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Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2022 Jun 15. Epub 2022 Jun 15.

4th Department of Internal Medicine - Haematology, Faculty of Medicine in Hradec Kralove, Charles University in Prague and University Hospital, Hradec Kralove, Czech Republic.

Background: Hairy cell leukemia (HCL) is a rare indolent lymphoproliferative disease with an accumulation of mature B lymphocytes with fine reticular chromatin and cytoplasm with typical hairy-like cytoplasmic projections. Rarely, hairy cell leukemia manifests as a lung infiltration. The differential diagnosis between infection and malignant involvement with hairy cell leukemia is often challenging in such situations. Read More

Expert Opin Pharmacother 2022 Jun 15:1-3. Epub 2022 Jun 15.

Department of Internal Medicine, Eisenhower Health, Rancho Mirage, CA, USA.

Cureus 2022 May 13;14(5):e24976. Epub 2022 May 13.

Hematology/Oncology, Veteran's Affairs Hospital, Memphis, USA.

Classical hairy cell leukemia (cHCL) and related mature lymphoid B-cell neoplasms including hairy cell leukemia variant (HCLv) and splenic diffuse red pulp lymphoma (SDRPL) are a rare subset of lymphoid neoplasms. cHCL accounts for around 2% of all leukemias and is characterized by a peripheral smear with large lymphoid cells with cytoplasmic projections giving the cells a hairy appearance, splenomegaly, and cytopenias. Majority of cHCL cases harbor a BRAFV600E mutation. Read More

Zhonghua Xue Ye Xue Za Zhi 2022 May;43(5):435

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.

Blood Adv 2022 May 18. Epub 2022 May 18.

University of Bologna, Italy.

Cladribine is regarded as the first treatment of choice of symptomatic hairy cell leukemia (HCL): it provides high rates of response and very long duration of remission in some cases. Disease-specific patients records have been reviewed at four European centers of excellence; all patients with HCL requiring treatment who received frontline cladribine have been extrapolated. Responses have been classified according to Consensus Resolution Criteria. Read More

Cancer Sci 2022 May 17. Epub 2022 May 17.

Division of Molecular Target and Gene Therapy Products, National Institute of Health Sciences, Kawasaki, Kanagawa, Japan.

BRAF mutations are frequently observed in melanoma and hairy-cell leukemia. Currently approved rapidly accelerated fibrosarcoma (RAF) kinase inhibitors targeting oncogenic BRAF V600 mutations have shown remarkable efficacy in the clinic, but their therapeutic benefits are occasionally hampered by acquired resistance due to RAF dimerization-dependent reactivation of the downstream MAPK pathway, which is known as paradoxical activation. There is also a concern that paradoxical activation of the MAPK pathway may trigger secondary cancer progression. Read More

JAAD Case Rep 2022 Jun 1;24:32-34. Epub 2022 Apr 1.

Department of Dermatology, Warren Alpert Medical School, Brown University, Providence, Rhode Island.

Pathol Oncol Res 2022 29;28:1610338. Epub 2022 Apr 29.

Department of Internal Medicine and Oncology, Semmelweis University, Budapest, Hungary.

Hairy cell leukaemia (HCL) is a rare B cell malignancy with an indolent course leading to pancytopaenia due to bone marrow infiltration. It has been proposed that HCL patients are at risk of developing a secondary malignancy, with a marked likelihood of the development of other hematologic malignancies including Hodgkin lymphoma and high-grade non-Hodgkin lymphomas. Here, we present the case of two patients who developed diffuse large B cell lymphoma after a long course of hairy cell leukaemia. Read More

Blood Adv 2022 May 2. Epub 2022 May 2.

Fundación Jimenez Díaz University Hospital, Madrid, Spain.

Br J Haematol 2022 May 2. Epub 2022 May 2.

Hematology, Department of Translational and Precision Medicine, Sapienza University, Rome, Italy.

J Cancer Res Clin Oncol 2022 Apr 27. Epub 2022 Apr 27.

Hematology Service, Department of Oncology and Clinical Pathology Service, Department of Diagnostics, University Hospital Geneva, 1211, Geneva, Switzerland.

Background: Hairy cell leukemia (HCL) is a rare chronic B cell malignancy, characterized by infiltration of bone marrow, blood and spleen by typical "hairy cells" that bear the BRAFV600E mutation. However, in addition to the intrinsic activation of the MAP kinase pathway as a consequence of the BRAFV600E mutation, the potential participation of other signaling pathways to the pathophysiology of the disease remains unclear as the precise origin of the malignant hairy B cells.

Materials And Methods: Using mRNA gene expression profiling based on the Nanostring technology and the analysis of 290 genes with crucial roles in B cell lymphomas, we defined a 17 gene expression signature specific for HCL. Read More

Hematol Rep 2022 Apr 13;14(2):135-142. Epub 2022 Apr 13.

Infectious Disease Unit, Azienda ULSS2 Marca Trevigiana, 31100 Treviso, Italy.

The present paper reports, to the best of our knowledge for the first time, the efficacy and tolerability of the combination of interferon (IFN)α-2a in pegylated formulation and rituximab after a "priming" phase with IFN in the frontline treatment of hairy cell leukemia (HCL) in a profoundly immunosuppressed patient with a infection at onset. This immunotherapy combination may represent a potential therapeutic option in patients with active severe infection and for whom the use of purine nucleoside analogues (PNA) is contraindicated. The benefits and drawbacks of remarkably rapid immune reconstitution in the context of opportunistic infections are highlighted as well, as the potentially paradoxical effects of immune recovery as a result of effective immunotherapy strategies, known as immune reconstitution inflammatory syndrome (IRIS), have to be taken into account when dealing with patients with opportunistic infections. Read More

Cancers (Basel) 2022 Apr 10;14(8). Epub 2022 Apr 10.

INSERM U1245, Normandie University, UNIROUEN, UNICAEN, 14032 Caen, France.

Hairy cell leukemia (cHCL) patients have, in most cases, a specific clinical and biological presentation with splenomegaly, anemia, leukopenia, neutropenia, monocytopenia and/or thrombocytopenia, identification of hairy cells that express CD103, CD123, CD25, CD11c and identification of the V600E mutation in the B-Raf proto-oncogene () in 90% of cases. Monocytopenia is absent in vHCL and SDRPL patients and the abnormal cells do not express CD25 or CD123 and do not present the mutation. Ten percent of cHCL patients are BRAF and the distinction between cHCL and HCL-like disorders including the variant form of HCL (vHCL) and splenic diffuse red pulp lymphoma (SDRPL) can be challenging. Read More

Ther Adv Hematol 2022 13;13:20406207221090886. Epub 2022 Apr 13.

Laboratoire Hématologie, CHU de Caen Normandie, avenue de Côte de Nacre, 14033 Caen Cedex, France.

Hairy cell leukemia (HCL) and HCL-like disorders such as hairy cell leukemia variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL) are rare indolent B-cell malignancies. Purine analogs (PNAs), alone or in association with rituximab (R), are the standard of care for HCL in the first-line setting. However, PNAs are toxic and patients may become resistant to these drugs. Read More

Ann Hematol 2022 Jun 18;101(6):1201-1210. Epub 2022 Apr 18.

State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, 300020, China.

Both characteristics and optimal treatment strategy for hairy cell leukemia-variant (HCL-v) remain elusive due to its rarity. We retrospectively analyzed the clinical features of HCL-v and the efficacy of first-line treatment options in a large Chinese cohort. In this study, we recruited 33 HCL-v patients (23 males and 10 females) with a median age of 59 years (range, 34-79 years). Read More

Curr Oncol Rep 2022 Apr 11. Epub 2022 Apr 11.

Hématologie, CHU de Caen Basse Normandie, Avenue Côte de Nacre, 14033, Caen Cedex, France.

Purpose Of Review: This article aims to bring an update on the recent discoveries in hairy cell leukemia (HCL), especially findings in pathophysiology and therapeutic advances.

Recent Findings: Major discoveries have been made in genetics and epigenetics of HCL. Moreover, the importance of several signaling pathways and tumor microenvironment has been recently highlighted. Read More

J Hematol 2022 Feb 10;11(1):21-28. Epub 2022 Jan 10.

Diagnostic Genomic Division, Hamad Medical Corporation (HMC), Doha, Qatar.

Herein, we describe the clinicopathologic and genetic characteristics of the first report of simultaneous bone marrow involvement by classical hairy cell leukemia (HCL) and leukemic non-nodal variant of mantle cell lymphoma (L-NN-MCL) with t(11;14)(q13;q32) with mutation and deletion of A 40-year-old asymptomatic man was investigated for incidental neutropenia and thrombocytopenia. Flow cytometry showed two distinct monotypic B-cell populations: one expressed CD19 (bright), CD20 (bright), FMC7, CD103, CD25, CD11c, CD123, and IgD (bright) and showed kappa light chain restriction (bright), consistent with HCL and the other kappa-restricted CD5/CD10-negative B-cell population with distinctive immunophenotypic features. The bone marrow biopsy is infiltrated by an abnormal B-lymphoid infiltrate with different patterns of infiltration in different marrow areas. Read More

Case Rep Oncol 2022 Jan-Apr;15(1):78-85. Epub 2022 Feb 7.

Department of Hematology and Oncology, Fox Chase Cancer Center, Philadelphia, Pennsylvania, USA.

Liquid biopsy is a valuable tool in advanced and metastatic cancers for detection of genomic alterations in tumors that facilitate personalized targeted therapy approaches. Analyzing circulating tumor DNA (ctDNA) using next-generation sequencing (NGS) provides an opportunity to detect tumor genomic changes during therapy and capture inter- and intra-heterogeneity of genomically divergent cancer cell evolution. Herein, we present a patient with metastatic castration-resistant prostate cancer, with progression to soft tissues, bone, and regional lymph nodes, who was treated with abiraterone plus prednisone, with excellent prostate-specific antigen response. Read More

Blood 2022 03;139(12):1924

University of California San Diego Health System.

Blood Cancer J 2022 03 17;12(3):43. Epub 2022 Mar 17.

Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Protein expression for 384 total and post-translationally modified proteins was assessed in 871 CLL and MSBL patients and was integrated with clinical data to identify strategies for improving diagnostics and therapy, making this the largest CLL proteomics study to date. Proteomics identified six recurrent signatures that were highly prognostic of survival and time to first or second treatment at three levels: individual proteins, when grouped into 40 functionally related groups (PFGs), and systemically in signatures (SGs). A novel SG characterized by hairy cell leukemia like proteomics but poor therapy response was discovered. Read More

Cancers (Basel) 2022 Feb 28;14(5). Epub 2022 Feb 28.

Department of Internal Medicine V, Hematology and Oncology, Medical University of Innsbruck, A-6020 Innsbruck, Austria.

Classic hairy cell leukemia (HCL) is an uncommon hematologic malignancy characterized by an excellent prognosis since purine analogues (PA), such as cladribine (2-CdA), have been introduced in the 1990s. However, most data on long-term outcomes is gathered from patients treated with PA first-line or include limited information on previous treatment outcomes, i.e. Read More

Chemistry 2022 May 1;28(27):e202104311. Epub 2022 Apr 1.

Molecular, Macromolecular Chemistry, and Materials, ESPCI Paris, PSL University, CNRS, 10 rue Vauquelin, 75005, Paris, France.

The synthesis of 2-C-glycals and 2-C-ribals was achieved in good yields using a nickel-catalyzed cross-coupling between 2-iodoglycals and 2-iodoribal respectively and Grignard reagents. The prepared 2-C-glycals and ribals were then transformed into 2-C-2-deoxyglycosides, 2-C-diglycosides and 2'-C-2'-deoxynucleosides. The developed method was applied to the synthesis of a 2-chloroadenine 2'-deoxyribonucleoside - a structural analogue of cladribine (Mavenclad®, Leustatin®) and clofarabine (Clolar®, Evoltra®), two compounds used in the treatment of relapsing-remitting multiple sclerosis and hairy cell leukemia. Read More

Int J Surg Pathol 2022 Mar 2:10668969221081432. Epub 2022 Mar 2.

Japan Baptist Hospital, Kyoto, Japan.

Hairy cell leukemia-Japanese variant (HCL-jv) shares some features with, but differs in other features from, HCL variant. Recently, it has been pointed out that HCL-jv and splenic diffuse red pulp small B-cell lymphoma (SDRPL) possibly constitute the same disease. We report a patient with HCL-jv, in which the neoplastic cells in the resected spleen were positive for CD11c, CD103, tartrate-resistant acid phosphatase (by immunohistochemistry), and weakly positive for cyclin D3. Read More

Ann Hematol 2022 May 26;101(5):1089-1096. Epub 2022 Feb 26.

Division of Hematology and Oncology, Scripps Clinic and Scripps MD Anderson, 10666 N. Torrey Pines Ave, La Jolla, CA, 92037, USA.

Hairy cell leukemia (HCL) is a rare hematologic disorder characterized by pancytopenia and splenomegaly for which a single course of cladribine is highly effective in inducing complete remissions. However, there is limited real-world data on outcomes and complications among geriatric patients with HCL treated with cladribine. We conducted a retrospective review of all patients 70 years or older within the Scripps Clinic HCL Database at the time of first treatment with cladribine. Read More

Cancers (Basel) 2022 Feb 18;14(4). Epub 2022 Feb 18.

INSERM1245, MICAH, Normandie University of Caen and Rouen, UNIROUEN, UNICAEN, Avenue de la Côte de Nacre, 14033 Caen, France.

Hairy cell leukemia (HCL) is characterized by abnormal villous lymphoid cells that express CD103, CD123, CD25 and CD11c. HCL-like disorders, including hairy cell leukemia variant (vHCL) and splenic diffuse red pulp lymphoma (SDRPL), have similar morphologic criteria and a distinct phenotypic and genetic profile. We investigated the immunophenotypic features of a large cohort of 82 patients: 68 classical HCL, 5 vHCL/SDRPL and 9 HCL-like NOS. Read More

Cancers (Basel) 2022 Feb 15;14(4). Epub 2022 Feb 15.

Department of Medicine V, Hematology, Oncology and Rheumatology, University of Heidelberg, 69120 Heidelberg, Germany.

Classic hairy cell leukemia (HCL) is a rare indolent B-cell lymphoproliferative disorder characterized by profound pancytopenia and frequent infectious complications due to progressive infiltration of the bone marrow and spleen. Lacking effective treatment options, affected patients were confronted with a dismal survival prognosis of less than 5 years when the disease was first described in 1958. Tremendous therapeutic advances were accomplished with the introduction of purine analogues such as cladribine in the 1990s, facilitating a near-normal life expectancy in most HCL patients. Read More

Curr Treat Options Oncol 2022 Jan 18;23(1):99-116. Epub 2022 Feb 18.

Department of Medicine, Leukemia Service, Memorial Sloan Kettering Cancer Center, New York, NY, USA.

Opinion Statement: Hairy cell leukemia variant (HCL-V) is a rare B cell lymphoproliferative disorder with a clinical-pathological distinction from the classic form of hairy cell leukemia (HCL-C). HCL-V is more aggressive in nature, has a higher tendency to be refractory to conventional purine analog pharmacotherapies, and leads to a poorer prognosis. Hence, these differing features bring paramount importance to the diagnosis and management of HCL-V. Read More

Cancers (Basel) 2022 Jan 29;14(3). Epub 2022 Jan 29.

Cancer Genomics Group, Southampton General Hospital, Tremona Road, Southampton SO16 6YD, UK.

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 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. Read More

Cancers (Basel) 2022 Jan 28;14(3). Epub 2022 Jan 28.

Clinical and Experimental Onco-Hematology Unit, Centro di Riferimento Oncologico di Aviano (CRO) IRCCS, 33081 Aviano, Italy.

mutations account for the most frequent mutations in human cancers, and are generally correlated with disease aggressiveness, poor prognosis, and poor response to therapies. KRAS is required for adult hematopoiesis and plays a key role in B cell development and mature B cell proliferation and survival, proved to be critical for B cell receptor-induced ERK pathway activation. In mature B cell neoplasms, commonly seen in adults, KRAS and RAS-MAPK pathway aberrations occur in a relevant fraction of patients, reaching high recurrence in some specific subtypes like multiple myeloma and hairy cell leukemia. Read More