Clinical staging system for hairy-cell leukemia

Abstract To find a clinical staging system for patients with hairy cell leukemia, 391 patients contributed by 22 centers were analyzed using the proportional hazard survival model. Attention was paid to nonsplenectomized patients to find a staging system to predict the survival length at the time of diagnosis. On the basis of hemoglobin level and spleen size at the time of diagnosis, 3 stages could be distinguished with significantly different prognoses (stages I-III). In addition, we addressed the question of splenectomy to identify those patients who benefit from the operation. Using arbitrary, but clinically relevant, criteria to call the operation beneficial, splenectomy appears to be indicated for patients with large spleens (greater than or equal to 4 cm under costal margin) or with smaller, but palpable, spleens when anemia (Hb less than 12 g/dl) is present. The third question concerned the splenectomized patients. To indicate the patients who have a poor postsplenectomy survival, a staging system was developed on the basis of hemoglobin level and number of neutrophils at 2–3 mo after the operation (stages A-C). The validity of the two staging systems was supported by the results of an analysis of an independent test series of patients. These staging systems may be helpful for the choice of therapy and in the planning of clinical trials in patients with hairy-cell leukemia.

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Clinical staging system for hairy-cell leukemia

Blood ◽

10.1182/blood.v60.3.571.bloodjournal603571 ◽

1982 ◽

Vol 60 (3) ◽

pp. 571-577 ◽

Cited By ~ 1

Author(s):

J Jansen ◽

J Hermans

Keyword(s):

Hairy Cell Leukemia ◽

Staging System ◽

Hemoglobin Level ◽

Clinical Staging ◽

Costal Margin ◽

Hairy Cell ◽

Cell Leukemia ◽

Staging Systems ◽

Clinical Staging System ◽

Choice Of Therapy

To find a clinical staging system for patients with hairy cell leukemia, 391 patients contributed by 22 centers were analyzed using the proportional hazard survival model. Attention was paid to nonsplenectomized patients to find a staging system to predict the survival length at the time of diagnosis. On the basis of hemoglobin level and spleen size at the time of diagnosis, 3 stages could be distinguished with significantly different prognoses (stages I-III). In addition, we addressed the question of splenectomy to identify those patients who benefit from the operation. Using arbitrary, but clinically relevant, criteria to call the operation beneficial, splenectomy appears to be indicated for patients with large spleens (greater than or equal to 4 cm under costal margin) or with smaller, but palpable, spleens when anemia (Hb less than 12 g/dl) is present. The third question concerned the splenectomized patients. To indicate the patients who have a poor postsplenectomy survival, a staging system was developed on the basis of hemoglobin level and number of neutrophils at 2–3 mo after the operation (stages A-C). The validity of the two staging systems was supported by the results of an analysis of an independent test series of patients. These staging systems may be helpful for the choice of therapy and in the planning of clinical trials in patients with hairy-cell leukemia.

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Hairy Cell Leukemia: A Retrospective Study of 235 Cases by the Italian Cooperative Group (ICGHCL) according to Jansen’s Clinical Staging System

Acta Haematologica ◽

10.1159/000206409 ◽

1984 ◽

Vol 72 (5) ◽

pp. 326-334 ◽

Cited By ~ 17

Author(s):

E.E. Damasio ◽

M. Spriano ◽

M. Repetto ◽

A.R. Vimercati ◽

E. Rossi ◽

...

Keyword(s):

Retrospective Study ◽

Hairy Cell Leukemia ◽

Staging System ◽

Clinical Staging ◽

Cooperative Group ◽

Hairy Cell ◽

Cell Leukemia ◽

Clinical Staging System

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Efficacy of Rituximab in Hairy Cell Leukemia.

Blood ◽

10.1182/blood.v108.11.4719.4719 ◽

2006 ◽

Vol 108 (11) ◽

pp. 4719-4719

Author(s):

Styliani I. Kokoris ◽

Maria K. Angelopoulou ◽

Zacharoula I. Galani ◽

Konstantinos Anargyrou ◽

Sotirios Sachanas ◽

...

Keyword(s):

Bone Marrow ◽

Interferon Alpha ◽

Hairy Cell Leukemia ◽

Response Rate ◽

Costal Margin ◽

Line Treatment ◽

Hairy Cell ◽

Cell Leukemia ◽

First Line ◽

First Line Treatment

Abstract BACKGROUND: Hairy Cell Leukemia (HCL) is a rare B-chronic lymphoproliferative disorder (BCLD) with an indolent course. First-line treatment modalities include 2-chlorodeoxyadenosine (2-CDA), 2-deoxycoformycin (2-DCF) and interferon-alpha. The efficacy of anti-CD20-Rituximab (R) in other BCLDs, as well as strong CD20 expression by HCL cells, indicate that R could be an alternative treatment of HCL. AIMS: The experience of a single Hematology Unit in the treatment of relapsed HCL with R. PATIENTS AND METHODS: We retrospectively analyzed all HCL patients (pts) who received R as salvage therapy in 1st or subsequent relapse. RESULTS: 13 patients treated with R were located among 110 patients diagnosed with HCL between 1980 and 2005. 11 were males and their median age before R treatment was 46 years (range: 42–88). 5 pts had splenomegaly with a median spleen size of 7cm below left costal margin (range:5–20cm). 3 pts has an absolute neutrophil count<1.5×109/L, 4 a hemoglobin <10g/dL and 5pts a platelet count<100×109/L. All patients displayed a typical immunophenotype from blood and/or bone marrow (CD20 strongly +, CD19+, CD22+, FMC-7+, CD11c+, CD25+, CD103+). Four of them were CD23+ and two CD10+. 8 pts received Rituximab at 1st relapse. Among them, one had received 2-DCF as first-line treatment, one 2-CDA and 6 interferon-alpha as induction and maintenance. 3 pts had received more than one prior treatments. Two pts received R at diagnosis, due to older age. The median time from diagnosis to R initiation was 61 months (range: 4–275). R was administered at 375mg/m2 weekly for 6 cycles. Overall response rate was 67%, with 4 pts showing a negative immunophenotype. One pt discontinued treatment after the first cycle due to the development of thrombocytopenia that was attributed to the drug. 7/8 responders showed a complete restoration of their cytopenias. No other complications were recorded, except of mild infusion-related symptoms. Among the responding pts, none has relapsed so far with a median follow-up of 14 months (range: 4–40+). Among partial responders, one achieved a complete response including a negative bone marrow and immunophenotype after R retreatment. CONCLUSIONS: R is a highly effective and tolerable treatment for HCL in relapse with a response rate of 67%. Retreatment or maintenance with R may be important, since ongoing responses are seen.

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Analysis of Parameters Predicting Treatment Efficacy and Outcome In Patients with Hairy Cell Leukemia (HCL) Receiving Subcutaneous Cladribine In the ICGHCL2004 Protocol (by the Italian Cooperative Group on HCL)

Blood ◽

10.1182/blood.v116.21.1369.1369 ◽

2010 ◽

Vol 116 (21) ◽

pp. 1369-1369

Author(s):

Francesco Forconi ◽

Emanuele Cencini ◽

Francesco Zaja ◽

Tamara Intermesoli ◽

Francesca Fiore ◽

...

Keyword(s):

Risk Factors ◽

Multivariate Analysis ◽

Hairy Cell Leukemia ◽

Single Agent ◽

Rapid Progression ◽

Costal Margin ◽

Hairy Cell ◽

Cell Leukemia ◽

Independent Risk Factors ◽

L 14

Abstract Abstract 1369 Introduction: Single-agent cladribine (2CdA) given intravenously or subcutaneously (sc) is highly effective in Hairy cell leukemia (HCL). However, HCL patients are projected to progress in their life-span or, in a minority, are refractory and may benefit from other treatment strategies. Parameters predicting efficacy of (sc)2CdA in HCL are scarcely known. Aims: In a national multicentre clinical trial (EudraCT code: ICGHCL2004) we prospectively sought parameters able to predict efficacy of sc2CdA in classic HCL requiring first treatment (1987 Consensus Criteria). Methods: Clinical data and samples were collected centrally for diagnostic revision according to the WHO criteria and for molecular analyses prior to treatment. Tumor IGH and TP53 analyses or Genome-wide DNA profiling was performed in the cases with >10% or >50% hairy cells (HC) in the test sample, respectively (Forconi, Blood 2009). Patients entering the study received 0.5–0.7 mg/kg sc2CdA as a single course. Efficacy endpoints were response to sc2CdA, treatment free interval (TFI), relapse free survival (RFS), and overall survival (OS). Responses were assessed on month 2 and 6 after treatment and defined according to the 1987 Consensus criteria. Complete Remissions (CR) and Partial Remissions (PR) were rated as beneficial responses (CR/PR), while minor Responses (mR) and No Responses (NR) were rated as failures (mR/NR). TFI was measured in all patients from sc2CdA initiation to time of new treatment requirement. RFS was measured in patients with CR/PR from sc2CdA initiation to relapse. OS was measured from sc2CdA initiation to death. Results: Of 148 patients, 116 were males (78,4%). Median values were: age 52 years (range 30–83), splenomegaly below costal margin 2 cm (0-16), hemoglobin 11,8 (5-17 g/dL), PLT 77×10e9/L (14-255), WBC 2,7×10e9/L (0,7-48), HC 0,2×10e9/L (0-38). Serum B2microglobulin was twice above upper limit (B2M>2X) in 9/122 patients (7,4%), IGH unmutated (UM) in 7/62 (11,3%) and TP53 mutated (mutTP53) in 2/62 (3,2%). Onehundred-forty patients (94,6%) had a CR/PR (101 CR, 68,2%; 39 PR, 26,4%) and 8 (5,4%) a mR/NR (5 mR, 3,4%; 3 NR, 2%). Risk factors of sc2CdA failure were splenomegaly (p=.001, best-cutoff> 3 cm bcm by ROC and Youden's Test), WBC (p<.001, best cut-off >10×10e9/L), HC counts (p<.001, best cut-off >5×10e9/L), and B2M>2X (p=.013). In 62 patients investigated for molecular features, UM-IGH and TP53 were confirmed as risk factors for mR/NR (p<.001 and p=.011). Conversely, age, performance status, 2CdA schedule, lymphadenopathy, neutrophil, haemoglobin and platelet counts, prior transfusions, time from diagnosis to treatment, Jansen score, LDH, marrow infiltration and hairy cell index failed to predict response. After a median follow-up of 37,5 months (range 12–67), 5 year TFI, RFS and OS were 67%, 71% and 94%, respectively. Causes of death were one infection in progressive refractory disease, 2 cardiac events and 3 second tumors. By Kaplan-Maier estimates, risk factors of shorter TFI at diagnosis were splenomegaly (p=.007), WBC>10×10e9/L (p<.001) or HC>5×10e9/L (p<.001), UM-IGH (p<.001), and TP53 disfunction (p=.002). By Cox multivariate analysis (covariates: HC>5×10e9/L, spleen>3cm, UM-IGH and mutTP53), UM-IGH (HR=8.1, 95%CI 1.7–38.1, p=.008, median TFI=8.5 months), high HC (HR=6.9, 95%CI 1.5–31.6, p=.012, TFI=27,4 months) and splenomegaly (HR=3.7, 95%CI 1.1–12.8, p=.039, TFI=49,3 months) scored as independent risk factors. Quality of response also predicted risk of short TFI (NR=mR>PR>CR, p<.001). In the patients with beneficial response, univariate analysis of clinical parameters identified WBC>5×10e9 (p=.016) and PR (p=.001)as risk factors of short RFS. Cox multivariate analysis revealed PR as the sole independent risk factor of relapse after a median of 46 months (HR=4.5, 95%CI 1,7-12,3, p=.003). 2CdA dose schedule was among other factors that did not associate with TFI or RFS. Conclusion: This data confirm that sc2CdA has potent activity in HCL. Tumor UM-IGH status, splenomegaly, high HC count (or leukocytosis) are confirmed as independent risk factors of treatment failure and rapid progression. RFS analysis identify PR as the sole independent factor of relapse risk in the patients initially responsive to sc2CdA. This analysis may have important implications for the selection of HCL patients that will require treatments alternative to single-agent 2CdA. Disclosures: No relevant conflicts of interest to declare.

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Red Cell Distribution Width (RDW) as a Marker of Disease Activity in Patients with Hairy Cell Leukemia

Acta Medica (Hradec Kralove Czech Republic) ◽

10.14712/18059694.2019.163 ◽

1998 ◽

Vol 41 (1) ◽

pp. 23-26

Author(s):

Ladislav Chrobák ◽

Pavel Žák ◽

Karel Podzimek ◽

Pravoslav Stránský

Keyword(s):

Hairy Cell Leukemia ◽

Mean Value ◽

Hemoglobin Level ◽

Red Cell Distribution Width ◽

Red Cell ◽

Cell Distribution ◽

Hairy Cell ◽

Cell Leukemia ◽

Distribution Width ◽

The Mean

Red cell distribution width (RDW) was examined in 18 patients with hairy cell leukemia (HCL) treated with 2- chlorodeoxyadenosine (2-CdA), in 5 patients treated with Interferon alpha (IFN-alpha) and in 9 patients subjected to splenectomy. Out of 18 patients treated with 2-CdA one patient was excluded of the study because of association of HCL with acquired sideroblastic anemia. In the remaining 17 patients the mean value of RDW before terapy was 18.8% (range 13.5% to 25.0%) and dropped after successful theapy after 6 to 12 months to the mean value of 13.6% (range 11.2% to 17.9%) and after 18 months to 13.4% (range 12.6% to 14.7%) (p = 0.00015 and p = 0.00049 respectivelly). The hemoglobin level increased from the mean value of 119 g/l (range 99 g/l to 157 g/l) before therapy to the mean value of 145.9 g/l (range 127 g/l to 172 g/l) after 6 to 12 months and after 18 months to 147.8 g/l (range 132 g/l to 168 g/l) (p = 0.000017 and p = 0.00036 respectively). The same trend was observed in the group of patients treated with IFN-alfa. The RDW decreased from the mean value of 21.3% (range 18.8% to 28.7%) to the mean value of 15.3% (range 12.4% to 16.7%), (p = 0.031). The hemoglobin level increased in this group of patients from the mean value of 115 g/l (range 98 g/l to 127 g/l) to the mean value of 136 g/l (range 127 g/l to 146 g/l) (p = 0.031). In 9 patients in complete hematologic remission 34 to 293 months after splenectomy the mean value of RDW was 13.9% (range 13.0% to 15.5%). Conclusion: Increased RDW in HCL is associated with active disease and is reversible after successful therapy. This phenomenon has not been reported in the literature yet. Preliminary results show that the increase of RDW may be due to the dyserythropoiesis.

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