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Keywords: Differential diagnosis; BRAF V600E mutation; hairy Cell Leukaemia; Hairy cell variant; therapy EN Differential diagnosis BRAF V600E mutation hairy Cell Leukaemia Hairy cell variant therapy 730 737 8 12/07/20 20201201 NES 201201 Methodology This guideline was compiled according to the BSH process (https://b-s-h.org.uk/guidelines). Cytoplasm is abundant and pale blue, with circumferential "hairy" projections.9 Discrete cytoplasmic vacuoles or rod-shaped inclusions (ribosome lamellar complexes) may be seen.3 Bone marrow aspirate and trephine biopsy Bone marrow aspiration is frequently unsuccessful, reflecting fibrosis induced by the hairy cell infiltrate. In a single-centre follow-up study of 242 patients,41 18 patients treated with cladribine (12 as first-line and six as second-line therapy) who remained in PR after bone marrow reassessment received a repeat treatment with cladribine 4-7 months after initial treatment, leading to CR in 14 patients, of whom all but one remained in CR at a median follow-up of 6 years. Treatment of relapsed or refractory classical HCL Despite the durable remissions achieved with front-line therapy, approximately 50% of patients will relapse. [Extracted from the article]

The t(14;19)(q32;q13), involving the BCL3 locus at chromosome 19q13 and the immunoglobulin heavy chain gene at 14q32, is a rare recurrent cytogenetic abnormality identified in B-cell neoplasms, most of which have been classified as chronic lymphocytic leukaemia (CLL) in the literature. We describe the clinicopathological, immunophenotypic and cytogenetic findings in seven patients with B-cell neoplasms associated with t(14;19)(q32;q13). There were five men and two women, with a median age of 48 years (range 33–68). All had absolute lymphocytosis, six had lymphadenopathy, and one had splenomegaly. Lymphocytes in blood and bone marrow aspirate smears were predominantly small and cytologically atypical. Flow cytometric immunophenotyping showed an atypical immunophenotype with low CLL scores. The growth pattern in bone marrow biopsy specimens was interstitial to diffuse; immunohistochemical stains were positive for bcl3 and negative for cyclin D1. Lymph node biopsy specimens of two patients revealed total architectural effacement by neoplasm with proliferation centres. In addition to t(14;19), cytogenetic studies demonstrated trisomy 12 in five patients. These results suggest that B-cell neoplasms with the t(14;19)(q32;q13) present frequently as leukaemia composed of small B-lymphocytes and share many features with CLL. However, these neoplasms also differ from CLL cytologically and in their immunophenotype. [ABSTRACT FROM AUTHOR]