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Hairy Cell Leukemia (hairy + cell_leukemia)
Selected AbstractsCD52 expression in hairy cell leukemiaAMERICAN JOURNAL OF HEMATOLOGY, Issue 4 2003Michael M. Quigley Abstract Hairy cell leukemia (HCL) is a rare chronic B-cell lymphoproliferative disorder characterized by splenomegaly, pancytopenia, and circulating atypical lymphocytes with circumferential cytoplasmic projections. Although uncommon, HCL cases refractory to standard therapy occur, and effective alternatives are limited. There is evolving literature supporting monoclonal antibody therapy in the treatment of B-cell lymphoid malignancies, including anti-CD52 (Campath-1H, alemtuzumab). We have examined nine cases of HCL and one case of HCL variant by flow cytometry for CD52 expression. All cases expressed CD52 antigen in 92,100% of the malignant cells. The demonstration of CD52 antigen expression on HCL cells provides the rationale for the use of alemtuzumab in refractory HCL. Am. J. Hematol. 74:227,230, 2003. © 2003 Wiley-Liss, Inc. [source] The value of tartrate resistant acid phosphatase (TRAP) immunoreactivity in diagnosis of hairy cell leukemia,APMIS, Issue 3 2005HAMPAR AKKAYA Hairy cell leukemia (HCL) is a rare chronic B-cell lymphoproliferative disorder characterized by splenomegaly, pancytopenia, and circulating atypical lymphocytes with circumferential cytoplasmic projections. We investigated the specificity and the sensitivity of anti-TRAP antibody immunoreactivity in 57 cases of HCL. We found that there is a statistically highly significant difference between TRAP immunoreactivities of the study and the control groups, and HCL can be diagnosed by TRAP immunoreactivity in bone marrow trephine biopsy materials with a specificity of 98.27 % and a sensitivity of 100%. [source] Risk factors for severe infection in patients with hairy cell leukemia: a long-term study of 73 patientsEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 3 2009Ghandi Damaj Abstract Although the survival of patients with hairy cell leukemia (HCL) has been improved by the therapeutic introduction of interferon , and purine analogs, it is still worsened by complications such as severe infections. In this long-term study, we identified factors influencing patient outcomes in 73 patients with HCL. Median age at diagnosis was 53 yr and the gender ratio (M/F) was 2.3. At the time of HCL diagnosis, 60 patients (82%) were symptomatic and 22 of these had an infection. After a median follow-up of 13 yr, eight patients had died of secondary cancer (n = 2), HCL progression (n = 1) and age-related complications (n = 5). The 10-yr overall survival (OS), progression-free survival and relapse rates were 91 ± 3%, 14 ± 5% and 87 ± 5%, respectively. In multivariate analyses, age >53 yr was the only factor adversely influencing OS and secondary cancer incidence, with adjusted hazard ratio (HR) of 9.30 (95%CI, 1.15,76.6; P = 0.037) and 2.80 (95%CI, 1.05,7.71; P = 0.04), respectively. Eleven patients developed severe infections. Absolute lymphocyte count (<1 × 109/L) at diagnosis was the only factor influencing the occurrence of severe infections, with an adjusted HR of 4.01 (P = 0.007). Strikingly, we did not observe any significant correlation between neutrophil or monocyte counts and the incidence of infection. We confirmed long-term survival in HCL but found a high incidence of infection , even late in the course of the disease. The absolute lymphocyte count at diagnosis is a risk factor for the occurrence of severe infections. In addition to careful monitoring of infections, prompt initiation of anti-HCL treatment should be considered in patients with low lymphocyte counts. [source] A population-based study of hairy cell leukemia in IsraelEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 5 2006Ora Paltiel Abstract:,Objectives: Few population-based data exist on the incidence and prognosis of hairy cell leukemia (HCL). Our objectives were to study the effect of socio-demographic factors on this rare disease and the risk of second malignancies occurring in HCL patients. Methods: We measured crude and age-adjusted incidence rates of HCL based on reporting to the Israel Cancer Registry (ICR) 1991,2001. Using Kaplan,Meier and multivariate analysis, we assessed survival by gender, ethnicity and geographic region. We ascertained additional primary tumors reported in this population and calculated standardized incidence ratios (SIRs) for tumors reported after the diagnosis of HCL. Results: The ICR registered 147 cases of HCL among males and 34 in females between 1991 and 2001. Age-adjusted incidence rates were 1.62/106/yr for women and 7.97/106/yr for men, with rates 1.5 times higher in Jewish than in non-Jewish (mainly Arab) men. Mean overall survival also differed by ethnicity. In a multivariate model, increasing age at diagnosis (P < 0.001), as well as Arab origin (P = 0.008) were associated with poorer survival but gender did not significantly affect the survival after controlling for age and ethnicity. Other primary malignancies were reported in 20 (11%) individuals, with a predominance of genito-urinary tumors (65%) among males. Secondary genito-urinary tumors were significantly increased above the expected population rates (SIR 3.23, 95% confidence interval: 1.39,6.36, P = 0.008). Conclusions: In the Israeli population, age and ethnicity were associated with prognosis of HCL. Variations in disease characteristics, stage of disease at diagnosis or differential access to treatment may contribute to these findings. Patients with HCL appear to be at increased risk for genito-urinary malignancies. [source] Treatment of late-stage Sézary syndrome with 2-ChlorodeoxyadenosineINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 6 2002Saskia A. Bouwhuis MD Background, 2-Chlorodeoxyadenosine (2-CdA), a purine adenosine analog, is safe and effective chemotherapy for patients with hairy cell leukemia and low-grade lymphomas. Adverse effects include neutropenia, lymphocytopenia, and infectious complications. Our objective was to evaluate the efficacy of 2-CdA (2,6 seven-day cycles) in the treatment of late-stage, recalcitrant Sézary syndrome. Methods, Retrospective review of medical records of six patients with Sézary syndrome who had received 2-CdA cycles at Mayo Clinic, Rochester between March 1995 and March 2000. Variables assessed from the records included improvement in global appearance, extent of erythroderma, size of lymph nodes, pruritus, and leukocyte, lymphocyte, and absolute Sézary cell counts. Results, Two patients, both with stage III Sézary syndrome, whose previous treatment consisted of only two modalities, responded well to the treatment, with moderate to total clearing of erythroderma and pruritus associated with a significant decrease in Sézary cell counts. The other four patients had only a partial response (one patient) or no response (three patients) to 2-CdA. The mortality rate was 50%. All three patients died of Staphylococcus aureus sepsis. However, only one patient was receiving 2-CdA treatment when he died. The other two patients died 8 and 9 weeks after the last 2-CdA cycle. This high mortality rate is attributed to infectious complications after 2-CdA treatment in patients with recalcitrant disease. Conclusion, 2-Chlorodeoxyadenosine shows efficacy in stage III Sézary syndrome, but it also carries a substantial risk of septic complications and mortality. It can be used if no other suitable alternatives are available. Caution should be exercised in all these patients regarding skin care and avoidance of infections or sepsis. [source] Identification of uniquely expressed transcription factors in highly purified B-cell lymphoma samples,,§AMERICAN JOURNAL OF HEMATOLOGY, Issue 6 2010Ulrika Andréasson Transcription factors (TFs) are critical for B-cell differentiation, affecting gene expression both by repression and transcriptional activation. Still, this information is not used for classification of B-cell lymphomas (BCLs). Traditionally, BCLs are diagnosed based on a phenotypic resemblance to normal B-cells; assessed by immunohistochemistry or flow cytometry, by using a handful of phenotypic markers. In the last decade, diagnostic and prognostic evaluation has been facilitated by global gene expression profiling (GEP), providing a new powerful means for the classification, prediction of survival, and response to treatment of lymphomas. However, most GEP studies have typically been performed on whole tissue samples, containing varying degrees of tumor cell content, which results in uncertainties in data analysis. In this study, global GEP analyses were performed on highly purified, flow-cytometry sorted tumor-cells from eight subgroups of BCLs. This enabled identification of TFs that can be uniquely associated to the tumor cells of chronic lymphocytic leukemia (CLL), diffuse large B-cell lymphoma (DLBCL), follicular lymphoma (FL), hairy cell leukemia (HCL), and mantle cell lymphoma (MCL). The identified transcription factors influence both the global and specific gene expression of the BCLs and have possible implications for diagnosis and treatment. Am. J. Hematol., 2010. © 2010 Wiley-Liss, Inc. [source] Management of hematological malignancies during pregnancy,AMERICAN JOURNAL OF HEMATOLOGY, Issue 12 2009Tina Rizack The management of hematological malignancies during pregnancy is a challenging endeavor, which not only requires technical skills and knowledge by the clinicians but also requires sound clinical judgment and compassion, keeping in mind the patient and family preferences and, ultimately, the wellbeing of the neonate. The incidence of hematological malignancies during pregnancy is rare, ranging from 1 in 1,000 to 1 in 10,000 deliveries, impeding the design and execution of large prospective studies. The purpose of this review is to evaluate the limited existing data and make useful suggestions in the management of acute and chronic leukemias, Hodgkin and non-Hodgkin lymphomas, plasma cell myeloma, and other hematological malignancies, such as myelodysplastic syndromes and hairy cell leukemia, during pregnancy. Am. J. Hematol. 2009. © 2009 Wiley-Liss, Inc. [source] CD52 expression in hairy cell leukemiaAMERICAN JOURNAL OF HEMATOLOGY, Issue 4 2003Michael M. Quigley Abstract Hairy cell leukemia (HCL) is a rare chronic B-cell lymphoproliferative disorder characterized by splenomegaly, pancytopenia, and circulating atypical lymphocytes with circumferential cytoplasmic projections. Although uncommon, HCL cases refractory to standard therapy occur, and effective alternatives are limited. There is evolving literature supporting monoclonal antibody therapy in the treatment of B-cell lymphoid malignancies, including anti-CD52 (Campath-1H, alemtuzumab). We have examined nine cases of HCL and one case of HCL variant by flow cytometry for CD52 expression. All cases expressed CD52 antigen in 92,100% of the malignant cells. The demonstration of CD52 antigen expression on HCL cells provides the rationale for the use of alemtuzumab in refractory HCL. Am. J. Hematol. 74:227,230, 2003. © 2003 Wiley-Liss, Inc. [source] The value of tartrate resistant acid phosphatase (TRAP) immunoreactivity in diagnosis of hairy cell leukemia,APMIS, Issue 3 2005HAMPAR AKKAYA Hairy cell leukemia (HCL) is a rare chronic B-cell lymphoproliferative disorder characterized by splenomegaly, pancytopenia, and circulating atypical lymphocytes with circumferential cytoplasmic projections. We investigated the specificity and the sensitivity of anti-TRAP antibody immunoreactivity in 57 cases of HCL. We found that there is a statistically highly significant difference between TRAP immunoreactivities of the study and the control groups, and HCL can be diagnosed by TRAP immunoreactivity in bone marrow trephine biopsy materials with a specificity of 98.27 % and a sensitivity of 100%. [source] Sustained long-term remissions with weekly interferon maintenance therapy in hairy cell leukemiaASIA-PACIFIC JOURNAL OF CLINICAL ONCOLOGY, Issue 3 2010Raj RAMAKRISHNA Abstract Aim: This study evaluates the efficacy of weekly ,-interferon (IFN) maintenance therapy in hairy cell leukaemia (HCL), a disease that remains incurable. Method: Nine patients (six male, three female, aged 41,69 yrs) with hairy cell leukaemia (HCL) received IFN 3mU s.c. once weekly as long-term maintenance therapy after achieving optimal clinical and hematological response to initial therapy with thrice weekly IFN. Results: Eight of the nine patients are in a state of sustained response at 3,17 years (median 12 years). Conclusion: Our results are similar to those from three previous studies using long-term IFN maintenance therapy, bringing the total number of patients in sustained remission to 118. We hope these reports will lead to a multi-centre, phase III study of IFN maintenance therapy (including pegylated IFN, given less frequently) in HCL patients achieving optimal response to initial therapy, be it IFN or a purine analogue. [source] | ||||||||||