Skin changes in hairy cell leukemia
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REVIEW ARTICLE
Skin changes in hairy cell leukemia Ewa Robak 1
&
Dorota Jesionek-Kupnicka 2
&
Tadeusz Robak 3,4
Received: 26 September 2020 / Accepted: 11 November 2020 # The Author(s) 2020
Abstract 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. Keywords Adverse drug reactions . Cladribine . Interferon . Skin neoplasms . Infectious . Hairy cell leukemia . Leukemia cutis . Skin . Cutaneous . Neutrophilic dermatoses . Secondary cancer . Melanoma . Vasculitis . Vemurafenib
Introduction Hairy cell leukemia (HCL) is a rare form of B cell indolent lymphoid leukemia involving mature and post-germinal center B lymphocytes, generally affecting the bone marrow, peripheral blood, and spleen. The disease comprises 2–3% of all leukemias [1, 2]. There is a 4:1 male predominance, and Caucasians are more frequently affected than other ethnic groups. The annual incidence is between 2.9 and 4.7 per million people per year [3–5]. HCL is characterized by progressive pancytopenia, splenomegaly, and hypercellular bone marrow. Lymph node infiltration and involvement of extranodal organs are rather infrequent. Rare clinical manifestations of HCL are occasionally reported [6–8]. These include bulky abdominal lymphadenopathy; tumor masses in the
* Tadeusz Robak [email protected]; [email protected] 1
Department of Dermatology, Medical University of Lodz, Lodz, Poland
2
Department of Pathology, Chair of Oncology, Medical University of Lodz, Lodz, Poland
3
Department of Hematology, Medical University of Lodz, Lodz, Poland
4
Copernicus Memorial Hospital, Ul. Ciolkowskiego 2, Lodz, Poland
mediastinum; paravertebral masses; pleural effusion and ascites; skin lesions; ocular-corneal involvement; uveitis; retinal artery thrombosis; neurological-meningeal compression; esophageal, gastric, and hepatic involvement; and skeletal lesion [1, 2]. Leukemic cells may also be identified in peripheral blood as mononuclear cells with abundant, slightly basophilic cytoplasm and circumferential cytoplasmic “hairy” projection. HCL cells typically show a distinctive immunophenotype coexpressing CD19, CD20, CD11c, CD25, CD103, and CD123. Recently, BRAF-V600E mutation has
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