Prospective evaluation of internal adenopathy in a cohort of 43 patients with hairy cell leukemia.

1994 ◽  
Vol 12 (2) ◽  
pp. 268-272 ◽  
Author(s):  
D Hakimian ◽  
M S Tallman ◽  
D K Hogan ◽  
A W Rademaker ◽  
E Rose ◽  
...  
Keyword(s):  
Hairy Cell Leukemia ◽  
Disease Duration ◽  
Disease Free Survival ◽  
Newly Diagnosed ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Free Survival ◽  
Median Disease Duration ◽  
Before And After

PURPOSE To determine the role of computed tomography (CT) in patients with hairy cell leukemia (HCL), we report a series of 43 patients prospectively evaluated for internal adenopathy by CT before and after treatment with 2-chlorodeoxyadenosine (2-CdA). PATIENTS AND METHODS CT was performed on 43 consecutive patients with HCL before and 3 months after a single cycle of 2-CdA. Twenty-four patients were previously diagnosed and 19 were newly diagnosed. Adenopathy was considered bulky if the greatest dimension of any confluent mass was between 5 and 10 cm and massive if greater than 10 cm. RESULTS Internal adenopathy was present in six of 43 patients (14%). Three of the six patients had massive abdominal adenopathy and one had bulky abdominal adenopathy. All six patients with adenopathy were previously diagnosed, while none of the 19 newly diagnosed patients had internal adenopathy. In those patients previously diagnosed, the six with adenopathy had a median disease duration of 68 months, while the 18 patients without adenopathy had a median disease duration of 24 months (P = .01). Adenopathy was more common in splenectomized patients. In previously diagnosed patients, adenopathy occurred in five of 10 (50%) splenectomized patients and one of 14 (7%) nonsplenectomized patients (P = .05). However, the 10 splenectomized patients had a median disease duration of 56 months, while the 14 nonsplenectomized patients had a median disease duration of 16 months (P = .004). All six patients had significant reduction in adenopathy 3 months after 2-CdA and were without residual HCL in the bone marrow. CONCLUSION Significant internal adenopathy in patients with HCL is more frequent than previously recognized. Adenopathy is rare at diagnosis and appears to be related to disease duration. As patients treated with 2-CdA have long disease-free survival durations, detection of significant adenopathy by CT scan may be important; however, routine CT scans are not recommended at the time of diagnosis.

Pentostatin induces durable remissions in hairy cell leukemia.

1991 ◽  
Vol 9 (2) ◽  
pp. 243-246 ◽  
Author(s):  
P A Cassileth ◽  
B Cheuvart ◽  
A S Spiers ◽  
D P Harrington ◽  
F J Cummings ◽  
...  
Keyword(s):  
Hairy Cell Leukemia ◽  
Overall Response Rate ◽  
Disease Free Survival ◽  
Maximal Response ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Free Survival ◽  
Disease Free

Fifty patients with hairy cell leukemia were treated with pentostatin (2'-deoxycoformycin; dCF) for a median of 3 months; 32 (64%) patients achieved complete remission (CR), and 10 (20%) patients achieved partial remission (PR), for an overall response rate of 84%. After reaching maximal response, no maintenance therapy was administered. The median duration of follow-up is now 39 months, and only four of 32 patients in CR and two of 10 patients in PR have relapsed. dCF therapy produces durable long-term, disease-free survival in patients with hairy cell leukemia.

scholarly journals Phase I Trial of Anti-CD22 Recombinant Immunotoxin Moxetumomab Pasudotox (CAT-8015 or HA22) in Patients With Hairy Cell Leukemia

2012 ◽  
Vol 30 (15) ◽  
pp. 1822-1828 ◽  
Author(s):  
Robert J. Kreitman ◽  
Martin S. Tallman ◽  
Tadeusz Robak ◽  
Steven Coutre ◽  
Wyndham H. Wilson ◽  
...  
Keyword(s):  
Phase I ◽  
Hairy Cell Leukemia ◽  
Neutralizing Antibodies ◽  
Disease Free Survival ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Free Survival ◽  
Recombinant Immunotoxin ◽  
Uremic Syndrome ◽  
Dose Levels

Purpose To conduct a phase I dose-escalation trial assessing safety and response of recombinant immunotoxin moxetumomab pasudotox (CAT-8015, HA22) in chemotherapy-resistant hairy cell leukemia (HCL). Patients and Methods Eligible patients had relapsed/refractory HCL after ≥ two prior therapies and required treatment because of abnormal blood counts. Patients received moxetumomab pasudotox 5 to 50 μg/kg every other day for three doses (QOD ×3), with up to 16 cycles repeating at ≥ 4-week intervals if patients did not experience disease progression or develop neutralizing antibodies. Results Twenty-eight patients were enrolled, including three patients each at 5, 10, 20, and 30 μg/kg, four patients at 40 μg/kg, and 12 patients at 50 μg/kg QOD ×3 for one to 16 cycles each (median, four cycles). Dose-limiting toxicity was not observed. Two patients had transient laboratory abnormalities consistent with grade 2 hemolytic uremic syndrome with peak creatinine of 1.53 to 1.66 mg/dL and platelet nadir of 106,000 to 120,000/μL. Drug-related toxicities in 25% to 64% of the 28 patients included (in decreasing frequency) grade 1 to 2 hypoalbuminemia, aminotransferase elevations, edema, headache, hypotension, nausea, and fatigue. Of 26 patients evaluable for immunogenicity, 10 patients (38%) made antibodies neutralizing more than 75% of the cytotoxicity of 1,000 ng/mL of immunotoxin, but this immunogenicity was rare (5%) after cycle 1. The overall response rate was 86%, with responses observed at all dose levels, and 13 patients (46%) achieved complete remission (CR). Only 1 CR lasted less than 1 year, with the median disease-free survival time not yet reached at 26 months. Conclusion Moxetumomab pasudotox at doses up to 50 μg/kg QOD ×3 has activity in relapsed/refractory HCL and has a safety profile that supports further clinical development for treatment of this disease.

Pentostatin and Cladribine in Hairy Cell Leukemia- a Retrospective Comparison of a Large Series with Long Follow up.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3472-3472 ◽  
Author(s):  
Monica Else ◽  
Nnenna Osuji ◽  
Ilaria Del ◽  
Estella Matutes ◽  
Rosa Ruchlemer ◽  
...  
Keyword(s):  
Hairy Cell Leukemia ◽  
Statistical Significance ◽  
Disease Free Survival ◽  
Disease Diagnosis ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Retrospective Comparison ◽  
Significant Difference ◽  
Alternative Agent

Abstract Complete responses (CR) of up to 95% have been reported with both pentostatin and cladribine in patients with hairy cell leukemia (HCL). Although responses are durable, relapses occur even 10 years after treatment. No trial has compared pentostatin to cladribine for HCL. Furthermore, few series achieve sufficient maturity to answer the question: can these agents induce a cure in HCL? We reviewed retrospectively 219 patients with HCL (median follow up (FU) from diagnosis: 12.6 years) to compare pentostatin with cladribine in the treatment of HCL, and to assess the potential for cure in this disease. Diagnosis of HCL and exclusion of HCL-variant were confirmed by central review. Overall response to 1st line pentostatin (n=185) was 96% with CR of 81% and median FU of 10.8 (range 0.3–17.9) years. Response to cladribine was 100% with CR of 82%. No significant difference in response was seen with cladribine (n=34) at median FU of 7.2 (range 0.5–11.5) years. Median disease free survival (DFS) for both agents was 10 years. 38% of patients relapsed 4.9 (range 1–16) years and 4.4 (range 1–10) years after treatment with pentostatin and cladribine, respectively. Although responses were maintained for pentostatin and cladribine when used at 2nd (94% and 100% respectively) and 3rd line (100% and 100% respectively) treatment, CR decreased significantly with each sequential relapse through 70% to 45% (p≤0.01). Attainment of CR at 1st line treatment was significantly associated with increased DFS (p=0.000) as compared to those achieving only partial response (PR). Figure Figure A similar result was seen at 2nd line therapy (p=0.000). DFS also showed a significant decline with sequential treatment (p=0.001) mirroring the reduced likelihood of achieving CR with increasing number of courses. There was some crossover of patients. At 1st relapse, 20 patients received pentostatin of whom 17 had previously received this agent, and 53 patients received cladribine of whom 44 were previously treated with pentostatin. Patients relapsing after an initial CR showed no significant difference in ability to re-attain CR as opposed to PR or no response, whether retreated with the same agent, or switched to the other. However, for patients who initially failed to achieve CR, switching to the alternative agent was associated with an increased rate of CR although this difference did not achieve statistical significance. Known 2nd malignancies (excluding basal cell carcinoma) occurred in 20 patients (9 %). In a separate group of 7 patients who were never treated with either agent, 2 patients developed 2nd malignancies. We demonstrated equivalent efficacies of pentostatin and cladribine in the treatment of HCL. Median survival has not been reached for either agent. At 10 years FU, the survival is 83% (pentostatin) and 90% (cladribine). DFS curves show no plateau for either agent with relapses occurring up to 16 years after pentostatin treatment. True cure in this disease thus remains elusive, however, our results suggest that first line CR with purine analogue therapy should be the prime aim of HCL treatment.

scholarly journals Current role of interferon in hairy cell leukemia therapy: a timely decision

2019 ◽  
Vol 41 (1) ◽  
pp. 88-90 ◽  
Author(s):  
Wellington Fernandes da Silva ◽  
Larissa Lane Cardoso Teixeira ◽  
Vanderson Rocha ◽  
Valeria Buccheri
Keyword(s):  
Hairy Cell Leukemia ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Current Role

scholarly journals Treatment of hairy cell leukemia with recombinant alpha-interferon

Blood ◽  
1986 ◽  
Vol 68 (2) ◽  
pp. 493-497 ◽  
Author(s):  
JR Quesada ◽  
EM Hersh ◽  
J Manning ◽  
J Reuben ◽  
M Keating ◽  
...  
Keyword(s):  
Hairy Cell Leukemia ◽  
Tumor Burden ◽  
Newly Diagnosed ◽  
Hairy Cell ◽  
Blood Products ◽  
Cell Leukemia ◽  
Recombinant Interferon ◽  
Mediastinal Adenopathy ◽  
Recombinant Interferon Alpha

Abstract Thirty patients with hairy cell leukemia were treated with recombinant interferon alpha-A (rIFN alpha A; Roferon-A); seven were previously untreated. Nine complete and 17 partial remissions were documented by bone marrow core biopsies. All patients' peripheral blood hematologic indexes either improved or normalized. Twelve of 13 patients with retroperitoneal or mediastinal adenopathy obtained remissions of tumor masses. All seven patients with splenomegaly showed prompt reduction in the size of the spleen to normal size. The incidence of complete remissions was significantly higher (P = .02) in previously untreated patients (5 of 7) than in those in whom splenectomy had been performed (4 of 23), a result presumed to be related to the pretreatment tumor burden. rIFN alpha A was well tolerated; mild fatigue was the most frequent complaint. In most patients, tumor remissions resulted in an improved quality of life: they eliminated the need for transfusing blood products and reduced the incidence of infections, and immune deficits were apparently restored in some of the patients. We conclude that rIFN alpha A is an effective therapy for all stages of hairy cell leukemia including previously untreated or newly diagnosed patients.

Hairy cell leukemias with unmutated IGHV genes define the minor subset refractory to single-agent cladribine and with more aggressive behavior

Blood ◽  
2009 ◽  
Vol 114 (21) ◽  
pp. 4696-4702 ◽  
Author(s):  
Francesco Forconi ◽  
Elisa Sozzi ◽  
Emanuele Cencini ◽  
Francesco Zaja ◽  
Tamara Intermesoli ◽  
...  
Keyword(s):  
Single Agent ◽  
Newly Diagnosed ◽  
Potential Mechanism ◽  
Hairy Cell ◽  
Multicenter Clinical Trial ◽  
Cell Leukemia ◽  
Event Free Survival ◽  
Free Survival ◽  
Aggressive Disease ◽  
High Incidence

Abstract Hairy cell leukemia (HCL) is generally responsive to single-agent cladribine, and only a minority of patients are refractory and with poor prognosis. HCLs generally express mutated (M) and, in a minority, unmutated (UM) IGHV. In a multicenter clinical trial in newly diagnosed HCL, we prospectively investigated clinical and molecular parameters predicting response and event-free survival after single-agent cladribine. Of 58 HCLs, 6 expressed UM-IGHV (UM-HCL) and 52 M-IGHV (M-HCL). Beneficial responses were obtained in 53 of 58 patients (91%), whereas treatment failures were observed in 5 of 58 patients (9%). Failures were associated significantly with UM-IGHV (5 of 5 failures vs 1 of 53 beneficial responses had UM-IGHV, P < .001), leukocytosis (3 of 5 vs 3 of 53, P = .006), and bulky spleen (4 of 5 vs 4 of 53, P < .001). The UM-HCL not benefiting from cladribine characteristically had bulky spleen (4 of 5, 80%), leukocytosis (3 of 5, 60%), and TP53 defects (2 of 5, 40%), and progressed rapidly after first treatment (median event-free survival, 7.5 months). Our data suggest that UM-HCLs identify the minor subgroup failing cladribine treatment and with more aggressive disease. High incidence of TP53 dysfunction indicates a potential mechanism of resistance to cladribine in the UM-HCL group. Overall, our data provide new molecular elements relevant for treatment concerns in HCL.

Sign in / Sign up

Export Citation Format

Share Document