Splenectomy followed by administration of rituximab is useful to treat a patient with hairy cell leukemia-variant

Yoshida, Toyonobu; Mihara, Keichiro; Sugihara, Sayaka; Arihiro, Koji; Mino, Tatsuji; Sasaki, Nozomu; Takihara, Yoshihiro

doi:10.1007/s00277-012-1612-1

Cited by 13 publications

(4 citation statements)

References 5 publications

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“…In the elderly, and in those who do not qualify for more intensive treatment, radiotherapy of the spleen may be considered as symptomatic treatment of hypersplenism [55]. Rituximab used after splenectomy may also be an effective treatment method [56]. Another treatment option is Moxe.…”

Section: Hcl Variantmentioning

confidence: 99%

New therapeutic options for hairy cell leukemia

2022

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Hairy cell leukemia (HCL) is a rare B-cell lymphoproliferative disorder characterized by pancytopenia, splenomegaly and increased susceptibility to infections. In 2011, BRAF gene mutation was identified in almost all the patients with the classical type of HCL. The purine analogs cladribine and pentostatin induce long-term remission in the majority of patients, and they remain the standard treatment for this type of leukemia.However, more than half of patients in complete response relapse over the long term, with a quarter of them relapsing within the first five years.Recently, new drugs have been developed and have demonstrated efficacy in refractory or relapsed HCL. The immunotoxin Moxetumomab pasudotox was registered for HCL in 2018.The BRAF kinase inhibitors vemurafenib and dabrafenib, as well as the Bruton kinase inhibitor ibrutinib, are also proven highly effective in clinical trials.

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Section: Hcl Variantmentioning

confidence: 99%

New therapeutic options for hairy cell leukemia

2022

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“…Splenectomy alone can lead to long-lasting partial remission in about two-thirds of patients [30] and maybe the best option because it not only removes the bulk of the disease but also corrects hypersplenism alleviating cytopenias. Moreover, splenectomy followed by Rituximab may be an additional treatment option [39]. HCL-v is resistant to agents effective against HCL, such as cladribine and pentostatin [26].…”

Section: Treatmentmentioning

confidence: 99%

Splenic B-Cell Lymphoma/Leukemia, Unclassifiable

Tizro¹,

Abdulbaki²,

Aggarwal³

et al. 2022

Lymphoma

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Splenic B-cell lymphoma/leukemia, which is unclassifiable, includes low-grade B-cell lymphoproliferative disorders that do not fit into any other splenic lymphoid neoplasm based on current WHO classification. Presently, two provisional entities, splenic diffuse red pulp small B-cell lymphoma (SDRPL) and hairy-cell leukemia variant (HCL-v), are the most recognizable members of this group. SDRPL is an uncommon malignancy representing less than 1% of all non-Hodgkin lymphomas. Frequent clinical manifestations include splenomegaly and lymphocytosis. SDRPL is currently considered a diagnosis of exclusion and requires clinical and paraclinical correlation, including blood smear, bone marrow and spleen morphology, and the correct immunophenotype (typically positive for CD20, DBA.44, and IgG; and negative for CD5, CD10, CD23, CD43, annexin A1, CD11c, CD25, CD103, and CD123), and cytogenetic findings. Cyclin D3 is expressed in the majority of SDRPL in contrast to other types of small B-cell lymphomas. HCL-v is a less common disease accounting for 0.4% of all chronic lymphoproliferative disorders. It resembles classical HCL and SDRPL by diffusely infiltrating the splenic red pulp but is considered biologically unrelated. Splenomegaly and atypical lymphocytosis without monocytopenia are common. Distinguishing features of HCL-v include morphology, immunophenotype (the absence of CD25, CD200, CD123, annexin A1, and TRAP), genotype (wild-type BRAF), and prognosis.

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“…Historical results indicate 74% PRs lasting for 1-10 years (median: four years) (82,83). Rituximab may be used as a consolidation therapy after splenectomy (84). In addition, splenic irradiation can be considered in elderly patients with high surgical risks (80).…”

Section: Morphologically Hcl-v Cells Show Hybrid Features Between Cla...mentioning

confidence: 99%

Hairy Cell Leukemia

Janus¹,

Robak²

2022

Leukemia

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Hairy cell leukemia is a rare, indolent, chronic lymphoid neoplasm originating from a mature B lymphocyte. Diagnosis is based on hairy cell morphology, immunological phenotype by flow cytometry and/or immunohistochemistry in trephine biopsy, and the presence of BRAF V600E somatic mutation. In the classic form of the disease, the purine nucleoside analogues pentostatin and cladribine are recommended for the first-line treatment. These agents induce durable and unmaintained complete response in more than 70% of cases and up to 35% of patients demonstrate overall survival longer than 20 years. When rituximab is combined with cladribine in early relapse, complete response can be achieved in 89-100% of patients, with a three-year risk of relapse of only 7%. More recently, several new drugs have been introduced for the treatment of patients with hairy cell leukemia. Clinical trials have confirmed that the Note to the Reader: This chapter is part of the book Leukemia (ISBN: 978-0-6453320-7-0), scheduled for publication in September 2022. The book is being published by Exon Publications,

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Splenectomy followed by administration of rituximab is useful to treat a patient with hairy cell leukemia-variant

Cited by 13 publications

References 5 publications

New therapeutic options for hairy cell leukemia

New therapeutic options for hairy cell leukemia

Splenic B-Cell Lymphoma/Leukemia, Unclassifiable

Hairy Cell Leukemia

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