How I manage patients with hairy cell leukaemia

Thompson, Philip A.; Ravandi, Farhad

doi:10.1111/bjh.14524

Cited by 32 publications

(36 citation statements)

References 94 publications

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“…Patients with hairy cell leukemia (HCL), a rare B cell malignancy characterized by high CD22 expression, typically present with pancytopenia and increased susceptibility to infection [ 1 ]. Although many patients achieve long-term complete remission with the purine nucleoside analogs pentostatin or cladribine [ 2 – 4 ], ~50% will relapse by 16 years and require additional treatment [ 5 ]. In later lines, purine nucleoside analogs offer lower complete response rate, shorter duration of response, and higher risk of cumulative toxicity compared with earlier treatment [ 5 – 7 ].…”

Section: Introductionmentioning

confidence: 99%

Moxetumomab pasudotox in relapsed/refractory hairy cell leukemia

et al. 2018

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This is a pivotal, multicenter, open-label study of moxetumomab pasudotox, a recombinant CD22-targeting immunotoxin, in hairy cell leukemia (HCL), a rare B cell malignancy with high CD22 expression. The study enrolled patients with relapsed/refractory HCL who had ≥2 prior systemic therapies, including ≥1 purine nucleoside analog. Patients received moxetumomab pasudotox 40 µg/kg intravenously on days 1, 3, and 5 every 28 days for ≤6 cycles. Blinded independent central review determined disease response and minimal residual disease (MRD) status. Among 80 patients (79% males; median age, 60.0 years), durable complete response (CR) rate was 30%, CR rate was 41%, and objective response rate (CR and partial response) was 75%; 64 patients (80%) achieved hematologic remission. Among complete responders, 27 (85%) achieved MRD negativity by immunohistochemistry. The most frequent adverse events (AEs) were peripheral edema (39%), nausea (35%), fatigue (34%), and headache (33%). Treatment-related serious AEs of hemolytic uremic syndrome (7.5%) and capillary leak syndrome (5%) were reversible and generally manageable with supportive care and treatment discontinuation (6 patients; 7.5%). Moxetumomab pasudotox treatment achieved a high rate of independently assessed durable response and MRD eradication in heavily pretreated patients with HCL, with acceptable tolerability.

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Section: Introductionmentioning

confidence: 99%

Moxetumomab pasudotox in relapsed/refractory hairy cell leukemia

et al. 2018

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“…In general, the recommendation is to consider MGFs on a case-by-case basis in patients with active infections. 57,58 However, the rate of neutropenia is high, ranging from 30% to 50%, 59 which meets the threshold for recommended use of growth factors in the NCCN Guidelines for MGFs. These guidelines suggest the use of growth factors for regimens that are associated with a high risk of febrile neutropenia (>20%) or an with intermediate risk (10%-20%) in patients who have additional risk factors, such as age >65 years, hepatic or renal dysfunction, prior chemoradiotherapy, tumor involvement of the bone marrow, or recent surgery.…”

Section: Hairy Cell Leukemiamentioning

confidence: 99%

Controversies Regarding Use of Myeloid Growth Factors in Leukemia

Poston

Becker

2017

J Natl Compr Canc Netw

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This review focuses on the data supporting the use of myeloid growth factors (MGFs) in patients being treated for acute myeloid leukemia, acute lymphoblastic leukemia, chronic myeloid leukemia, and hairy cell leukemia, for which neutropenia is a common complication of treatment. However, due to the lack of randomized trial data or conflicting results of clinical studies, comprehensive guidelines have been difficult to formulate. Moreover, to date, these diagnoses have not been addressed in the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for MGFs. However, in most cases, the general principles have been included in the applicable NCCN Guidelines for each individual cancer site.

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“…Diğer bir pürin analogu olan 2-Deoxycoformycin (pentostatin) ile de benzer sonuçlar bildirilmiştir (7). Bu iki ajan arasında çapraz rezistans yoktur.…”

Section: Bulgularunclassified

Clinical Characteristics and Outcome in Patients with Hairy Cell Leukemia: a Single Center Experience

Kelkitli¹,

Meletli²,

Turğut³

2017

LLM Dergi

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Amaç: Tüylü hücreli lösemi (THL) nadir görülen, pansitopeni ve splenomegali ile karakterize klonal, kronik B hücreli lenfoproliferatif bir hastalıktır. Bu çalışmanın amacı retrospektif olarak THL'li hastaların klinik özelliklerini ve tedavi sonuçlarını değerlendirmektir. Hastalar ve Yöntem: Bu çalışmada kliniğimizde Mart 2011-Eylül 2017 tarihleri arasında THL tanısı ile tedavi edilen 25 hastayı retrospektif olarak değerlendirdik. Bulgular: Hastaların 5 (%20)'i kadın, 20 (%80)'si erkekti. Ortanca yaş 67 (38-84) idi. Hastaların %80'inde splenomegali mevcuttu. Pansitopeni ile başvuran hasta sayısı %68 idi. Tedavi öncesi ortalama beyaz küre 3140 ± 7347 mm 3 , hemoglobin 10.9 ± 2.5 g/dL, trombosit 84.000 ± 75.425 mm 3 , monosit 40 ± 192 mm 3 idi. Hastaların %90'ında monositopeni mevcuttu. Tartarat-rezistan asit fosfataz (TRAP) pozitifliği %60 görülürken, BRAF V600E %20 pozitif, %20 negatif idi. Ancak hastaların %60'ında BRAFV600E çalışılamadı. Tüm hastalarımız tedavide kladribin 0.1 mg/kg/gün 7 gün intravenöz almışlardı. Hastaların tamamı bir kür kladribin tedavisi ile tam remisyon elde etmişti. Hastaların 5 (%20)'inde relaps görülmüştü. Relaps görülene kadar geçen ortalama süre 33 (min: 0, maks: 69) aydı. Relaps gözlenen hastalar tekrar 0.1 mg/kg 7 gün kladribin tedavisi ve Sağlık Bakanlığından endikasyon dışı onayı alınarak rituksimab 375 mg/m 2 /hafta 4 hafta boyunca tedavi edildi. Üç hasta sepsis nedeni ile eks oldu. Ortalama takip süresi 56 (min: 1, maks: 71) ay idi. Sonuç: Splenomegali ve sitopeniler ile başvuran hastalarda THL mutlaka akılda tutulmalıdır. Monositopeninin THL için spesifik bir laboratuvar bulgusu olduğu bilinmelidir. Çalışmamızda da gösterildiği gibi bir kürlük kladribin tedavisi ile oldukça yüz güldürücü yanıtlar elde edilmektedir.

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How I manage patients with hairy cell leukaemia

Cited by 32 publications

References 94 publications

Moxetumomab pasudotox in relapsed/refractory hairy cell leukemia

Moxetumomab pasudotox in relapsed/refractory hairy cell leukemia

Controversies Regarding Use of Myeloid Growth Factors in Leukemia

Clinical Characteristics and Outcome in Patients with Hairy Cell Leukemia: a Single Center Experience

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