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Search Results (3)

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Keywords = hairy cell leukaemia

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12 pages, 10633 KiB  
Review
Splenic Diffuse Red Pulp Small B-Cell Lymphoma with Overlapping Clinical and Immunophenotypic Features with Hairy Cell Leukaemia: A Case Report and a Review of the Literature
by Mirette Hanna, Michola Trus and Erica DiMaria
Genes 2025, 16(4), 467; https://doi.org/10.3390/genes16040467 - 19 Apr 2025
Viewed by 260
Abstract
Background: Splenic B-cell lymphomas and leukaemias include hairy cell leukaemia, splenic marginal zone lymphoma, splenic diffuse red pulp small B-cell lymphoma, and splenic B-cell lymphoma/leukaemia with prominent nucleoli. The main diagnostic challenge is to differentiate between splenic B-cell lymphomas and leukaemias due to [...] Read more.
Background: Splenic B-cell lymphomas and leukaemias include hairy cell leukaemia, splenic marginal zone lymphoma, splenic diffuse red pulp small B-cell lymphoma, and splenic B-cell lymphoma/leukaemia with prominent nucleoli. The main diagnostic challenge is to differentiate between splenic B-cell lymphomas and leukaemias due to highly overlapping clinical, morphologic, and phenotypic features in the absence of splenectomies for diagnostic purposes. Case presentation: We describe a case of a 78-year-old woman who presented with weight loss and was subsequently found to have pancytopenia, lymphocytosis, and splenomegaly. Peripheral blood smear showed a homogenous population of small- to medium-sized lymphocytes having oval nuclei, condensed chromatin, and villous cytoplasmic projections. Bone marrow showed B-cell infiltrate in a predominantly intrasinusoidal pattern. Except for cyclin D1 and BRAF, the immunophenotype was similar to that of hairy cell leukaemia. This was further supported by the lack of BRAF p.V600E mutation by polymerase chain reaction. A diagnosis of splenic diffuse red pulp small B-cell lymphoma was thus favored based on the lack of cyclin D1 expression and pattern of infiltration in the bone marrow biopsy. Conclusions: Awareness of this infrequent clinical presentation and immunophenotype of splenic diffuse red pulp small B-cell lymphoma is crucial for diagnosis and devising appropriate therapeutic strategies for the patient. Full article
(This article belongs to the Section Human Genomics and Genetic Diseases)
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23 pages, 2041 KiB  
Review
The Genomics of Hairy Cell Leukaemia and Splenic Diffuse Red Pulp Lymphoma
by David Oscier, Kostas Stamatopoulos, Amatta Mirandari and Jonathan Strefford
Cancers 2022, 14(3), 697; https://doi.org/10.3390/cancers14030697 - 29 Jan 2022
Cited by 10 | Viewed by 4285
Abstract
Classical hairy cell leukaemia (HCLc), its variant form (HCLv), and splenic diffuse red pulp lymphoma (SDRPL) constitute a subset of relatively indolent B cell tumours, with low incidence rates of high-grade transformations, which primarily involve the spleen and bone marrow and are usually [...] Read more.
Classical hairy cell leukaemia (HCLc), its variant form (HCLv), and splenic diffuse red pulp lymphoma (SDRPL) constitute a subset of relatively indolent B cell tumours, with low incidence rates of high-grade transformations, which primarily involve the spleen and bone marrow and are usually associated with circulating tumour cells characterised by villous or irregular cytoplasmic borders. The primary aim of this review is to summarise their cytogenetic, genomic, immunogenetic, and epigenetic features, with a particular focus on the clonal BRAFV600E mutation, present in most cases currently diagnosed with HCLc. We then reflect on their cell of origin and pathogenesis as well as present the clinical implications of improved biological understanding, extending from diagnosis to prognosis assessment and therapy response. Full article
(This article belongs to the Special Issue Genomics of Rare Hematologic Cancers)
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5 pages, 615 KiB  
Case Report
A Case of Hemophagocytic Lymphohistiocytosis Triggered by Disseminated Tuberculosis and Hairy Cell Leukaemia after SARS-CoV2 Infection
by Alessandro Cellini, Andrea Visentin, Massimiliano Arangio Febbo, Susanna Vedovato, Serena Marinello, Ivo Tiberio, Livio Trentin and Carmela Gurrieri
Appl. Sci. 2022, 12(2), 564; https://doi.org/10.3390/app12020564 - 7 Jan 2022
Viewed by 1589
Abstract
Hemophagocytic Lymphohistiocytosis (HLH) is a rare but life-threatening disease that can occur either as a primary condition or as a consequence of a variety of triggers, including infectious diseases. Here we present a case of secondary HLH triggered by systemic Mycobacterium tuberculosis infection [...] Read more.
Hemophagocytic Lymphohistiocytosis (HLH) is a rare but life-threatening disease that can occur either as a primary condition or as a consequence of a variety of triggers, including infectious diseases. Here we present a case of secondary HLH triggered by systemic Mycobacterium tuberculosis infection in a 59-year-old immunocompromised Hairy Cell Leukemia and previous SARS-CoV2 infected patient. This case report underlines the role of Etoposide-based chemotherapy in treating the severe inflammation that is the defining factor of HLH, suggesting how, even when such therapy is not effective, it may still give the clinicians time to identify the underlying condition and start the appropriate targeted therapy. Moreover, it gives insight on our decision to treat the underlying haematological condition with a BRAF-targeted therapy rather than purine analog-based chemotherapy to reduce the risk of future severe infections. Full article
(This article belongs to the Special Issue Improving Diagnosis and Therapy of Lymphoproliferative Diseases: Latest Advances and Prospects)
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