Characteristics and treatment patterns among US patients with hairy cell leukemia: a retrospective claims analysis

Divino, Victoria; Karve, Sudeep; Gaughan, Andrew; DeKoven, Mitch; Gao, Guozhi; Knopf, Kevin; Lanasa, Mark C.

doi:10.2217/cer-2017-0014

Cited by 14 publications

(15 citation statements)

References 21 publications

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“…Both show that the mean age at diagnosis in the Jewish population to be 55 years, similar to that recorded in most other Western countries (3), while the mean age at diagnosis was slightly younger, 49 years, in the Arab population. HCL was also more frequent in Jews (88% of the entire cohort), which is similar to that encountered in chronic lymphocytic leukemia, which is also more common in Ashkenazi Jews (23)(24)(25). In this respect a genome-wide association study is indeed still needed to confirm these different ethnicity patterns.…”

Section: Discussionsupporting

confidence: 54%

Hairy Cell Leukemia: Retrospective Analysis of Demographic Data and Outcome of 203 Patients from 12 Medical Centers in Israel

Inbar¹,

Herishanu

Goldschmidt

et al. 2018

Anticancer Res

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Background/Aim: In this retrospective study, we summarized the national Israeli experience with hairy cell leukemia (HCL) in a large cohort of patients with a long followup. Patients and Methods: Demographic data, and relevant laboratory and clinical parameters were analyzed, emphasizing the outcome after first-line treatment with cladribine. Results: Data on 203 patients was collected from 12 medical centers during 1985-2015. Mean and median follow-up were 7.5 years and 5.18 years (interquartile range=0.1-40 years), and 5-and 10-year survival were 96% and 90.62%, respectively. The median age of diagnosis was 55.5 years for Jews and 49 years for Arabs (p=0.021), and most patients were males (81.77%); 52.2% were Ashkenazi Jews, 36.1% Sephardic Jews and 11.7% were Arab, Druze or other ethnicity. Cladribine was given to 159 patients (80.7%%) and most (62%) received intravenous (i.v.) and 38% received subcutaneous (s.c.) therapy. Overall survival and time to next treatment were not significantly different between the two schedules (i.v., s.c.). In univariate analysis of a variety of factors, only age >65 years had a negative impact on outcome, with shorter overall survival. It is of interest that Arab patients with HCL were diagnosed at an earlier age, but had a similar clinical course and outcome to both Ashkenazi and Sephardic Jews. Hairy cell leukemia (HCL) is an uncommon indolent B-cell lymphoproliferative disorder occurring in 3/1,000,000 patients (1). It was first described by Bouroncle et al. in 1958 as "leukemic reticuloendotheliosis"(2) and only later was the term HCL first used describing the characteristic 'hairy' surface features of the leukemia cells. Clinically, the disorder is characterized by pancytopenia, splenomegaly and recurrent infections (3). It is more common in men (4:1 men/women ratio), with a median age at diagnosis of 55 years (4). Diagnosis of HCL is usually made by histopathology after bone marrow biopsy and in combination with a characteristic flow cytometric profile showing CD19 + , CD20 + , CD11c + , CD25 + , CD103 + , CD123 + , CD200 + , CD27 − , and light chain restriction (3). In 2011, molecular studies by Tiacci et al. showed that almost all cases of HCL were associated with a 6423

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Section: Discussionsupporting

confidence: 54%

Hairy Cell Leukemia: Retrospective Analysis of Demographic Data and Outcome of 203 Patients from 12 Medical Centers in Israel

Inbar¹,

Herishanu

Goldschmidt

et al. 2018

Anticancer Res

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“…Rituximab was used as a historical control to determine sample size, as it was the most frequently used nonchemotherapy treatment in relapsed/refractory HCL with a CR rate of 13% in the largest study [ 12 , 22 ]. A sample size of 77 patients was planned to provide 90% power to detect a difference between 13% and 28% in durable complete response rates using a 2-sided significance level of 0.05.…”

Section: Methodsmentioning

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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“…No randomized trials have compared both drugs, and there is no evidence to date in the literature proving the superiority of one drug over the other. In a large representative US database including 749 HCL patients, cladribine was utilized in more than 75% of patients requiring first‐line treatment . In two large retrospective series including both agents, 76%‐83% of patients receiving PNAs achieved complete response (CR) and 31%‐33% partial response (PR), with no significant difference in CR or PR observed between both agents.…”

Section: What Has Recently Improved Understanding Of Hairy Cell Leukementioning

confidence: 99%

Hairy cell leukemia: 2020 update on diagnosis, risk stratification, and treatment

2019

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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. They are 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 three or four based on the CD11C, CD103, CD123, and CD25 expression. Also, 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. The VH4-34 positive HCL cases are associated with poor prognosis. Treatment: Risk adapted therapy with purine nucleoside analogs (PNA) are indicated in symptomatic first line HCL patients. The use of PNA followed by rituximab represents an alternative option. Management of progressive or refractory disease is based on the use of BRAF inhibitors associated or not with MEK inhibitors, recombinant immunoconjugates targeting CD22 or BCR inhibitors.

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Characteristics and treatment patterns among US patients with hairy cell leukemia: a retrospective claims analysis

Cited by 14 publications

References 21 publications

Hairy Cell Leukemia: Retrospective Analysis of Demographic Data and Outcome of 203 Patients from 12 Medical Centers in Israel

Hairy Cell Leukemia: Retrospective Analysis of Demographic Data and Outcome of 203 Patients from 12 Medical Centers in Israel

Moxetumomab pasudotox in relapsed/refractory hairy cell leukemia

Hairy cell leukemia: 2020 update on diagnosis, risk stratification, and treatment

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