Hairy cell leukemia variant: the importance of differential diagnosis

Rudolf-Oliveira, Renata Cristina Messores; Pirolli, Mayara Marin; Souza, Fernanda Santos de; Michels, Juliana; Santos-Silva, Maria Cláudia

doi:10.1016/j.bjhh.2015.01.003

Cited by 3 publications

(3 citation statements)

References 4 publications

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“…Salam and Abdel-Wahab 8 have cited in their studies that in very rare cases, patients with classic HCL may present CBCs with WBC counts greater than 10,000 leukocytes/mm 3 . Rudolf-Oliveira et al 14 , in turn, reported that leukocytosis is a more common finding in patients with HCL-variant. Several authors define that the classification of HCL into classic or variant can be based on criteria such as the patient's WBC count; therefore, according to this criterion, it is possible that the patient in question had classic HCL, however, with some genetic mutation that resulted in an increase in WBC count and consequently led to a fatal outcome 1,3,8 .…”

Section: Discussionmentioning

confidence: 99%

“…According to the clinical laboratory and immunophenotypical criteria, the case presented in this article may refer to classic HCL that could have presented a good prognosis because of the absence of splenomegaly and the patient's young age. However, anemia, thrombocytopenia, high WBC count, the impossibility of investigating the BRAFV600E mutation that could better characterize the classic form of the disease, and the acute evolution of his condition possibly impaired the patient's prognosis, as has been well described in the literature 3,7,8,11,13,14 .…”

Section: Discussionmentioning

confidence: 99%

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Relato de caso incomum de leucemia de células pilosas de evolução aguda em paciente adulto jovem, Amazônia, Brasil

Nascimento¹,

Junior²

2021

Rev. Med. (São Paulo)

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A Leucemia de Células Pilosas (LCP) é uma doença rara, de origem desconhecida, de curso indolente, e que se apresenta no hemograma associada a pancitopenia e linfócitos com projeções citoplasmáticas. No presente estudo apresentamos o relato de caso de um paciente de 31 anos, masculino, hipocorado, sem esplenomegalia, admitido no Hospital Municipal de Rondon do Pará, Amazônia, no dia 31.12.2018 para a realização de exames laboratoriais em função de rebaixamento do estado geral. O hemograma revelou: anemia (7.5g/dL de hemoglobina e 20% de hematócrito), plaquetopenia (28.200/mm3) e hiperleucocitose (179.000/mm3) as custas de 88% de linfócitos com projeções citoplasmáticas finas e alongadas sugestivas de células pilosas. No dia 07.01.2019 houve piora do quadro do paciente com evolução a óbito antes mesmo da confirmação diagnóstica pelo exame de imunofenotipagem que revelou tratava-se de população clonal de 68,3% de células pilosas com expressão dos antígenos: CD19, CD20, CD79b, CD23, IgM, CD200, CD38, FMC-7, CD25 e CD103. Não tendo sido possível a determinação de quais fatores foram determinantes para esse quadro.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Relato de caso incomum de leucemia de células pilosas de evolução aguda em paciente adulto jovem, Amazônia, Brasil

Nascimento¹,

Junior²

2021

Rev. Med. (São Paulo)

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“…In comparison to HCL, patients with an HCL variant (HCL-v) are often older, present with lymphocytosis, and are resistant to common treatments using purine nucleoside analogs [ 3 , 4 ]. Typically, patients with HCL-v are treated with more aggressive therapies often containing anthracycline and rituximab [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

A Unique Hairy Cell Leukemia Variant

Jian

Hsia

2016

Case Rep Oncol

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A 65-year-old woman presented with easy bruising, left upper quadrant pain, decreased appetite, and weight loss. She had splenomegaly and lymphocytosis (lymphocyte count of 11.6 × 10⁹/l), with remarkably abnormal appearing morphology. Her hemoglobin and platelet counts were normal. Peripheral blood flow cytometry revealed a monoclonal B-cell population expressing CD11c, CD25, CD19, CD20, and CD103. An initial diagnosis of hairy cell leukemia (HCL) was made, and the patient was treated with a standard 5-day course of cladribine. However, her lymphocytosis improved transiently, with a relapse 4 months later. There was no improvement in her splenomegaly. An HCL variant (HCL-v) was considered based on her resistance to treatment with a purine nucleoside analog. A subsequent splenectomy improved symptoms. Two years after, the patient suffered a relapse and underwent 6 cycles of CHOP-R (cyclophosphamide, hydroxydaunomycin, oncovin, prednisone, and rituximab), achieving partial remission. While under observation, she progressed with lymphocytosis 6 months later and was treated with pentostatin. There was no significant improvement in her disease, and she died 8 weeks following treatment initiation. HCL-v is a clinically more aggressive mature B-cell lymphoma than HCL with worse splenomegaly, higher lymphocyte counts, and resistance to typical HCL therapy with purine nucleoside analogs. Early recognition of HCL-v in the history, physical examination, and investigations with morphology and flow cytometry is key to patient management. Further, as in our case of HCL-v, cell morphology can be distinctly atypical, with large nucleoli and extremely convoluted nuclei. The distinction between HCL and HCL-v is important as HCL-v patients require more aggressive therapy and closer follow-up.

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