Hairy cell leukemia and COVID-19 adaptation of treatment guidelines

Grever, Michael R.; Andritsos, Leslie A.; Banerji, Versha; Barrientos, Jacqueline C.; Bhat, Seema A.; Blachly, James S.; Call, Timothy G.; Cross, Matt; Dearden, Claire; Demeter, Judit; Dietrich, S.; Falini, Brunangelo; Forconi, Francesco; Gladstone, Douglas E.; Gozzetti, Alessandro; Iyengar, Sunil; Johnston, James B.; Juliusson, Gunnar; Kraut, Eric H.; Kreitman, Robert J.; Lauria, Francesco; Lozanski, Gerard; Parikh, Sameer A.; Park, Jae; Polliack, Aaron; Ravandi, Farhad; Robak, Tadeusz; Rogers, Kerry A.; Saven, Alan; Seymour, John F.; Tadmor, Tamar; Tallman, Martin S.; Tam, Constantine S.; Tiacci, Enrico; Troussard, Xavier; Zent, Clive S.; Zenz, Thorsten; Zinzani, Pier Luigi; Wörmann, Bernhard

doi:10.1038/s41375-021-01257-7

Cited by 35 publications

(36 citation statements)

References 50 publications

(41 reference statements)

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“…Attempts to control the infection should be pursued prior to instituting the PNA. If it is not possible to control the infection 73 or in the event of a health crisis (Sars‐Cov‐2), 74 the use of alpha‐interferon (IFN) or vemurafenib as bridging therapy could be required transiently. Note that the use of IFN, a possible alternative in pregnant women, becomes more and more difficult due to a production stop by the laboratories.…”

Section: Update On Hcl Treatment and Hcl‐like Disordersmentioning

confidence: 99%

Hairy cell leukemia 2022: Update on diagnosis, risk‐stratification, and treatment

2021

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Disease Overview: Hairy cell leukemia (HCL) and HCL-like disorders, including HCL variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogeneous group of mature lymphoid B-cell disorders characterized by the identification of hairy cells, a specific genetic profile, a different clinical course, and the need for appropriate treatment. Diagnosis: Diagnosis of HCL is based on morphological evidence of hairy cells, an HCL immunologic score of 3 or 4 based on the CD11C, CD103, CD123, and CD25 expression, the trephine biopsy which makes it possible to specify the degree of tumoral medullary infiltration and the presence of BRAF V600E somatic mutation. Risk Stratification: Progression of patients with HCL is based on a large splenomegaly, leukocytosis, a high number of hairy cells in the peripheral blood, and the immunoglobulin heavy chain variable region gene mutational status. VH4-34-positive HCL cases are associated with a poor prognosis. Treatment: Patients should be treated only if HCL is symptomatic. Chemotherapy with risk adapted therapy purine analogs (PNAs) are indicated in first-line HCL patients. The use of chemo-immunotherapy combining PNAs and rituximab (R) represents an increasingly used therapeutic approach. Management of relapsed/ refractory disease is based on the use of BRAF inhibitors (BRAFi) plus rituximab or MEK inhibitors (MEKi), recombinant immunoconjugates targeting CD22 or BrutonTyrosine Kinase inhibitors (BTKi). However, the optimal sequence of the different treatments remains to be determined. The Bcl2-inhibitors (Bcl-2i) can play a major role in the future. | INTRODUCTIONHairy cell leukemia (HCL) is recognized as an entity by the World Health Organization since 2008 1 and in the last 2017 revision of the WHO classification of lymphoid neoplasms. 2 HCL, four to five times more frequent in men than women, accounts for 2% of all leukemias with approximately 1.100 new HCL cases in the United States. 3 The few available population-based studies are limited. [4][5][6][7][8] When including HCL and HCL-like disorders, the overall age-adjusted to the 2000 US population incidence rate is 0.7 per 100 000 in men and 0.3/100 000 in women. When adjusting to the worldwide population, the incidence rate of HCL is 0.3 and 0.1/100 000, respectively, and remains relatively stable over time. 8 It is lower in non-Hispanic/black, Hispanic, or Asian/pacific Islander. Despite an improvement of overall (OS) and relative survival (RS), a significantly lower OS is observed among African American individuals compared with other ethnic groups. 6 The mortality rates for patients with HCL is similar to those of the general population 5 years after diagnosis. 9 HCL must be differentiated from other

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Section: Update On Hcl Treatment and Hcl‐like Disordersmentioning

confidence: 99%

Hairy cell leukemia 2022: Update on diagnosis, risk‐stratification, and treatment

2021

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“…The recent coronavirus disease 2019 (Covid‐19) pandemic has posed new treatment challenges, especially at disease onset. Due the highly immunosuppressive effect of purine analogues that may induce an increased susceptibility to a more severe course of Covid‐19 infection, it has been recommended to establish an active surveillance in patients with low but stable blood counts, while considering the use of targeted agents – albeit applied “off‐label” – as first‐line options, given their reduced immunosuppressive potential 17 …”

Section: Introductionmentioning

confidence: 99%

“…Due the highly immunosuppressive effect of purine analogues that may induce an increased susceptibility to a more severe course of Covid-19 infection, it has been recommended to establish an active surveillance in patients with low but stable blood counts, while considering the use of targeted agentsalbeit applied "off-label"as first-line options, given their reduced immunosuppressive potential. 17 This works presents our institutional experience with the treatment of HCL patients over a period of time of thirty years, which covers all the approaches applied for the treatment of this disease, ranging from splenectomy, to purine analogues and newer targeted agents.…”

Section: Introductionmentioning

confidence: 99%

The treatment of hairy cell leukemia with a focus on long lasting responses to cladribine: A 30‐year experience

et al. 2021

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The treatment of hairy cell leukemia (HCL) has considerably changed over years.Purine analogues, namely cladribine, now represent the treatment of choice. One hundred and eighty-four patients were followed between 1986 and 2018 and treated according to era-specific guidelines. Responses were classified by combining Consensus Resolution criteria and marrow immunohistochemistry. Patients were grouped according to the number of treatment lines they received. Patients treated first line responded in 86% of cases, with complete response (CR) in 44% of cases.Response rates remained high throughout the first four lines (84%, 81%, 79% for the second line onward, with CR in 38%, 37%, 15% of cases respectively). One hundred and twenty-two patients received cladribine as first line treatment, with a response rate of 86% and a CR rate of 54%. Among the 66 CR patients, 45 (68%) have never received further therapy: 11 patients are in continuous CR between 5 and 10 years after treatment, 14 between 10 and 20 years and three patients at more than 20 years. Median time-to-next treatment (TTNT) for frontline cladribine-treated patients was 8.2 years: partial responders had a significantly shorter median TTNT than CR patients (5.3 years vs median not reached at 25.8 years, p < 0.001). Patients with HCL require subsequent lines of therapy in more than 50% of cases. Purine analogues allow significant response rates when applied first line and upon retreatment. Some patients may enjoy long lasting treatment-free intervals after one course of cladribine.

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“…A case report describes a 54-year-old male with relapsed HCL 10 years after initial treatment who experienced respiratory failure from COVID-19 and had a difficult recovery [ 215 ]. A consensus document recommended caution in treating HCL patients with immunosuppressive agents during the COVID-19 pandemic, including both purine analogs which deplete T-cells and normal B-cells, and CD20 Mabs like rituximab which induce prolonged depletions of normal B-cells [ 216 ]. In addition to placing COVID-19 infected HCL patients at increased risk for adverse outcomes, immunosuppressive treatments pose a second potential problem, namely preventing effective vaccination.…”

Section: Treating Hcl In the Era Of Covid-19mentioning

confidence: 99%

“…In addition to placing COVID-19 infected HCL patients at increased risk for adverse outcomes, immunosuppressive treatments pose a second potential problem, namely preventing effective vaccination. For this reason, the consensus document recommends that vaccinated HCL patients obtain antibody testing to confirm immunity [ 216 ]. It is known that rituximab prevents effective vaccination to influenza vaccine at least 6 months after the last dose [ 217 ], but data are not yet available for HCL patients.…”

Section: Treating Hcl In the Era Of Covid-19mentioning

confidence: 99%

Diagnosis and treatment of hairy cell leukemia as the COVID-19 pandemic continues

Kreitman

Arons

2022

Blood Reviews

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Hairy cell leukemia (HCL) is an indolent B-cell malignancy, usually driven by the BRAF V600E mutation. For 30 years, untreated and relapsed HCL was successfully treated with purine analogs, but minimal residual disease (MRD) remained in most patients, eventually causing relapse. Repeated purine analogs achieve decreasing efficacy and increasing toxicity, particularly to normal T-cells. MRD-free complete remissions (CRs) are more common using rituximab with purine analogs in both 1 st -line and relapsed settings. BRAF inhibitors and Ibrutinib can achieve remission, but due to persistence of MRD, must be used chronically to prevent relapse. BRAF inhibition combined with Rituximab can achieve high MRD-free CR rates. Anti-CD22 recombinant immunotoxin moxetumomab pasudotox is FDA-approved in the relapsed setting and is unique in achieving high MRD-free CR rates as a single-agent. Avoiding chemotherapy and rituximab may be important in ensuring both recovery from COVID-19 and successful COVID-19 vaccination, an area of continued investigation.

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Hairy cell leukemia and COVID-19 adaptation of treatment guidelines

Cited by 35 publications

References 50 publications

Hairy cell leukemia 2022: Update on diagnosis, risk‐stratification, and treatment

Hairy cell leukemia 2022: Update on diagnosis, risk‐stratification, and treatment

The treatment of hairy cell leukemia with a focus on long lasting responses to cladribine: A 30‐year experience

Diagnosis and treatment of hairy cell leukemia as the COVID-19 pandemic continues

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