Skin changes in hairy cell leukemia

Robak, Ewa; Jesionek-Kupnicka, Dorota; Robak, Tadeusz

doi:10.1007/s00277-020-04349-z

Cited by 22 publications

(18 citation statements)

References 110 publications

(159 reference statements)

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“…13 At diagnosis, HCL is usually characterized by infections, splenomegaly, or the presence of cytopenias. Autoimmune or unusual clinical manifestations are occasionally reported as bulky abdominal lymph nodes, pleural effusions and ascites, skin lesions 14 or destructive bone lesions some of which can mimic multiple myeloma. 15,16 With the high frequency of routine peripheral blood analyses, hairy cells can also be detected in asymptomatic patients.…”

Section: How the Diagnosis Of Hairy Cell Leukemia And Hcl-like Disord...mentioning

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: How the Diagnosis Of Hairy Cell Leukemia And Hcl-like Disord...mentioning

confidence: 99%

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

2021

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“…In 15–30% of cases, patients present with autoimmune disorders [ 33 ], including vasculitis and psoriasis [ [34] , [35] , [36] , [37] ]. The skin is involved in 10–12% of patients with HCL, usually due to autoimmune or infectious etiologies, and direct leukemic involvement of the skin (leukemia cutis) is much rarer [ 38 ]. Bone lesions have also been recognized as a feature of HCL [ [39] , [40] , [41] ].…”

Section: How To Diagnose Hclmentioning

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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“…If symptomatic, they typically present with symptoms related to worsening pancytopenia (fatigue, bruising, gingival bleeding, epistaxis, menorrhagia), splenomegaly (abdominal fullness, discomfort growing after eating) and recurrent infections. Rare clinical manifestations include polyarteritis nodosa, cutaneous leukocytoclastic vasculitis, bone involvement or central nervous system involvement (22,23).…”

Section: Clinical Presentationmentioning

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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Skin changes in hairy cell leukemia

Cited by 22 publications

References 110 publications

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

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

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

Hairy Cell Leukemia

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