Outcomes and second neoplasms in hairy cell leukemia: A retrospective cohort

Silva, Wellington F; Neto, Abel Costa; Rosa, Lidiane Inês da; Siqueira, Isabela Assis de; Duffles, Guilherme; Velloso, Elvira Deolinda Rodrigues Pereira; Rego, Eduardo Magalhães; Rocha, Vanderson; Buccheri, Valéria

doi:10.1016/j.leukres.2019.06.001

Cited by 7 publications

(9 citation statements)

References 37 publications

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“…Patients with HCL are at risk of second malignancies 102–109 . The long‐term OS of patients with HCL must be considered and the drugs we use must be safe and nontoxic.…”

Section: Evaluation Of Secondary Cancer Riskmentioning

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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“…Patients with HCL are at risk of second malignancies 102–109 . The long‐term OS of patients with HCL must be considered and the drugs we use must be safe and nontoxic.…”

Section: Evaluation Of Secondary Cancer Riskmentioning

confidence: 99%

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

2021

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“…The occurrence of secondary cancers in HCL patients is a subject of debate. Data from worldwide cancer registries and studies tend to demonstrate an increased incidence of secondary cancers; This is especially true for hematological malignancies, with cumulative incidence varying from 5% to 32%, probably depending on the time of follow‐up . Observed‐to‐Expected Ratio (OER) of second malignancies is usually increased: 1.01 (CI95%: 0.74‐1.33) .…”

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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“…The association between HCL and second primary malignancies remains controversial [37][38][39][40][41]. The overall incidence of second primary tumors in patients with HCL ranges from 19.9 to 24% [41][42][43][44][45]. The most common include second primary malignancies of the skin, melanoma and non-melanoma skin cancer [43,44].…”

Section: Secondary Skin Neoplasmsmentioning

confidence: 99%

“…The overall incidence of second primary tumors in patients with HCL ranges from 19.9 to 24% [41][42][43][44][45]. The most common include second primary malignancies of the skin, melanoma and non-melanoma skin cancer [43,44]. Existing data suggests that patients with HCL have a higher risk of nonmelanoma skin cancers [45]; therefore, these patients should be closely examined, especially when secondary risk factors exist like fair skin and extensive sun burden.…”

Section: Secondary Skin Neoplasmsmentioning

confidence: 99%

Skin changes in hairy cell leukemia

2020

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Skin lesions have been reported in about 10–12% of hairy cell leukemia (HCL) patients. Most are etiologically related to autoimmune or infectious processes, although secondary cutaneous neoplasms and drug-induced lesions are also reported. However, leukemia cutis with the direct infiltration of the skin by leukemic cells is extremely rare in HCL patients. This paper reviews the epidemiology, pathogenesis, clinical symptoms, diagnosis, and approach to treating skin lesions in HCL. A literature review of the MEDLINE database for articles in English concerning hairy cell leukemia, skin lesions, leukemia cutis, adverse events, infectious, cutaneous, drug reactions, neutrophilic dermatoses, secondary neoplasms, and vasculitis was conducted via PubMed. Publications from January 1980 to September 2020 were scrutinized. Additional relevant publications were obtained by reviewing the references from the chosen articles.

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Outcomes and second neoplasms in hairy cell leukemia: A retrospective cohort

Cited by 7 publications

References 37 publications

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

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

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

Skin changes in hairy cell leukemia

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