy (cyclophosphamide 4 g/m(2)) and daily G-CSF. The first leucapheresis was performed on the first day the peripheral blood absolute CD34(+) cell count was > 20 cells/microl. All patients underwent 2 leucaphereses on consecutive days. The patients were randomised to undergo either the first or second leucapheresis using the COBE Spectra. The target duration of the procedure on the COBE Spectra was 2 total blood volumes, and for the Haemonetics MCS(+) it was 20 cycles with four recirculations. Between September 2003 and March 2005, 60 patients were entered in the study. COBE Spectra version 6 processed significantly larger volumes of blood than the Haemonetics MCS(+) (8,845 and 5,680 ml, respectively, P < 0.01). The absolute yield of mononuclear cells (2.1 vs. 1.5 x 10(8)/kg, P = 0.04), CFU-GM (11 vs. 3 x 10(4)/kg, P = 0.01) and CD34(+) cells (3 vs. 1.7 x 10(6)/kg, P = 0.02) were all significantly higher with the COBE Spectra version 6, as were the yields per unit volume of blood processed. In conclusion, our study shows that COBE Spectra Version 6 is faster and has a better yield than the Haemonetics MCS(+), in patients with multiple myeloma.

Bone marrow transplantation from matched related donors for patients with Fanconi anemia using low-dose busulfan and cyclophosphamide as conditioning.

Seventeen patients with Fanconi anemia (FA) underwent allogeneic bone marrow transplantation (BMT) from matched related donors (MRD) between January 1999 and June 2003. Median age at BMT was 11 years. Conditioning regimen consisted of low-dose cyclophosphamide (CY; 40 mg/kg) and busulfan (BU; 6 mg/kg) with the addition of lymphoglobulin (20 mg/kg) in two patients. Graft-versus-host disease (GVHD) prophylaxis included cyclosporine A (CsA) and methotrexate (MTX; 5 mg/m(2) at day 1, 3, 6). All patients engrafted (for an absolute neutrophil count >0.5 x 10(9)/L) after a median time of 12 days (range 10-16 days). Fourteen patients (82%) had sustained grafts, whereas three others (18%) rejected grafts between day +39 and +80 after transplantation. Two of them are still alive after successful second PBSC transplantation and one died. Acute and chronic GVHD occurred in 23% and 13% of patients, respectively. With a median follow-up of 16 months (range 3-53 months), survival rate was 72% and Karnofsky score was at least 90%. The low-dose BU/CY regimen, in FA patients allografted from an HLA-matched related donor, allowed engraftment with relative low toxicity. Early graft failure (GF) remains a problem and may require modification of this regimen.

Prevalence of inherited prothrombotic abnormalities and central venous catheter-related thrombosis in haematopoietic stem cell transplants recipients.

In this prospective study, we assessed the incidence of central venous catheter (CVC)-related thrombosis in haematopoietic stem cell transplant (HSCT) recipients. We determined the contribution of inherited prothrombotic abnormalities in blood coagulation to CVC-related thrombosis in these patients. The study was conducted between May 2002 and September 2004. CVCs were externalized, nontunneled, polyurethane double lumen catheters. Before catheter insertion, laboratory prothrombotic markers included factor V Leiden, the prothrombin gene Gly20210A mutation, plasma antithrombin levels, and protein C and S activity. All patients were systematically examined by ultrasonography just before, or <24 h after, catheter removal, and in case of clinical signs of thrombosis. A total of 171 patients were included during the 28-month study period. Five (2.9%) and three (1.7%) patients had evidence of protein C and protein S deficiency, respectively. Only one patient had an antithrombin deficiency (0.6%). In total, 10 patients (5.8%) were heterozygous for the factor V Leiden mutation, and one patient had heterozygous prothrombin G20210A mutation (0.6%). We observed a CVC-related thrombosis in 13 patients (7.6%). Thrombosis was diagnosed in four out of 20 patients (20%) with a inherited prothrombotic abnormality compared to nine of 151 patients (6%) who did not have a thrombophilic marker (relative risk 3.3 CI 95% 1.1-9.9). Our results suggest that inherited prothrombotic abnormalities contribute substantially to CVC-related thrombosis in HSCT recipients. In view of physicians' reluctance to prescribe prophylactic anticoagulant treatment in these patients, a priori determination of inherited prothrombotic abnormalities may form a basis to guide these treatment decisions.

First-line thalidomide-dexamethasone therapy in preparation for autologous stem cell transplantation in young patients (<61 years) with symptomatic multiple myeloma.

Thalidomide-dexamethasone therapy was given in patients (<61 years) with previously untreated symptomatic multiple myeloma. The aim of this study was to assess the efficacy and toxicity of this combination as first-line therapy, and to determine its effect on stem cell collection and engraftment. During first-line therapy, thalidomide and dexamethasone were administered for 75 days (200 mg/day) and 3 months, respectively. The monthly dose of dexamethasone was 20 mg/m2/day for 4 days, with cycles repeated on days 9 to 12 and 17 to 20 on the first and the third month of therapy. After first-line therapy, a collection of peripheral blood stem cells (PBSC) was performed. Between May 2003 and September 2004, 60 patients were included. On an intent-to-treat basis, the overall response (> or =partial response) rate was 74%, including 24% of patients who obtained a complete remission. Grade 3-4 toxicities consisted of infections (12%), deep-vein thrombosis (3%), constipation (5%), and neuropathy (5%). A total of 58 patients (96%) proceeded to PBSC mobilisation and yielded a median number of 8 x 10(6) CD34+ cells/kg. First-line thalidomide-dexamethasone therapy is effective and relatively well tolerated in young patients with symptomatic multiple myeloma. This combination does not affect PBSC mobilisation.

Difference in time to positivity is useful for the diagnosis of catheter-related bloodstream infection in hematopoietic stem cell transplant recipients.

Catheter-related bloodstream infections are associated with recognized morbidity and mortality. Accurate diagnosis of such infections results in proper management of patients and in reducing unnecessary removal of catheters. We carried out a prospective study in a bone marrow transplant unit to assess the validity of a test based on the earlier positivity of central venous blood cultures in comparison with peripheral blood cultures for predicting catheter-related bacteremia. Between May 2002 and June 2004, 38 bloodstream infections with positive simultaneous central venous catheter and peripheral vein blood cultures were included. A total of 22 patients had catheter-related bacteremias and 16 had noncatheter-related bacteremias, using the catheter-tip culture/clinical criteria as the criterion standard to define catheter-related bacteremia. Differential time to positivity of 120 min or more was associated with 86% sensitivity and 87% specificity. In conclusion, differential time to positivity of 120 min or more is sensitive and specific for catheter-related bacteremia in hematopoietic stem cell transplant recipients who have nontunnelled short-term catheters.

Rejection of the second allogeneic graft in a child with Fanconi anemia reversed by antilymphocyte globulin and donor lymphocyte infusion.

Rejection after allogeneic bone marrow transplantation for Fanconi anemia (FA) is a complication with a high risk of mortality. We describe a patient who, following a second episode of rejection after a second allogeneic stem cell transplantation, was successfully treated with antilymphocyte globulin, followed by donor lymphocyte infusion. At three and a half years after donor lymphocyte infusion, she is alive with a Karnofsky score of 90%. Her molecular chimerism is of donor origin. Thus, donor lymphocyte infusion can be considered as a therapy option for rejection after allogeneic bone marrow transplantation for FA.

[Infectious complications related to non-tunneled central venous catheters in immunocompromised patients: prospective study about 210 cases]. / Les infections liées aux cathéters veineux centraux non tunnélisés chez des patients immunodéprimés : étude prospective à propos de 210 cas.

OBJECTIVE: To evaluate infectious complications related to non-tunneled central venous catheter in immunocompromised patients, in a bone marrow unit. METHODS: From July to April 2002, we inserted 210 non-tunneled central venous catheters in 139 immunocompromised patients (52 F/87 M). The mean age was 26 years (3-56 years). Our study included 33 children aged from 3 to 15 years, on whom 46 catheters were placed. The catheters were placed for the following indications: 145 catheters were used in subjects who received a bone marrow transplantation, 58 catheters were placed in subjects who received chemotherapy for acute leukemia and seven catheters were used in patients who received immunosuppressive therapy. RESULTS: The mean duration of catheterization was 33 days (7-114 days). There were 3.1 catheter-related infections per 1000 catheter-days. Coagulase-negative Staphylococci were implicated in 64% of cases. We observed two pneumothorax (0.9%), one arterial puncture (0.4%) and two catheter-related thrombosis (0.9%). CONCLUSION: Non-tunneled catheters in immunocompromised patients (adults and children) is a safe technique, and is an alternative to the Hickman catheters which are most widely used today in patients undergoing bone marrow transplantation.

Proinflammatory cytokines and their role in the development of major transplant-related complications in the early phase after allogeneic bone marrow transplantation.

Serum levels of interleukin-6 (IL-6), interleukin-8 (IL-8) and tumor necrosis factor (TNF)-alpha were frequently measured during the first 30 days after allogeneic bone marrow transplantation (BMT) in 84 consecutive adult patients. Major transplant-related complications (MTCs) occurred in 33% of cases and included veno-occlusive liver disease, idiopathic pneumonia syndrome, severe endothelial leakage syndrome and >grade II acute graft-versus-host disease. Compared with patients having minor complications, those with MTCs developed higher levels at times of maximal clinical signs (all cytokines, P<0.001), between days 0-5 post-BMT (IL-6 and IL-8, P<0.05) and days 6-10 (L-6, P<0.001; IL-8 and TNF, P<0.01) post-BMT. We could not discriminate patterns of cytokine release that were specific for any subtype of MTC. Higher levels of IL-8 during days 0-5 were associated (P=0.044) with early (<40 days) death. Multivariate analysis including patient and transplant characteristics as well as post-BMT levels of C-reactive protein showed that high average levels of one or more of the cytokines within the first 10 days post-BMT were independently associated with MTC (Odd's ratio: 2.3 [1.2-4.5], P=0.011). This study shows that systemic release of proinflammatory cytokines contributes to the development of MTC and provides a rationale for pre-emptive anti-inflammatory treatment in selected patients.

The impact of partial T cell depletion on overall transplant-related toxicity, graft function and survival after HLA-identical allogeneic bone marrow transplantation in standard risk adult patients with leukemia.

In this single-center study, a consecutive cohort of 59 adult patients transplanted with HLA-identical bone marrow and receiving graft-versus-host disease (GVHD) prophylaxis with either standard cyclosporine/methotrexate (n = 33) or partial T cell depletion (E-rosetting) (TCD, n = 26 were analyzed). Only patients with chronic myeloid leukemia in first chronic phase or acute leukemia/myelodysplasia in first or second remission were included. Except for age (median 28 vs 42 years), both groups were comparable in terms of diagnosis, conditioning regimen and growth factor support. TCD significantly reduced >grade II acute GVHD (0 vs 24%, P = 0.02), chronic GVHD (8.5 vs 45%, P = 0.007) and other major bone marrow transplant (BMT)-related complications (4 vs 36%, P = 0.005). TCD decreased overall transplant-related mortality (11.5 vs 36%, P = 0.04). In the TCD group faster neutrophil (13 vs 22 days, P = 0.02) and platelet recoveries (18 vs 26 days, P < 0.001) were noted. The relapse risk was higher after TCD (57.5 vs 21.5%, P = 0.04). Overall survival probability at 10 years was identical in both groups (54 vs 53.5%, P = 0.33). We found a relationship between the number of T cells in the graft and the occurrence of major complications (P < 0.001) and relapse (P = 0.03). This comparative analysis shows that graft-derived T cells have a major role in overall BMT-related toxicity and that partial TCD is an acceptable approach in terms of survival for patients between 40 and 50 years of age.

[Atypical defibrination syndromes and acute leukemias with a t(9,22) translocation, apropos of 2 cases]. / Syndromes de défibrination atypiques et leucémies aiguës à translocation t(9,22); à propos de deux observations.

We report two cases of atypical defibrination syndromes in patients with respectively acute monoblastic leukemia (chronic myeloid leukemia initially) and acute lymphoblastic leukemia. Hemostasis studies show low fibrinogen level, elevated D-dimers, decreased alpha 2 antiplasmin and factor V, normal antithrombin III values. Plasminogen is below the normal range in one patient. Soluble complexes, which are an important argument for diagnosis of intravascular coagulation disease, are not detected in both patients. Primary or secondary hyperfibrinolysis seems also excluded since euglobulin clot lysis time was normal. Enzymatic proteolysis of fibrinogen (or fibrin) by the blast cells has been reported by some authors; this mechanism could account for the hemostasis abnormalities observed in these two patients.

[Hairy cell leukemia: report of 8 cases]. / Leucémie à tricholeucocytes à propos de 8 cas.

Hairy cell leukemia haemopathy is a rare lymphoïd haemopathy type B. 8 cases are reported and diagnosed at Hôpital Aziza Othmana over a period of 20 years between 1979 and 1999, 7 men and one women. The mean age of the patients is 51 years, with externe ages from 42 to 81 years. 4 patients consulted for an infections and, or anaemia syndrome. The disease was revealed due to the presence of an isolated splenomegaly in other cases. At the clinical examination, the spleen is hypertrophied in 7 patients out of 8. Pancytopenia is observed in 50% of the patients. Only one patient has presented a moderated hyperleukocytosis at 11,000/mm3 related to the presence of moving on tricholeukocytes. The myelogramme is pocr. It allowed to mention the diagnosis in 6 cases out of 8. Bone Marrow biopsy revealed a diffuse infiltration by TCL with a reticulinic fibrosis in all patients. 4 patients out of 8 have been splenectomized. Cytopenies have been corrected in all patients. Only one patient has been treated by alpha Interferon for 3 years with a partial hematological response. A relapse was observed once the Interferon was stopped. With the introduction of new drugs such purine analogues. The HCL treatment has been revolutionarized thanks to the improvement of the rate of complete response (from 10% to 80% of CR). If splenectomy is still observed in HCL for splenomegalic and or severe cytopenia, our findings could be improved thanks to new purine analogues.