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Indian J Hematol Blood Transfus 2020 Jul 2;36(3):571-572. Epub 2019 Dec 2.

Department of Internal Medicine, Saint Vincent Hospital, 123 Summer Street, Worcester, Massachusetts 01608 United States.

Am J Ophthalmol Case Rep 2020 Sep 6;19:100774. Epub 2020 Jun 6.

Department of Ophthalmology, Wake Forest School of Medicine, 1 Medical Center Blvd, Winston-Salem, NC, 27157, USA.

Purpose: To report a case of endogenous endophthalmitis.

Observations: An immunosuppressed patient with a history of hairy cell leukemia undergoing chemotherapy presents with rapidly progressing panophthalmitis. The patient was treated medically without improvement and underwent a pars plana vitrectomy which showed multiple retinal abscesses. Read More

Int J Med Sci 2020 30;17(10):1375-1384. Epub 2020 May 30.

Blood Diseases Institute, Xuzhou Medical University, Xuzhou, Jiangsu, China.

Cladribine is a purine nucleoside analog used to treat B-cell chronic lymphocytic leukemia and hairy cell leukemia, also functions as an inhibitor of DNA synthesis to block the repair of the damaged DNA. The therapeutic role of cladribine against diffuse large B-cell lymphoma cells (DLBCL) is still undefined. In the present study, we demonstrated that cladribine inhibited cell proliferation and induced G phase arrest in human DLBCL cells. Read More

Cold Spring Harb Perspect Med 2020 Jun 29. Epub 2020 Jun 29.

Human Oncology and Pathogenesis Program, Department of Medicine, Leukemia Service, Department of Medicine, Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York 10065, USA.

Mutations in members of the mitogen-activated protein kinase (MAPK) pathway are extensively studied in epithelial malignancies, with mutations being one of the most common alterations activating this pathway. However, mutations are overall quite rare in hematological malignancies. Studies over the past decade have identified high-frequency , , and other kinase alterations in two groups of MAPK-driven hematopoietic neoplasms: hairy cell leukemia (HCL) and the systemic histiocytoses. Read More

Biomolecules 2020 Jun 20;10(6). Epub 2020 Jun 20.

Laboratory of Molecular Biology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892-4264, USA.

Hepatocellular carcinoma (HCC) accounts for most liver cancers and represents one of the deadliest cancers in the world. Despite the global demand for liver cancer treatments, there remain few options available. The U. Read More

Clin Med Insights Blood Disord 2020 10;13:1179545X20926188. Epub 2020 Jun 10.

Floyd & Delores Jones Cancer Institute, Virginia Mason Medical Center, Seattle, WA, USA.

The spleen is among the most common extranodal sites for Hodgkin and non-Hodgkin lymphomas (NHLs); however, among lymphomas arising from the spleen, primary splenic lymphomas (PSLs) are rare. The group of PSLs includes primary splenic diffuse large B-cell lymphoma (PS-DLBCL), splenic red pulp small B-cell lymphoma, splenic marginal zone lymphoma (SMZL), and a splenic hairy cell leukemia variant. Delineating between the PSL variants can be challenging, especially as fine-needle aspirate and core needle biopsy of the spleen are not routinely offered at most medical centers. Read More

J Allergy Clin Immunol Pract 2020 Jun 15. Epub 2020 Jun 15.

Service de Dermatologie et d'Allergologie, Hôpital Tenon, Paris HUEP, APHP, Paris, Sorbonne Universités, Paris, France; Centre d'Immunologie et des Maladies Infectieuses - Paris (Cimi-Paris), INSERM U1135, Paris, France.

Turk J Haematol 2020 06 16. Epub 2020 Jun 16.

University of Siena, Department of Hematology, Siena, Italy.

J Pharm Sci 2020 Jun 11. Epub 2020 Jun 11.

Analytical Sciences, Biopharmaceutical Development, AstraZeneca, One MedImmune Way, Gaithersburg, MD 20878, USA. Electronic address:

Asparagine (Asn) deamidation is a common posttranslational modification in which Asn is converted to aspartic acid or isoaspartic acid. By introducing a negative charge, deamidation could potentially impact the binding interface and biological activities of protein therapeutics. We identified a deamidation variant in moxetumomab pasudotox, an immunotoxin Fv fusion protein drug derived from a 38-kDa truncated Pseudomonas exotoxin A (PE38) for the treatment of hairy-cell leukemia. Read More

Ann Hematol 2020 Jun 12. Epub 2020 Jun 12.

Department of Hematology, Medical University of Lodz and Copernicus Memorial Hospital, Ul. Ciolkowskiego 2, Lodz, Poland.

Cytometry A 2020 Jun 9. Epub 2020 Jun 9.

MLL Munich Leukemia Laboratory, Munich, Germany.

The wealth of information captured by multiparameter flow cytometry (MFC) can be analyzed by recent methods of computer vision when represented as a single image file. We therefore transformed MFC raw data into a multicolor 2D image by a self-organizing map and classified this representation using a convolutional neural network. By this means, we built an artificial intelligence that is not only able to distinguish diseased from healthy samples, but it can also differentiate seven subtypes of mature B-cell neoplasm. Read More

Blood Cancer J 2020 May 27;10(5):62. Epub 2020 May 27.

Laboratoire d'Hematologie Biologique, CHU de Caen, Caen, France.

In total, 279 patients with hairy-cell leukemia (HCL) were analyzed, with a median follow-up of 10 years. Data were collected up to June 2018. We analyzed responses to treatment, relapses, survival, and the occurrence of second malignancies during follow-up. Read More

Mol Biol Rep 2020 Jun 26;47(6):4365-4372. Epub 2020 May 26.

Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

BRAF V600E is a disease defining mutation for hairy cell leukemia (HCL), which helps in its diagnosis and differentiation from morphologically similar splenic marginal zone lymphoma (SMZL) and HCL-variant (HCL-v). Forty eight cases:HCL(n = 34), SMZL(n = 11) and HCL-v(n = 3) were included. Of these, 32 were retrospective and 16 were prospective. Read More

Indian J Hematol Blood Transfus 2020 Apr 9;36(2):438-440. Epub 2019 Sep 9.

2Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India.

Indian J Hematol Blood Transfus 2020 Apr 28;36(2):267-270. Epub 2019 Sep 28.

1Department of Medical Oncology, Cancer Institute (WIA), Chennai, Tamil Nadu 600036 India.

HCL is an uncommon B cell lympho-proliferative disorder with high remission rates. There is paucity of data on the long-term outcome of HCL from India. We retrospectively collected data from individual case records of patients with HCL who were treated in Cancer Institute, Chennai from January 2001 until January 2018. Read More

Clin Lymphoma Myeloma Leuk 2020 Apr 22. Epub 2020 Apr 22.

Department of Hematology, Ankara Ataturk Research and Training Hospital, Ankara, Turkey.

Background: Hairy cell leukemia is a rare B-cell lymphoproliferative disorder. It has an indolent course with relapse and remission periods. The aim of this study was to investigate the clinical characteristics and risk factors affecting the outcome of patients with hairy cell leukemia. Read More

Mult Scler Relat Disord 2020 May 3;43:102151. Epub 2020 May 3.

Multiple Sclerosis Center "A. Cardarelli "Hospital, Naples, Italy; Neurological Clinic and Stroke Unit "A. Cardarelli "Hospital, Naples, Italy.

Background: Cladribine tablets were recently approved for the treatment of Relapsing-Remitting Multiple Sclerosis (RRMS), reducing B cells and T cells, followed by reconstitution of the adaptive immune system, with transient and mild effects on the innate one. Cladribine is also the standard first-line and subsequent treatment for Hairy-Cell Leukemia (HCL), frequently complicated by neutropenic fever. Recombinant human Granulocyte Colony-Stimulating Factor (G-CSF; Filgrastim) has been proved to reduce neutropenia by increasing neutrophil count. Read More

Blood Res 2020 Jun;55(2):123-127

Departments of Pathology and Laboratory Medicine, IMS and Sum Hospital, S'O'A Deemed to be University, Bhubaneswar, India.

Clin Chem Lab Med 2020 May 8. Epub 2020 May 8.

Universitat Pompeu Fabra, Department of Experimental and Health Sciences, Pompeu Fabra University, Barcelona Biomedical Research Park, Dr. Aiguader 88, 08003 Barcelona, Spain.

Expert Opin Pharmacother 2020 May 7:39-46. Epub 2020 May 7.

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

Introduction: Hairy cell leukemia (HCL) is a B-cell lymphoid malignancy that accounts for approximately 2% of all leukemias. Treatment with purine nucleoside analogs (PNA) results in a high response rate and remains the standard of care. Long term follow-up shows that most patients relapse and require retreatment. Read More

J Cancer Res Ther 2020 Jan-Mar;16(1):120-126

Department of Hematology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttra Pradesh, India.

Objective: The presence of specific chemotherapeutic protocols for hairy cell leukemia (HCL) makes it essential to discriminate this entity from other lymphoproliferative disorders. Hence, awareness of the variations in clinical presentations and immunophenotypic aberrancies is requisite to ensure diagnostic accuracy.

Materials And Methods: A retrospective study was carried out to analyze the clinical-pathological profile of patients with HCL diagnosed over a period of 81 months (2010-September 2017) in our institute. Read More

Curr Treat Options Oncol 2020 Apr 30;21(6):44. Epub 2020 Apr 30.

Laboratory of Hematology, Academic Hospital of Caen, Caen, Normandy, France.

Opinion Statement: Despite its rarity, hairy cell leukemia (HCL) remains a fascinating disease and the physiopathology is becoming more and more understood. The accurate diagnosis of HCL relies on the recognition of hairy cells by morphology and flow cytometry (FCM) in the blood and/or bone marrow (BM). The BRAF V600E mutation, an HCL-defining mutation, represents a novel diagnostic parameter and a potential therapeutic target. Read More

Leuk Lymphoma 2020 Apr 22:1-3. Epub 2020 Apr 22.

Department of Pathology, Peter MacCallum Cancer Centre, Melbourne, Australia.

Curr Oncol Rep 2020 Apr 16;22(5):42. Epub 2020 Apr 16.

The Royal Marsden Hospital and the Institute of Cancer Research, Sutton, UK.

Purpose Of Review: To summarise diagnostic clinical/laboratory findings and highlight differences between classical hairy cell leukaemia (HCLc) and hairy cell leukaemia variant (HCLv). Discussion of prognosis and current treatment indications including novel therapies, linked to understanding of the underlying molecular pathogenesis.

Recent Findings: Improved understanding of the underlying pathogenesis of HCLc, particularly the causative mutation BRAF V600E, leading to constitutive activation of the MEK/ERK signalling pathway and increased cell proliferation. Read More

Dtsch Arztebl Int 2020 Mar;117(10):176

Leuk Res Rep 2020 17;13:100197. Epub 2020 Mar 17.

Division of Hematology and Medical Oncology, Mayo Clinic, 4500 San Pablo Rd S, Jacksonville, FL, United States.

A 75-year-old male evaluated for pancytopenia. Abnormal lymphocytes with hairy projections noted on peripheral blood. Bone marrow examination showed diffuse proliferation of CD20+ B-lymphocytes. Read More

Dermatol Online J 2020 Feb 15;26(2). Epub 2020 Feb 15.

Department of Dermatology, University of New Mexico, School of Medicine, Albuquerque, NM.

Grover disease (GD) is an acquired, nonfamilial, nonimmune mediated, transient or persistent acantholytic dermatosis. Herein, we present a 72-year-old man who had clinical and histopathologic findings of GD following two weeks of treatment with vemurafenib without MEK inhibitor. The patient was successfully treated with topical emollients and a high-potency corticosteroid. Read More

Mil Med 2020 Apr 1. Epub 2020 Apr 1.

Department of Hematology and Oncology, Washington DC Veterans Medical Center, 50 Irving St NW, Washington, DC 20422.

The industrial solvents benzene and trichloroethylene (TCE) are known carcinogens, and these solvents contaminated the drinking water at Marine Corps Base Camp Lejeune from the 1950s to 1980s. Benzene and TCE are linked to the hematopoietic cancers acute myelocytic and lymphocytic leukemia and chronic lymphocytic leukemia. We report the case of a veteran stationed at Marine Corps Base Camp Lejeune during this period who developed hairy cell leukemia (HCL), a rare form of lymphocytic leukemia. Read More

Biomolecules 2020 Mar 27;10(4). Epub 2020 Mar 27.

Laboratory of Medical Genetics, Faculty of Biology and Environmental Protection, University of Lodz, 90-236 Lodz, Poland.

Many biologically-active plant-derived compounds have therapeutic or chemopreventive effects. The use of plant in vitro cultures in conjunction with modern genetic engineering techniques allows greater amounts of valuable secondary metabolites to be obtained without interfering with the natural environment. This work presents the first findings concerning the acquisition of transgenic hairy roots of overexpressing the gene encoding squalene synthase 1 from () (SOPSS hairy loot lines) involved in terpenoid biosynthesis. Read More

Genomics Inform 2020 Mar 31;18(1):e6. Epub 2020 Mar 31.

Alfred I. duPont Hospital for Children, Wilmington, DE 19803 , USA.

Acute leukemia represents the most common pediatric malignancy comprising diverse subtypes with varying prognosis and treatment outcomes. New and targeted treatment options are warranted for this disease. Patient-derived xenograft (PDX) models are increasingly being used for preclinical testing of novel treatment modalities. Read More

Cureus 2020 Feb 14;12(2):e6995. Epub 2020 Feb 14.

Department of Medical Education, College of Medicine, King Faisal University, Al-Ahsa, SAU.

Background Multiple sclerosis (MS) is an autoimmune and demyelinating inflammatory disease that affects the central nervous system (CNS). The etiology of the disease remains unknown. Multiple theories highlight genetic, environmental, and infectious factors that may a role. Read More

Cureus 2020 Jan 31;12(1):e6825. Epub 2020 Jan 31.

Pathology, Albany Medical Center, Albany, USA.

Histoplasmosis is an endemic fungal infection that can lead to disseminated disease, especially in immunosuppressed patients. Hairy cell leukemia is a rare, slow-growing hematological malignancy. Concurrence of histoplasmosis and hairy cell leukemia is extremely rare. Read More

Int J Med Sci 2020 18;17(3):325-331. Epub 2020 Jan 18.

Department of Hematology, The First Affiliated Hospital of China Medical University, Shenyang, Liaoning, 110001, P.R. China.

Hairy cell leukemia (HCL) is a rare chronic B-cell lymphoproliferative disorder. It has two pathological subtypes: classical HCL (HCL-C) and HCL-variant (HCL-V). HCL-C and HCL-V are distinct in morphology and immunophenotype. Read More

Case Rep Oncol 2019 Sep-Dec;12(3):922-927. Epub 2019 Dec 17.

Hematology Department, National Center for Cancer Research (NCCCR), Hamad Medical Corporation (HMC), Doha, Qatar.

Hairy cell leukemia (HCL) is rare type of leukemia. This neoplasm is well-known to present with pancytopenia and splenomegaly. HCL is associated with BRAF mutation in 100% of cases. Read More

J Clin Oncol 2020 May 28;38(14):1527-1538. Epub 2020 Feb 28.

Medical Oncology Service, National Cancer Institute, National Institutes of Health, Bethesda, MD.

Purpose: Single-agent purine analog, usually cladribine, has been the standard first-line therapy of hairy cell leukemia (HCL) for 30 years. High complete remission (CR) rates often include minimal residual disease (MRD), leading to relapse and repeated treatments. Rituximab can clear MRD, but long-term results are unknown and optimal timing of rituximab undefined. Read More

Br J Clin Pharmacol 2020 Jul 16;86(7):1367-1376. Epub 2020 Mar 16.

AstraZeneca, Gaithersburg, MD, USA.

Aims: To characterize the pharmacokinetics (PK) of moxetumomab pasudotox, an anti-CD22 recombinant immunotoxin, in adults with relapsed or refractory hairy cell leukaemia, we examined data from a phase 1 study (Study 1001; n = 49) and from the pivotal clinical study (Study 1053; n = 74).

Methods: Data from both studies were pooled (n = 123) to develop a population PK model. Covariates included demographics, disease state, liver and kidney function, prior treatment, and antidrug antibodies (ADAs). Read More

Orphanet J Rare Dis 2020 Feb 13;15(1):47. Epub 2020 Feb 13.

Division of Hematology and Oncology, The University of New Mexico, Albuquerque, NM, USA.

Background: Purine nucleoside analogs (PNAs) are the recommended first-line treatment for patients with hairy cell leukemia (HCL), but they are associated with adverse events (AEs). Due to a lack of real-world evidence regarding AEs that are associated with PNAs, we used commercial data to assess AE rates, AE-related health care resource utilization (HCRU), and costs among PNA-treated patients with HCL. Adults aged ≥18 years with ≥2 claims for HCL ≥30 days apart from 1 January 2006 through 31 December 2015 were included. Read More

Pathol Int 2020 May 12;70(5):280-286. Epub 2020 Feb 12.

Department of Pathology, Kurume University, School of Medicine, Fukuoka, Japan.

Primary splenic low-grade B-cell lymphoma of the red pulp comprises hairy cell leukemia (HCL) and splenic B-cell lymphoma/leukemia, unclassifiable (SPLL-U). SPLL-U is a rare disease that includes subtypes of a hairy cell leukemia-variant (HCL-v), splenic diffuse red pulp small B-cell lymphoma (SDRPL) and other types that are known as narrow sense SPLL-U (SPLL-U-NS). Notably, limited information is available regarding the BRAF mutation (V600E) and cyclin D3 expression in subtypes of SPLL-U. Read More

PLoS One 2020 10;15(2):e0227586. Epub 2020 Feb 10.

Laboratory of Molecular Biology, National Cancer Institute, National Institutes of Health, Bethesda, MD , United States of America.

Hairy cell leukemia (HCL) is a purine analog-responsive B-cell malignancy containing the BRAF V600E mutation, expressing CD22, CD11c, CD103, tartrate resistant acid phosphatase (TRAP) CD25, CD123, and annexin 1A. BRAF V600E and the latter 4 markers are usually absent in the more aggressive and chemoresistant variant HCLv. To evaluate differences between HCL and HCLv, expression microarrays comparing HCL with HCLv were performed for 24694 genes using 47323 probes. Read More

Leuk Res 2020 Mar 31;90:106315. Epub 2020 Jan 31.

Section of Hematology, Department of Medicine, Yale University School of Medicine, New Haven, USA. Electronic address:

Blood 2020 01;135(5):392

University of Perugia.

Leukemia 2020 Jul 28;34(7):1934-1938. Epub 2020 Jan 28.

Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.

Int J Mol Sci 2019 Nov 30;20(23). Epub 2019 Nov 30.

Institute of Hematology and Transfusion Medicine, Indiry Gandhi 14, 02-776 Warsaw, Poland.

Acid sphingomyelinase (aSMase) is involved in the generation of metabolites that function as part of the sphingolipid signaling pathway. It catalyzes the breakdown of sphingomyelin into ceramide, a bioactive lipid that, among other roles, is involved in regulation of apoptosis. Dry drop blood test (DBS) and colorimetric 2-step enzymatic assay were used to assess the activity of human blood aSMase, beta-galactosidase, and beta-glucosidase, these enzymes are lysosomal hydrolases that catalyze the degradation of related sphingolipids, of sphingolipid signaling molecules. Read More

Blood Cells Mol Dis 2020 03 29;81:102392. Epub 2019 Nov 29.

Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Centre, Service d'Hématologie Clinique, Paris, France; Université Paris Descartes, Faculté de Médecine Sorbonne Paris Cité, Paris, France.

Mastocytosis is a mast cell disease caused by functionally defective infiltrating mast cells and CD34+ mast cell precursors. The heterogeneous group of mast cell disorders is categorized into five variants in the updated 2017 World Health Organization (WHO) classification among those systemic mastocytosis with an associated neoplasm (SM-AHN). Except for myeloid neoplasia, lymphoproliferative disorders associated to SM-AHN are more scarce. Read More

Indian J Hematol Blood Transfus 2019 Oct 13;35(4):692-698. Epub 2019 May 13.

3İnternal Medicine, Department of Hematology, İzmir Katip Celebi University, Izmir, Turkey.

We aimed to analyze the characteristics and response rates of different treatment modalities in hairy cell leukemia patients over 20 diagnosed as hairy cell leukemia (HCL). Clinical data, response rates and survival outcome of the patients who were diagnosed with HCL were retrospectively analyzed. Fifty-two patients with a median age of 50 (28-87) years were enrolled in the study. Read More

Leukemia 2020 May 18;34(5):1454-1457. Epub 2019 Nov 18.

Department of Medicine V, Heidelberg University Hospital, Heidelberg, Germany.

PLoS One 2019 12;14(11):e0225096. Epub 2019 Nov 12.

Department of Pathology, Hallym University Dongtan Sacred Heart Hospital, Hwaseong-si, Gyeonggi-do, Republic of Korea.

SOX11 is a transcription factor that is normally expressed in the fetal brain and has also been detected in some malignant tumors, including mantle cell lymphoma (MCL). MCL is a mature B-cell lymphoma that characteristically expresses cyclin D1, which has been used as a diagnostic tumor marker. SOX11 has also recently emerged as a tumor marker for MCL, particularly in cyclin D1-negative MCLs and to distinguish between MCLs and other cyclin D1-positive lymphomas. Read More

Pol Przegl Chir 2019 Jan;91(5):38-40

Katedra i Klinika Chirurgii Ogólnej i Transplantacyjnej, Uniwersytecki Szpital Kliniczny im. N. Barlickiego w Łodzi, Polska.

Background: Steroid-induced diabetes (SIDM) is a frequently found clinical condition since steroid-based therapies continue to be widely used in hospital and ambulatory care. Recommended optimal treatment of SIDM includes similar glucose lowering strategies as in type 2 diabetes. This typical management cannot cure the disease, it can only control blood glucose. Read More

J Immunol Methods 2020 Feb 30;477:112688. Epub 2019 Oct 30.

BioPharmaceuticals R&D, AstraZeneca, South San Francisco, CA 94080, USA. Electronic address:

Biologics are potentially immunogenic and can elicit immune response. Complex biologics, such as bispecific antibodies or multi-domain molecules can induce anti-drug antibodies (ADA) with specificity to different domains. Domain specific ADAs may differently affect drug efficacy and safety, and thus, characterization of ADA domain specificity has become a regulatory expectation for multi-domain biologics. Read More