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J Clin Pathol 2019 Mar 14. Epub 2019 Mar 14.

Division of Cellular and Molecular Pathology, Addenbrooke's Hospital, Cambridge, UK.

Aims: V600E detection assists in the diagnosis of hairy cell leukaemia (HCL); however, testing practices vary. We evaluated the clinical utility of 5 mutation testing strategies for use on bone marrow trephines (BMT).

Methods: 11 HCL, 5 HCL 'mimic', 2 treated HCL and 10 normal BMT specimens were tested for mutant comparing Sanger sequencing, pyrosequencing, amplicon-based next generation sequencing (NGS), automated (Idylla) PCR and immunohistochemistry (IHC). Read More

Blood 2019 Mar;133(11):1264

National Institutes of Health Clinical Center.

Pathol Int 2019 Mar 7. Epub 2019 Mar 7.

CHRU Brest, Department of Pathology, Brest, F-29220, France.

BRAF mutation detection is worthwhile for the management of patients with some advanced cancers. The tumor samples are sometimes difficult to analyze using DNA-based molecular methods because of poor tumor DNA quality or quantity. Anti-BRAFV600E VE1 immunohistochemistry (IHC) has been proposed as a valuable ancillary tool to analyze some "molecularly challenging" tumor samples. Read More

Lancet Oncol 2019 Mar;20(3):e187

Institute of Hematology and Center for Hemato-Oncology Research (C.R.E.O.), University and Hospital of Perugia, Perugia, Italy.

Ann Pharmacother 2019 Mar 6:1060028019836775. Epub 2019 Mar 6.

1 Memorial Sloan Kettering Cancer Center, New York, NY, USA.

Objective: To review the literature for the treatment of classical and variant hairy cell leukemia (HCL, HCLv), evaluating efficacy, safety, and supportive care involved in the use of purine analogues (PAs), interferon, BRAF inhibitors, monoclonal antibodies, Bruton's tyrosine kinase inhibitors, and new immunotoxin, moxetumomab pasudotox-tdfk (MPT). An electronic literature search of PubMed (January 1958 to January 2019) was conducted in PubMed using the MESH terms hairy cell leukemia, hairy cell leukemia variant, cladribine, pentostatin, rituximab, interferon, vemurafenib, moxetumomab pasudotox.

Study Selection And Data Extraction: Studies written in the English language were considered for this article. Read More

Hematol Transfus Cell Ther 2019 Jan-Mar;41(1):88-90. Epub 2018 Jun 11.

Instituto do Câncer do Estado de São Paulo (ICESP), Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, SP, Brazil.

Expert Rev Hematol 2019 Feb 20:1-11. Epub 2019 Feb 20.

a Leukemia Service , Memorial Sloan Kettering Cancer Center , New York , NY , USA.

Introduction: Hairy cell leukemia (HCL) is a rare, chronic B-cell lymphoproliferative disorder characterized by distinctive morphologic features and an indolent clinical course. The discovery of a recurrent activating mutation in BRAF (BRAF V600E) as a disease-defining genetic event in HCL has substantial diagnostic and therapeutic implications. Areas covered: Herein the authors review the role of BRAF V600E and RAF-MEK-ERK signaling in the pathogenesis of HCL, anecdotal clinical reports of BRAF inhibitor monotherapy in management of relapsed or refractory HCL, larger phase 2 trials investigating efficacy of BRAF inhibitor therapy for HCL, adverse effects commonly associated with BRAF inhibitor therapy, including cutaneous toxicity, and mechanisms of therapeutic resistance. Read More

Blood Adv 2019 Feb;3(3):384-396

Cancer Sciences Unit, Cancer Research UK and NIHR Experimental Cancer Medicine Centres, University of Southampton, Southampton, United Kingdom.

Classic hairy cell leukemia (HCL) is a tumor of mature clonal B cells with unique genetic, morphologic, and phenotypic features. DNA methylation profiling has provided a new tier of investigation to gain insight into the origin and behavior of B-cell malignancies; however, the methylation profile of HCL has not been specifically investigated. DNA methylation profiling was analyzed with the Infinium HumanMethylation27 array in 41 mature B-cell tumors, including 11 HCL, 7 splenic marginal zone lymphomas (SMZLs), and chronic lymphocytic leukemia with an unmutated (n = 7) or mutated (n = 6) immunoglobulin gene heavy chain variable (IGHV) region or using IGHV3-21 (n = 10). Read More

Br J Haematol 2019 Jan 31. Epub 2019 Jan 31.

Division of Molecular Genetic Epidemiology, German Cancer Research Centre (DKFZ), Heidelberg, Germany.

Improvement of survival in lymphocytic leukaemia has been accompanied by the occurrence of second primary cancer (SPCs). Based on Swedish Family Cancer Database, we applied bi-directional analyses in which relative risks (RRs) were calculated for any SPCs in patients with chronic lymphocytic leukaemia (CLL), acute lymphoblastic leukaemia (ALL) and hairy cell leukaemia (HCL) and the risks of these leukaemias as SPCs. After CLL, RRs were significant for 20 SPCs, and high for skin squamous cell cancer (24·58 for in situ and 7·63 for invasive), Merkel cell carcinoma (14·36), Hodgkin lymphoma (7·16) and Kaposi sarcoma (6·76). Read More

BMC Infect Dis 2019 Jan 25;19(1):85. Epub 2019 Jan 25.

Department of Internal Medicine, St Antonius Hospital, Nieuwegein/ Utrecht, Koekoekslaan 1, 3435, CM, Nieuwegein, The Netherlands.

Background: Ecthyma gangrenosum is a cutaneous infectious usually associated with P. aeruginosa. It usually develops In patients with an underlying immunodeficiency. Read More

Cureus 2018 Nov 16;10(11):e3600. Epub 2018 Nov 16.

Hematology & Oncology, Allegheny Health Network, Pittsburgh, USA.

As the number of cancer survivors grows, so does the number of co-occurring primary malignancies and secondary malignancies. In rare cases, single driver mutations can be responsible for concomitant primary malignancies. By understanding the mechanisms that drive multiple primary malignancies (MPM), clinicians are capable of targeting molecular pathways that drive oncogenesis resulting in the successful treatment of many malignancies while also reducing the side effects of conventional chemotherapy. Read More

Case Rep Infect Dis 2018 24;2018:4294013. Epub 2018 Dec 24.

Division of Infectious Diseases, Warren Alpert Medical School of Brown University, Providence, USA.

We describe a case of disseminated mucormycosis () diagnosed on autopsy, in a man who had been working in construction with undiagnosed neutropenia from hairy-cell leukemia, which is rarely associated with invasive mold infections. Galactomannan values in both blood and bronchoalveolar lavage were strongly positive. There is an unmet need for accurate noninvasive fungal diagnostic tests. Read More

Blood Adv 2019 Jan;3(2):116-118

Division of Hematology, Department of Internal Medicine, The Ohio State University, Columbus, OH.

Cytometry B Clin Cytom 2018 Dec 28. Epub 2018 Dec 28.

Flow Cytometry Unit, Hematology Laboratory, ICO-Hospital Germans Trias i Pujol, Institut de Recerca Josep Carreras, Universitat Autònoma de Barcelona, Badalona, Spain.

Background: The concept of borderline lymphoproliferative disorder (LPD) has not been clearly defined.

Methods: This study aimed to classify patients with leukemic LPD (n = 597, excluding hairy cell leukemia, mantle cell lymphomas, and CD10-positive LPDs) into CLL or non-CLL applying three diagnostic strategies (the D'Arena and CLLflow scores and CD43 expression) and to better characterize unclassified patients.

Results: Patients with concurring CLL-like (n = 441) or non-CLL like (n = 99) results with the three diagnostic strategies were determined to have CLL and non-CLL, respectively. Read More

Asian Pac J Cancer Prev 2018 Dec 25;19(12):3383-3392. Epub 2018 Dec 25.

Clinical Pathology Department, Faulty of Medicine, Cairo University, Egypt. Email:

Background: Multiparameter flow cytometry is a useful tool for diagnostic evaluation of mature B-cell neoplasms (MBN). Recently, it has been shown that assessment of CD200 expression may improve the distinction between chronic lymphocytic leukemia (CLL; CD200 positive) and mantle cell lymphoma (MCL; CD200 negative), but any potential as a prognostic marker for CLL remains to be established. Materials and methods: This cross sectional study was conducted on sixty-seven patients newly diagnosed as having mature B-cell lymphoproliferative disorders Levels of CD 200 in lymphoma cells were assessed. Read More

J Cell Biochem 2018 Dec 11. Epub 2018 Dec 11.

Institute of Biochemistry and Biophysics, University of Tehran, Tehran, Iran.

Tyrosine kinase inhibitor (TKI)-based therapy has created promising results among much chronic myeloid leukemia (CML) patients. Imatinib as a relatively specific inhibitor of Bcr-Abl is at present one of the undisputed therapeutic agent for newlydiagnosed patients with CML. However, the occurrence of imatinib-resistance enlightens the urgent need to identify other therapeutic agents against CML. Read More

Case Rep Oncol Med 2018 14;2018:1736854. Epub 2018 Nov 14.

Specialist Care Australia, Launceston, Tasmania, Australia.

Hairy cell leukemia (HCL) and Merkel cell carcinoma (MCC) are two rare malignancies with distinct cells of origin. HCL is a lymphoid malignancy of mature B cells, and MCC derives from neuroendocrine cell origin. HCL has a favorable prognosis with most patients achieving long-term remission and potential cure. Read More

Ann Hematol 2018 Dec 4. Epub 2018 Dec 4.

Department of Haematology, St. Vincent's University Hospital, Dublin, Ireland.

Int J Dermatol 2018 Nov 27. Epub 2018 Nov 27.

Division of Dermatology, Department of Internal Medicine, Ohio State University Wexner Medical Center, Columbus, OH, USA.

Leuk Res 2018 Dec 5;75:58-60. Epub 2018 Nov 5.

University of Missouri, 1 Hospital Dr, McHaney Hall, Columbia, MO 65212, USA. Electronic address:

Hum Pathol 2018 Nov 17. Epub 2018 Nov 17.

General Pathology & Cytopathology Unit, Department of Medicine-DIMED, University of Padova, Padova, Italy.

Cortactin is a cytoskeletal-remodeling adaptor protein, playing an oncogenic role in solid tumors. Little is known on Cortactin expression in non-Hodgkin B-cell lymphomas (B-NHL). The present study aimed to characterize Cortactin expression in B-NHL and to assess its role in the differential diagnosis of such entities. Read More

Acta Clin Croat 2018 Jun;57(2):362-365

Department of Hematology, Sestre milosrdnice University Hospital Centre, Zagreb, Croatia.

T-cell large granular lymphocytic leukemia (T-LGLL) is an uncommon but probably underdiagnosed disease caused by clonal proliferation of large granular lymphocytes. Diagnosis is typically based on the high number of morphologically characteristic lymphoid cells and finding of an abnormal immunophenotype by flow cytometry. Because of its relatively indolent clinical behavior, observation is often an appropriate therapy. Read More

Anticancer Res 2018 Nov;38(11):6423-6429

The Ruth and Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel

Background/aim: In this retrospective study, we summarized the national Israeli experience with hairy cell leukemia (HCL) in a large cohort of patients with a long follow-up.

Patients And Methods: Demographic data, and relevant laboratory and clinical parameters were analyzed, emphasizing the outcome after first-line treatment with cladribine.

Results: Data on 203 patients was collected from 12 medical centers during 1985-2015. Read More

Drugs 2018 Nov;78(16):1763-1767

Springer, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.

Moxetumomab pasudotox-tdfk (LUMOXITI™), an anti CD22 recombinant immunotoxin, has been developed by MedImmune and its parent company AstraZeneca for the treatment of hairy cell leukaemia. The product, discovered at the National Cancer Institute, is an optimised version of immunotoxin CAT-3888. Moxetumomab pasudotox is composed of the Fv fragment of an anti-CD22 monoclonal antibody fused to a 38 kDa fragment of Pseudomonas exotoxin A, PE38. Read More

Leukemia 2019 Feb 19;33(2):541-545. Epub 2018 Oct 19.

Department of Haematology, University of Cambridge, Cambridge, UK.

JAMA 2018 Oct;320(15):1528

Leuk Lymphoma 2018 Oct 15:1-3. Epub 2018 Oct 15.

a Division of Hematology-Oncology , Scripps Clinic , La Jolla , CA , USA.

Indian J Pathol Microbiol 2018 Oct-Dec;61(4):532-536

Molecular Division, Tata Memorial Centre, Hematopathology Laboratory, Mumbai, Maharashtra, India.

Background: BRAFV600E mutation has been reported as a unique genetic lesion of hairy cell leukemia (HCL), a subset of which lacks this lesion and shows adverse outcomes.

Aims: To determine the prevalence of BRAFV600E in HCL from our center and derive clinicopathological correlation, if any.

Materials And Methods: A 9-year retrospective analysis of 46 consecutive cases of HCL diagnosed on morphology and immunophenotyping was done. Read More

Cancer Discov 2018 Nov 27;8(11):OF1. Epub 2018 Sep 27.

The FDA approved moxetumomab pasudotox-tdfk, a CD22-directed recombinant immunotoxin, for patients with relapsed/refractory hairy cell leukemia who have not responded to at least two prior treatments, including a purine nucleoside analogue. Read More

Acta Haematol 2018 18;140(2):114-120. Epub 2018 Sep 18.

Centro de Haematología y Medicina Interna de Puebla, Clínica Ruiz, Puebla, Mexico.

In 1963 Jean Bernard introduced the concept of "geographic hematology" and distinguished 2 branches, i.e., "ethnic hematology," which deals with differences between populations, and "environmental hematology," which considers factors such as food habits, infections, and others. Read More

Blood 2018 Sep;132(11):1216

University Medical Center Utrecht.

N Engl J Med 2018 Sep;379(11):1072-1081

From the Departments of Medicine (C.A.I., R.W.T.), Radiology (F.J.S.), and Pathology (B.J.S.), Massachusetts General Hospital, and the Departments of Medicine (C.A.I., R.W.T.), Radiology (F.J.S.), and Pathology (B.J.S.), Harvard Medical School - both in Boston.

J Am Acad Dermatol 2018 Sep 8. Epub 2018 Sep 8.

Division of Dermatology, Department of Internal Medicine, Ohio State University Wexner Medical Center, Columbus, Ohio.

J Cancer Res Ther 2018 Jul-Sep;14(5):1130-1134

Department of Hematology, SGPGI, Lucknow, Uttar Pradesh, India.

Hairy cell leukemia (HCL) is a rare, low-grade mature B-cell neoplasm with a characteristic clinical, morphological, immunophenotypic, and more recently described molecular (BRAF p.V600E mutation) profile. It typically affects middle-aged to elderly male who present with pancytopenia and massive splenomegaly. Read More

Br J Dermatol 2019 Jan;180(1):e3

Department of Dermatology, University of Rochester Medical Center, Rochester, NY, U.S.A.

Vnitr Lek 2018 ;64(5):501-507

Aplastic anemia - bone marrow failure (AA) is defined as pancytopenia with hypocellular bone marrow without signs of marrow fibrosis or of presence of abnormal cells. Recent studies showed that most of AA cases might be mediated by immune mechanisms. Toxic agent leads to expression of neoantigens or cryptic antigens on the surface of pluripotent hematopoietic stem cells with subsequent activation of immune effector cells and induction of stem cell apoptosis. Read More

J Natl Compr Canc Netw 2018 09;16(9):1035-1040

Division of Medical Oncology, Department of Internal Medicine

Mutations in the RAS/RAF/MEK/ERK pathway leading to constitutive activation and uncontrolled cellular growth have been identified in various human malignancies, making this pathway a target for potential therapeutics. The activating mutation is one well-characterized oncogenic mutation that has been described and targeted with clinical success in various malignancies, including melanoma and hairy cell leukemia. Although -directed treatments have yielded clinical benefit in a subset of tumor types, such as melanoma, thyroid cancer, and lung cancer, BRAF inhibition fails to confer a clinical benefit in colon cancer. Read More

Am J Hematol 2018 Dec 2;93(12):1568-1569. Epub 2018 Oct 2.

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

Clin Case Rep 2018 Aug 2;6(8):1651-1652. Epub 2018 Jul 2.

Service d'Hématologie Clinique Centre Hospitalier Universitaire Hôpital Robert Debré Reims France.

Hairy cell leukemia (HCL) is a rare B-cell lymphoproliferative disorder. Skeletal involvement is an unusual manifestation of HCL, complicating the course of the disease in approximately 3% of patients. We describe a case of skull involvement by HCL, a localization rarely reported in the literature. Read More

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Aug;26(4):1220-1224

Department of Hematology, Tianjin Medical University Cancer Hospital; National Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy; Tianjin 300060, China.E-mail:

The 2016 world health organization (WHO) classification of B cell chronic lymphoproliferative disease (B-CLPD) includes chronic lymphocytic leukemia (CLL), B prolymphocytic leukemia, (B-PLL), hairy cell leukemia (HCL), marginal zone lymphoma (MZL), follicular lymphoma (FL), mantle cell lymphoma (MCL), Waldenstrom macroglobulinemia (LPL/WM). All the above-mentioned diseases are partially similar in cell morphology, immunophenotype and molecular genetics, but significantly different in treatment and prognosis. Currently, many new drugs targeted at cell cycle and apoptosis pathway, such as proteasome inhibitor immune modulators and histone deacetylase inhibitors, have achieved encouraging results in B-CLPD, which bring new hope for patients with B-CLPD. Read More

Mult Scler J Exp Transl Clin 2018 Apr-Jun;4(2):2055217318783767. Epub 2018 Jun 26.

BartsMS, Blizard Institute, Queen Mary University of London, United Kingdom.

Background: A considerable number of people with multiple sclerosis (pwMS) live in low- and middle-income countries (LMIC), where lack of resource adversely affects access to effective disease-modifying treatment.

Objective: The objective of this commentary is to propose a useful cost-effective disease-modifying treatment option for pwMS in LMIC with potential high efficacy and high convenience to the pwMS and treating physician.: We propose using generic 2-chloro-2'-deoxyadenosine (cladribine), a small molecule licensed for treatment of people with hairy cell leukaemia, as a solution of this significant equity imbalance. Read More

Pathol Res Pract 2018 Dec 27;214(12):1952-1958. Epub 2018 Jul 27.

Department of Pathology, Xinjiang Medical University Cancer Hospital, New City District Suzhou Street No. 789, Urumqi, 830000, China.

Background: To study the effects of splenectomy on treatment and diagnosis of tumours of lymphoid tissue of the spleen.

Methods: Fifty-three cases were reviewed from Peking University People's Hospital from 2002 to 2017. According to WHO classification of tumours of haematopoietic and lymphoid tissues (2008) and classification updated (2016), the cases were studied by microscopy, immunohistochemistry and in situ hybridization, combined with the bone marrow biopsy and clinical examination. Read More

Br J Haematol 2018 Dec 24;183(5):702. Epub 2018 Jul 24.

Institute of Haematology and Centre of Haemato-Oncology Research (CREO), University and Hospital of Perugia, Perugia, Italy.

Int J Hematol 2018 Oct 24;108(4):416-422. Epub 2018 Jul 24.

Division of Hematology, Respiratory Medicine and Oncology, Department of Internal Medicine, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga, 849-8501, Japan.

Hairy cell leukemia (HCL) is a rare B-cell lymphoid malignancy that is difficult to distinguish from other morphological variants. The frequency of HCL has not been determined accurately in Japan. Recent studies revealed that the BRAF V600E mutation is the causal genetic event in HCL. Read More

Zhonghua Xue Ye Xue Za Zhi 2018 Jun;39(6):491-495

Institute of Hematology & Blood Disease Hospital, CAMS & PUMC, State Key Laboratory of Experimental Hematology, Tianjin 300020, China.

To investigate the curative effect of hairy cell leukemia by clatabine. The clinical data of 24 patients with hairy cell leukemia treated by cladribine from November 2006 to October 2017 were analyzed retrospectively, then the curative effect and adverse drug reaction were analyzed. ① A total of 24 patients including 22 male and 2 female, and the median age was 49. Read More

Clin Chem Lab Med 2018 Dec;57(2):e34-e36

Department of Internal Medicine 1, Hospital of the Brothers of St. John of God, Graz, Austria.

Leukemia 2018 Aug 20;32(8):1768-1777. Epub 2018 Jul 20.

Developmental Therapeutics Consortium, Chicago, IL, USA.

This is a pivotal, multicenter, open-label study of moxetumomab pasudotox, a recombinant CD22-targeting immunotoxin, in hairy cell leukemia (HCL), a rare B cell malignancy with high CD22 expression. The study enrolled patients with relapsed/refractory HCL who had ≥2 prior systemic therapies, including ≥1 purine nucleoside analog. Patients received moxetumomab pasudotox 40 µg/kg intravenously on days 1, 3, and 5 every 28 days for ≤6 cycles. Read More

Oncotarget 2018 Jun 22;9(48):28866-28876. Epub 2018 Jun 22.

Normandie Univ, INSERM U1245, Université de Caen, Caen, France.

Classical hairy cell leukemia (HCL-c) is a rare lymphoid neoplasm. mutation, detected in more than 80% of the cases, is described as a driver mutation, but additional genetic abnormalities appear to be necessary for the disease progression. For cases of HCL-c harboring a wild-type gene, the differential diagnosis of the variant form of HCL (HCL-v) or splenic diffuse red pulp lymphoma (SDRPL) is complex. Read More

Cell Cycle 2018 3;17(8):985-996. Epub 2018 Jul 3.

b School of Medicine , University of Colorado Anschutz Medical Campus , Aurora , CO , USA.

Cladribine (2CdA), a synthetic purine analog interfering with DNA synthesis, is a medication used to treat hairy cell leukemia (HCL) and B-cell chronic lymphocytic leukemia. Entinostat, a selective class I histone deacetylase (HDAC) inhibitor, shows antitumor activity in various human cancers, including hematological malignancies. The therapeutic potential of cladribine and entinostat against multiple myeloma (MM) remains unclear. Read More