scholarly journals Intensive chemotherapy of hairy cell leukemia in patients with aggressive disease

Blood ◽  
1985 ◽  
Vol 65 (1) ◽  
pp. 115-119 ◽  
Author(s):  
F Calvo ◽  
S Castaigne ◽  
F Sigaux ◽  
M Marty ◽  
L Degos ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Partial Response ◽  
Hairy Cell Leukemia ◽  
Progressive Disease ◽  
Complete Response ◽  
Intensive Chemotherapy ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Biopsy Specimens ◽  
The Mean

Abstract Seven patients with hairy cell leukemia were treated by intensive chemotherapy because they were considered to have a progressive disease and a poor short-term prognosis. The mean age was 47 years (range, 36 to 58). Six of seven patients had prior splenectomies with minor or transient hematologic responses. One patient had no spleen enlargement. The seven patients had never received any cytotoxic drugs and had prolonged granulocytopenia (less than 300/microL) with recurrent, severe infectious episodes. Chemotherapy included Rubidazone (zorubicine hydrochloride) 450 mg/m2 on day 1, arabinosyl cytosine 200 mg/m2/d from day 1 to day 5, and cyclophosphamide, 2,000 mg/m2 on day 5. Responses were assessed through examination of repeat bone marrow biopsy specimens and blood counts. A complete response was defined as normal blood counts associated with the disappearance of hairy cell infiltration and fibrosis on the bone marrow biopsy specimens. A partial response was defined as normal blood counts with persistence of leukemic cells in the bone marrow. Three patients achieved a complete response, and one patient had a partial response. Three patients died of infectious complications during induction chemotherapy. For the responding patients, the mean duration of aplasia was 37 +/- 5 days. Follow-up for the responding patients has been 44+, 24, 32+, and 23+ months. One patient with a complete response died while on maintenance therapy. We conclude that complete and prolonged histologic remission of hairy cell leukemia can be obtained with intensive chemotherapy. The toxicity of chemotherapy is such, however, that progressive disease after splenectomy needs to be more clearly defined.

scholarly journals Intensive chemotherapy of hairy cell leukemia in patients with aggressive disease

Blood ◽  
1985 ◽  
Vol 65 (1) ◽  
pp. 115-119 ◽  
Author(s):  
F Calvo ◽  
S Castaigne ◽  
F Sigaux ◽  
M Marty ◽  
L Degos ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Partial Response ◽  
Hairy Cell Leukemia ◽  
Progressive Disease ◽  
Complete Response ◽  
Intensive Chemotherapy ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Biopsy Specimens ◽  
The Mean

Seven patients with hairy cell leukemia were treated by intensive chemotherapy because they were considered to have a progressive disease and a poor short-term prognosis. The mean age was 47 years (range, 36 to 58). Six of seven patients had prior splenectomies with minor or transient hematologic responses. One patient had no spleen enlargement. The seven patients had never received any cytotoxic drugs and had prolonged granulocytopenia (less than 300/microL) with recurrent, severe infectious episodes. Chemotherapy included Rubidazone (zorubicine hydrochloride) 450 mg/m2 on day 1, arabinosyl cytosine 200 mg/m2/d from day 1 to day 5, and cyclophosphamide, 2,000 mg/m2 on day 5. Responses were assessed through examination of repeat bone marrow biopsy specimens and blood counts. A complete response was defined as normal blood counts associated with the disappearance of hairy cell infiltration and fibrosis on the bone marrow biopsy specimens. A partial response was defined as normal blood counts with persistence of leukemic cells in the bone marrow. Three patients achieved a complete response, and one patient had a partial response. Three patients died of infectious complications during induction chemotherapy. For the responding patients, the mean duration of aplasia was 37 +/- 5 days. Follow-up for the responding patients has been 44+, 24, 32+, and 23+ months. One patient with a complete response died while on maintenance therapy. We conclude that complete and prolonged histologic remission of hairy cell leukemia can be obtained with intensive chemotherapy. The toxicity of chemotherapy is such, however, that progressive disease after splenectomy needs to be more clearly defined.

scholarly journals Treatment of hairy cell leukemia with recombinant alpha interferon: I. Quantitative study of bone marrow changes during the first months of treatment

Blood ◽  
1986 ◽  
Vol 67 (3) ◽  
pp. 817-820 ◽  
Author(s):  
G Flandrin ◽  
F Sigaux ◽  
S Castaigne ◽  
C Billard ◽  
M Aguet ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Hairy Cell Leukemia ◽  
Alpha Interferon ◽  
Initial Increase ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Bone Marrow Biopsies ◽  
Biopsy Specimens ◽  
Hairy Cells ◽  
Normal Controls

Abstract Seventeen patients with hairy cell leukemia (HCL) were treated with low doses of recombinant alpha interferon (IFN) for over 4 months. Marked improvement was observed in peripheral blood and bone marrow in 15 of 17 patients. Comparison of pretreatment values and hemograms obtained after 4 months of treatment showed a marked decrease in circulating hairy cells (P less than .01), a decrease in the number of lymphocytes (P less than .01), a rise in the number of platelets (P less than .05), granulocytes (P less than .05), and monocytes (P less than .01), and a rise in the hemoglobin level (P less than .01). Transient reduction in the number of granulocytes was noted during the first month. Correction of thrombocytopenia often appeared within 2 months and usually preceded improvement of anemia, monocytopenia, and neutropenia. Bone marrow biopsy specimens were taken before treatment and 2, 4, and 7 months after its initiation. The volumes occupied by hairy cells, cells of the myeloid lines, and adipocytes were studied by stereological analysis of semithin sections. Decrease in the volume occupied by hairy cells was seen after 4 months of treatment (P less than .01), and the volume continued to decrease at the seventh month (P less than .05). Hairy cells were no longer detected on bone marrow biopsies of 4 of 17 patients by the fourth month and in 3 of 8 additional patients by the seventh month. A rise in the volume occupied by normal myeloid cells was visible by the second month of treatment (P less than .01). Nevertheless, the volume occupied by granulocytes remained lower than in the normal controls (P less than .01). After an initial increase during the first 2 months of treatment (P less than .01), the overall cellularity remained unchanged at 4 months and decreased significantly (P less than .05) at 7 months. Except for biopsies at 2 months, mean cellularity was below that of control biopsies (P less than .01).

scholarly journals Progress report on chlorambucil therapy in postsplenectomy patients with progressive hairy cell leukemia

Blood ◽  
1981 ◽  
Vol 57 (3) ◽  
pp. 464-467 ◽  
Author(s):  
HM Golomb
Keyword(s):  
Bone Marrow ◽  
Hairy Cell Leukemia ◽  
Progressive Disease ◽  
Single Agent ◽  
Objective Response ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Hairy Cells ◽  
Total Bone Marrow ◽  
Single Agent Chemotherapy

Abstract Of eight patients with progressive hairy cell leukemia who were treated with daily doses of an alkylating agent (chlorambucil, 4 mg) for at least 6 mo, seven have had an objective response, as measured by improved blood counts. Two patients were pancytopenic and had almost total bone marrow replacement with hairy cells at the initiation of chemotherapy; approximatley 6 mo later, their blood counts and bone marrow had improved dramatically. The five other patients had the leukemic form of the disease, and all responded to therapy. It is important to identify postsplenectomy patients with progressive disease in order to initiate low-dose single-agent chemotherapy.

scholarly journals Progress report on chlorambucil therapy in postsplenectomy patients with progressive hairy cell leukemia

Blood ◽  
1981 ◽  
Vol 57 (3) ◽  
pp. 464-467
Author(s):  
HM Golomb
Keyword(s):  
Bone Marrow ◽  
Hairy Cell Leukemia ◽  
Progressive Disease ◽  
Single Agent ◽  
Objective Response ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Hairy Cells ◽  
Total Bone Marrow ◽  
Single Agent Chemotherapy

Of eight patients with progressive hairy cell leukemia who were treated with daily doses of an alkylating agent (chlorambucil, 4 mg) for at least 6 mo, seven have had an objective response, as measured by improved blood counts. Two patients were pancytopenic and had almost total bone marrow replacement with hairy cells at the initiation of chemotherapy; approximatley 6 mo later, their blood counts and bone marrow had improved dramatically. The five other patients had the leukemic form of the disease, and all responded to therapy. It is important to identify postsplenectomy patients with progressive disease in order to initiate low-dose single-agent chemotherapy.

Rituximab For Hairy-Cell Leukemia (HCL): A Multicentric Retrospective Study Of 41 Cases

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5308-5308
Author(s):  
Mathieu Leclerc ◽  
Felipe Suarez ◽  
Marie-Pierre Noël ◽  
Anne Vekhoff ◽  
Xavier Troussard ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Combination Therapy ◽  
Hairy Cell Leukemia ◽  
Complete Response ◽  
Median Number ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Front Line ◽  
Line Therapy ◽  
Time To Relapse

Abstract Introduction the purine analogs (PA) cladribine (CDA) and pentostatin have dramatically improved the prognosis of HCL and are considered the standard of care both in front-line therapy and at relapse. However, some patients still fail to respond or will eventually relapse after treatment with PA. Chimeric anti-CD20 monoclonal antibody rituximab has shown significant activity in HCL and is an option for relapsed/refractory patients either alone or in combination with PA. Methods we retrospectively reviewed 49 treatments with rituximab for classical HCL, undertaken in 41 patients (pts) from 10 centers in France and Belgium between july 2002 and september 2012. Patients were included if they had received at least 3 infusions of rituximab. Eight pts were treated twice with rituximab. Complete hematologic response (CHR) was defined as recovery of normal blood counts (without circulating HCL cells) and absence of HCL-related symptoms. CHR was further divided into 3 groups: (i) stringent complete response (sCR), if bone marrow evaluation was normal; (ii) unconfirmed complete response (uCR), if bone marrow evaluation was not done and (iii) CHR with persistent medullar infiltration (iCHR). Partial response (PR) corresponded to a ≥ 50% improvement for every CHR-defining criterion or normalization of at least one blood count, without circulating HCL cells. Results characteristics of pts before treatment are summarized in table 1. Rituximab was given as front-line therapy in 8 cases (16.3%). When used at relapse/progression, the median number of previous lines was 3 (range 1-8) and all pts had already received PA (both CDA and pentostatin in one third of them). Rituximab was given alone in 55% of cases, while it was combined with a PA in the 45% remaining cases, mostly CDA (18 of 22 cases). The median number of infusions was 4 (3-12). After treatment, median absolute neutrophil count (ANC), hemoglobin (Hb) level and platelet count were 2.75 x 109/L, 135 g/L and 180 x 109/L respectively. Persistent significant neutropenia (ANC < 1 x 109/L) and anemia (Hb < 100g/L) were each found in only 2 cases and 10.6% of patients had platelets < 100 x 109/L. Overall response rate (ORR) was 89.6% with 70.8% CHR including 6 sCR (12.5%), 26 uCR (54.2%) and 2 iCHR (4.2%). PR was achieved in 9 cases (18.8%) and 5 pts were non responders (10.4%). All the 8 pts who received rituximab as front-line therapy (along with CDA in 5 of them) achieved CHR. In the relapsed pts, ORR was 87.5% (including 65% CHR) with a better outcome for those having received both rituximab and PA (100% ORR including 85.7% CHR versus 79.2% ORR and 54.2% CHR after rituximab alone). The 5 pts who failed to respond were relapsed pts treated with rituximab alone. Interestingly, all the 8 pts who were re-challenged with rituximab responded again to treatment (6 uCR, 1 iCHR and 1 PR). Multivariate analysis identified 3 independent prognostic factors for response to rituximab: absence of previous therapy (OR=0.027 [0,001-0,555], p=0,0192), combination therapy (OR=10,120 [1,227-83,485], p=0,0316) and ANC before treatment (OR=1,002 [1,001-1,004], p=0,006). The median follow-up is 36 months (4-117). Relapse or progression was observed in 15 cases (34.1%), with a median time to relapse of 19 months (2-39). Relapse rate was higher (54.5%) and time to relapse shorter (17.5 months) when rituximab was administered alone, as compared to combination therapy with a PA (10% and 38.5 months respectively). Overall, 3-year relapse-free survival is 68.3%. Six pts have died and 3-year overall survival is 90.3%. Conclusion this study confirms the efficacy of rituximab in HCL patients, mostly when combined to PA. Nevertheless, the relapse rate is high and time to relapse short when rituximab is used as monotherapy beyond front-line treatment. Further prospective studies are warranted to confirm the superiority of the combination PA + rituximab over PA alone. Disclosures: Off Label Use: Rituximab for the treatment of hairy-cell leukemia.

Alpha-2 interferon therapy of hairy-cell leukemia: a multicenter study of 64 patients.

1986 ◽  
Vol 4 (6) ◽  
pp. 900-905 ◽  
Author(s):  
H M Golomb ◽  
A Jacobs ◽  
A Fefer ◽  
H Ozer ◽  
J Thompson ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Hairy Cell Leukemia ◽  
Interferon Therapy ◽  
Complete Response ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Minor Response ◽  
Serious Infections ◽  
Hairy Cells ◽  
A Minor

Sixty-four patients with hairy-cell leukemia (HCL) (61 had undergone prior splenectomy) were treated with alpha-2 interferon (Intron A, Schering Corp, Kenilworth, NJ) subcutaneously three times per week at a dosage of 2 X 10(6) U/m2. Three patients (5%) demonstrated a complete response (CR) with apparent eradication of hairy cells from the bone marrow, 45 patients (70%) showed a partial response (PR) defined as normalization of all three blood counts associated with decreased involvement in the bone marrow, and nine patients (14%) showed a minor response that included improvement in at least one blood count. Three patients had no response, three patients died before completing 1 month of therapy, and one patient refused further therapy after 1 month of therapy. The median platelet count returned to normal by the second month of treatment. The median hemoglobin returned to greater than 12 mg/dL by the fourth month of treatment, and the median granulocyte count to greater than 1,500/mu by the fifth month of treatment. Bone marrow biopsy analysis during interferon therapy demonstrated a decrease in median hairy-cell index by more than half. Transfusion of both RBCs and platelets were decreased within 4 months of initiating treatment. Serious infections, which averaged four per month in 16 of the 64 patients before interferon therapy, were rarely observed after the first month of treatment. Treatment-induced toxicity was mild, consisting primarily of influenza-like symptoms, fatigue, and minor skin disorders. Alpha-2 interferon therapy is highly effective in reversing the course of progressive HCL and should be considered the treatment of choice for a minimum of 12 months in patients who have progressive disease post-splenectomy.

scholarly journals Treatment of hairy cell leukemia with recombinant alpha interferon: I. Quantitative study of bone marrow changes during the first months of treatment

Blood ◽  
1986 ◽  
Vol 67 (3) ◽  
pp. 817-820
Author(s):  
G Flandrin ◽  
F Sigaux ◽  
S Castaigne ◽  
C Billard ◽  
M Aguet ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Hairy Cell Leukemia ◽  
Alpha Interferon ◽  
Initial Increase ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Bone Marrow Biopsies ◽  
Biopsy Specimens ◽  
Hairy Cells ◽  
Normal Controls

Seventeen patients with hairy cell leukemia (HCL) were treated with low doses of recombinant alpha interferon (IFN) for over 4 months. Marked improvement was observed in peripheral blood and bone marrow in 15 of 17 patients. Comparison of pretreatment values and hemograms obtained after 4 months of treatment showed a marked decrease in circulating hairy cells (P less than .01), a decrease in the number of lymphocytes (P less than .01), a rise in the number of platelets (P less than .05), granulocytes (P less than .05), and monocytes (P less than .01), and a rise in the hemoglobin level (P less than .01). Transient reduction in the number of granulocytes was noted during the first month. Correction of thrombocytopenia often appeared within 2 months and usually preceded improvement of anemia, monocytopenia, and neutropenia. Bone marrow biopsy specimens were taken before treatment and 2, 4, and 7 months after its initiation. The volumes occupied by hairy cells, cells of the myeloid lines, and adipocytes were studied by stereological analysis of semithin sections. Decrease in the volume occupied by hairy cells was seen after 4 months of treatment (P less than .01), and the volume continued to decrease at the seventh month (P less than .05). Hairy cells were no longer detected on bone marrow biopsies of 4 of 17 patients by the fourth month and in 3 of 8 additional patients by the seventh month. A rise in the volume occupied by normal myeloid cells was visible by the second month of treatment (P less than .01). Nevertheless, the volume occupied by granulocytes remained lower than in the normal controls (P less than .01). After an initial increase during the first 2 months of treatment (P less than .01), the overall cellularity remained unchanged at 4 months and decreased significantly (P less than .05) at 7 months. Except for biopsies at 2 months, mean cellularity was below that of control biopsies (P less than .01).

scholarly journals Vemurafenib and Rituximab in Patients with Hairy Cell Leukemia Previously Treated with Moxetumomab Pasudotox

2021 ◽  
Author(s):  
Tadeusz Robak ◽  
Agnieszka Janus ◽  
Krzysztof Jamroziak ◽  
Enrico Tiacci ◽  
Robert J Kreitman
Keyword(s):  
Bone Marrow ◽  
Hairy Cell Leukemia ◽  
Combined Treatment ◽  
Complete Response ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Hematologic Response ◽  
Highly Active ◽  
End Of Treatment ◽  
Heavily Pretreated

The purine nucleoside analogues cladribine and pentostatin are highly-active first-line therapeutic treatments for hairy cell leukemia (HCL), resulting in complete response rates of 80 to 90%. However, HCL patients continue to relapse, and sooner or later, most require subsequent lines of treatment. This report presents the relapsed patients with classic HCL who were treated with vemurafenib (mostly at the low dose of 240 mg twice daily for 16 weeks) combined with rituximab after the failure of several lines of therapy including cladribine with or without rituximab and moxetumomab pasudotox. Two patients achieved CR MRD (-) after combined treatment with vemurafenib and rituximab, with a hematologic response ongoing after 38 months from the end of treatment in one patient and a relapse of cytopenias occurring after 13 months in the other patient. A third patient normalized her blood counts and this hematologic response, which was not evaluated in the bone marrow at the end of treatment, was lost after 18 months. The last patient died due to infection and multi-organ failure, too early to verify response to vemurafenib. Two patients who had relapsed after vemurafenib and rituximab derived meaningful clinical benefit from retreatment with the same agents, but eventually relapsed again and started indefinite therapy with dabrafenib and trametinib with normalization of the blood counts despite heavy bone marrow infiltration in the only patient so far evaluable. The outcomes of these cases indicate that novel targeted agents, and in particular vemurafenib combined with rituximab, improve the prognosis of HCL patients, even those heavily pretreated with PNAs and moxetumomab pasudotox

Long term follow up after treatment of hairy cell leukemia with 2-CdA

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 16518-16518
Author(s):  
M. Ramzi ◽  
M. Haghshenas
Keyword(s):  
Partial Response ◽  
Hairy Cell Leukemia ◽  
Complete Response ◽  
Second Malignancy ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Duration Of Response ◽  
Long Term Follow Up

16518 Background: Hairy cell leukemia (HCL) is a chronic B cell disorder that follows an indolent but progressive course. The ability of new purine analogue deoxyadenosine to induce long lasting complete remission in patients with hairy cell leukemia has revolutionized the treatment of this disease. We report the long term outcomes of patients with HCL treated in Shiraz, in south of Iran,with this drug. Methods: Between October 1993 till April 2004, 79 patients with classic symptomatic hairy cell leukemia were treated with 2-cholorodeoxy adenosine (2-CdA) with dose of 0.1 mg/kg of body weight per day by continuous intravenous infusion for 7 days. Results: sixty nine (87%) of patients achieved an initial complete response and 10 (12.6) a partial response with an overall median duration of response follow up of 78 months. Five patients had relapsed at a median of 43 months. All of 5 patients after relapse treated with second courses of 2-CdA, 4 (80%) achieved second complete responses and one (20%) partial response. In our study we had not any case of second malignancy after treatment with 2-CdA with median follow up of 82.5 months. Conclusion: This study confirms single course of 2-CdA induced long lasting complete response in the vast majority of patients. Relapse rate for complete responders were low. Patients who relapse can be successfully retreated with this drug, so we conclude 2-CdA had high efficacy and a favorable acute and long term toxicity profile in our patients in south of Iran, without any increase in risk of second malignancy. No significant financial relationships to disclose.

scholarly journals Vemurafenib and Rituximab in Patients with Hairy Cell Leukemia Previously Treated with Moxetumomab Pasudotox

2021 ◽  
Vol 10 (13) ◽  
pp. 2800
Author(s):  
Tadeusz Robak ◽  
Agnieszka Janus ◽  
Krzysztof Jamroziak ◽  
Enrico Tiacci ◽  
Robert J. Kreitman
Keyword(s):  
Bone Marrow ◽  
Hairy Cell Leukemia ◽  
Residual Disease ◽  
Combined Treatment ◽  
Complete Response ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Hematologic Response ◽  
Highly Active ◽  
End Of Treatment

The purine nucleoside analogues cladribine and pentostatin are highly-active first-line therapeutic treatments for hairy cell leukemia (HCL), resulting in complete response rates of 80% to 90%. However, HCL patients continue to relapse, and sooner or later, most require subsequent lines of treatment. This report presents the cases of four relapsed patients with classic HCL who were treated with vemurafenib (mostly at the low dose of 240 mg twice daily for 16 weeks) combined with rituximab after the failure of several lines of therapy including cladribine with or without rituximab and moxetumomab pasudotox. Two patients achieved minimal residual disease negative complete response after combined treatment with vemurafenib and rituximab, with a hematologic response ongoing after 38 months from the end of treatment in one patient and a relapse of cytopenias occurring after 13 months in the other patient. A third patient normalized her blood counts and this hematologic response, which was not evaluated in the bone marrow at the end of treatment, was lost after 18 months. The last patient died due to infection and multi-organ failure, too early to verify response to vemurafenib. Two patients who had relapsed after vemurafenib and rituximab derived meaningful clinical benefit from retreatment with the same agents, but eventually relapsed again and started indefinite therapy with dabrafenib and trametinib leading to normalization of the blood counts (despite heavy bone marrow infiltration in the only patient so far evaluable in that regard). The outcomes of these cases indicate that novel targeted agents and, in particular, vemurafenib, combined with rituximab, improve the prognosis of HCL patients, even those heavily pretreated with PNAs and moxetumomab pasudotox.

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