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Cureus 2021 Apr 4;13(4):e14284. Epub 2021 Apr 4.

Public Health, Broward County Health Department, Fort Lauderdale, USA.

Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) is a rare, aggressive neoplasm that frequently relapses and requires the use of multiple treatment modalities. PC-ALCL most commonly presents in patients around the age of 60 and clinically manifests as red, single or sometimes grouped nodular lesions in the skin that tend to ulcerate over time. Although cases are limited to the skin, the extracutaneous spread has been occasionally reported. Read More

Medicine (Baltimore) 2021 May;100(18):e25770

Department of Radiation Oncology, General Hospital of Central Treater Command of PLA, Hongshan District, Wuhan, China.

Introduction: CD30+ primary cutaneous anaplastic large cell lymphoma (PC-ALCL) is a rare T-cell neoplasm, and has been reported to present with an indolent behavior. The PC-ALCL with aggressive behavior has not been reported in the literature.

Patient Concerns: We treated a patient with PC-ALCL that exhibited indolent behavior in the past 2 years and aggressive behavior within the last 3 months before presentation. Read More

J Am Acad Dermatol 2021 Apr 30. Epub 2021 Apr 30.

Memorial Sloan Kettering Cancer Center, New York, NY. Electronic address:

Primary cutaneous T-cell lymphomas (CTCLs) are defined as lymphomas with a T-cell phenotype that present in the skin without evidence of systemic or extracutaneous disease at initial presentation. CTCLs other than Mycosis Fungoides (MF) and Sézary syndrome (SS) account for approximately one-third of CTCLs and encompass a heterogenous group of non-Hodgkin lymphomas ranging from indolent lymphoproliferative disorders to aggressive malignancies with a poor prognosis. The spectrum of CTCLs continues to broaden as new provisional entities are classified. Read More

Clin Case Rep 2021 Apr 11;9(4):2373-2381. Epub 2021 Mar 11.

Herbert Wertheim College of Medicine at Florida International University Miami FL USA.

Anaplastic large-cell lymphoma (ALCL) is a CD30 + lymphoproliferative disorder that may manifest with skin involvement. We present a rare case of Agent Orange-induced ALCL with cutaneous involvement of the hand, surgical excision, and follow-up treatment. Read More

Mol Clin Oncol 2021 Jun 15;14(6):121. Epub 2021 Apr 15.

Unit of Dermatology, University of Padua, Padova I-35128, Italy.

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare CD30 lymphoproliferative disorder characterized by the development of lesions ranging from papules to large tumors. Most cases present as localized disease, however multifocal and generalized involvement of the skin can occur. Several treatments have been proposed for PCALCL; however a highly effective standard approach to multifocal disease has not yet been elucidated. Read More

Rev Med Liege 2021 Apr;76(4):224-231

) Service de Dermatologie, CHU Liège, Belgique.

Recently, brentuximab vedotin (BV) (Adcetris®) obtained the reimbursement in Belgium for the treatment of the primary cutaneous NKT-cell lymphomas mycosis fungoides (MF), large cell anaplastic lymphoma and lymphomatoid papulosis type A. BV is a monoclonal antibody directed against the CD30 expressed on tumoral T cells. The inhibition of this pathway releases the process of apoptosis leading to the cell death of the tumoral cells. Read More

Pediatr Dermatol 2021 Mar 19. Epub 2021 Mar 19.

Dermatology Program, Boston Children's Hospital, Boston, MA, USA.

Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) is a CD30+ lymphoproliferative disorder that rarely occurs in children. Although there are currently no consensus guidelines for the treatment of cutaneous lymphoma in the pediatric population, the isolated form of PC-ALCL is typically managed by surgical excision or external beam radiation therapy. We report the case of a 6-year-old girl with primary cutaneous anaplastic large cell lymphoma that was treated with brachytherapy with no recurrence after 21 months of follow-up, suggesting that brachytherapy may be considered as a treatment for pediatric cutaneous large cell anaplastic lymphoma. Read More

Am J Clin Pathol 2021 03;155(4):479-497

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.

Objectives: This review describes our approach to the diagnosis of all 4 anaplastic large cell lymphoma (ALCL) entities.

Methods: ALCLs are a group of CD30-positive mature T-cell lymphomas with similar morphologic and phenotypic characteristics but variable clinical and genetic features. They include systemic ALK-positive ALCL, systemic ALK-negative ALCL, primary cutaneous ALCL, and the recently described provisional entity breast implant-associated ALCL. Read More

Virchows Arch 2021 Feb 18. Epub 2021 Feb 18.

Department of Pathology, Dermatopathology Section, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Mycosis fungoides with large cell transformation (MFLCT) can be difficult to distinguish from primary cutaneous CD30+ T cell lymphoproliferative disorders (PC CD30+ LPD), especially primary cutaneous anaplastic large cell lymphoma (PC-ALCL). This diagnostic distinction is critical for appropriate patient management. GATA3 has been proposed to be useful in the discrimination between these two entities. Read More

BMJ Case Rep 2021 Feb 5;14(2). Epub 2021 Feb 5.

Department of Clinical Hematology, King George's Medical University, Lucknow, Uttar Pradesh, India.

We present a case of 50-year-old man with history of ulcerative right axillary mass for 6 months. Axillary lymphadenopathy and organomegaly were absent. Microscopic examination showed sheets of pleomorphic cells which were mitotically active. Read More

Diagn Pathol 2021 Jan 5;16(1). Epub 2021 Jan 5.

Department of Pathology, Fudan University Shanghai Cancer Center, 270 Dong-an Road, Xuhui District, Shanghai, 200032, China.

Background: Anaplastic large cell lymphoma (ALCL) with uniform CD56 expression is a rare condition, that has been described in limited literature, and its clinicopathological features have not yet been well illustrated. The aim of our study was to fully investigate the clinical, histological, immunohistochemical and molecular features of CD56+ ALCL.

Methods: The clinical and histological characteristics of CD56+ ALCL cases were retrospectively evaluated. Read More

Turk J Haematol 2021 02 4;38(1):49-56. Epub 2021 Jan 4.

İstanbul University, İstanbul Faculty of Medicine, Department of Pathology, İstanbul, Turkey

Objective: Lymphomatoid papulosis (LyP) is an indolent skin disease with variable clinical features classified among the primary cutaneous CD30+ T-cell lymphoproliferative disorders. It may show association with cutaneous and systemic lymphomas. We aimed to identify the frequency and characteristics of associated lymphomas among Turkish patients with LyP and to determine the risk factors for secondary lymphomas. Read More

Clin Lymphoma Myeloma Leuk 2021 Mar 13;21(3):e272-e276. Epub 2020 Dec 13.

Oncology Center, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is an uncommon lymphoproliferative disorder, mainly associated with textured implants. The average time from the breast implants to the development of BIA-ALCL is about 7 to 10 years, and the median age at the time of diagnosis is in the mid-50s. The exact incidence and prevalence of BIA-ALCL are not known. Read More

Cureus 2020 Oct 28;12(10):e11228. Epub 2020 Oct 28.

Dermatology, Colentina Clinical Hospital, Bucharest, ROU.

Primary cutaneous CD30-positive lymphoproliferative disorders represent the second most common subgroup of cutaneous T-cell lymphomas and include lymphomatoid papulosis, primary cutaneous anaplastic large cell lymphoma, and borderline lesions. Primary cutaneous anaplastic large cell lymphoma is characterized by the presence of solitary or localized nodules or tumors located on the extremities or the cephalic or cervical region. Large plaque parapsoriasis is a chronic inflammatory disorder that associates a high risk of progression to mycosis fungoides. Read More

Am J Surg Pathol 2021 Jun;45(6):796-802

Department of Pathology & Dermatology, The Ohio State University Wexner Medical Center, Columbus, OH.

Cutaneous anaplastic large-cell lymphoma (C-ALCL) represents one of the entities within the group of CD30-positive lymphoproliferative disorders of the skin. Most cases are ALK-negative, though isolated cases of ALK-positive C-ALCL have also been reported. By definition, the diagnosis of C-ALCL requires the expression of CD30 in >75% of the cells. Read More

J Oncol Pharm Pract 2020 Oct 25:1078155220968615. Epub 2020 Oct 25.

Pharmacy Department IUCT (Institut Universitaire du Cancer) Oncopole, Institut Claudius Regaud, Toulouse, France.

Introduction: Brentuximab vedotin (Bv) has been approved for the treatment of Refractory/Relapsed (R/R) Anaplastic Large Cell Lymphomas (ALCL) and cutaneous T-Cell Lymphomas, but is also effective in other CD30+ malignancies. We report here the outcomes of patients with various R/R Peripheral T Cell Lymphoma (PTCL) treated with Bv in real life practice.

Method: This was a retrospective, single-center study based on medical records of patients with R/R PTCL treated either with Bv alone or in combination with chemotherapy. Read More

Orbit 2020 Oct 1:1-7. Epub 2020 Oct 1.

Department of Neurosciences, Reproductive Sciences and Dentistry, University of Naples Federico II, Naples, Italy.

Purpose: Two new cases of primary cutaneous CD30+ anaplastic large-cell lymphoma (cALCL) of the eyelid are reported; these are analysed alongside existing cases to identify challenges relating to the diagnosis and management of such rare lesions.

Material And Methods: A review of existing literature on the PubMed database is conducted using the keywords: 'eyelid lymphoid proliferations', 'lymphoma of the eyelid', and 'primary cutaneous CD30+, ALK-anaplastic large-cell lymphoma of the eyelid'. Two new cases of cALCL are reported. Read More

J Coll Physicians Surg Pak 2020 Jul;30(7):749-753

Department of Nuclear Medicine, Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, Pakistan.

Objective: To describe clinical features and treatment options in pediatric patients with ALCL (Anaplastic large cell lymphoma) and their outcome over a span of 10 years.

Study Design: A retrospective-observational study.

Place And Duration Of Study: Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, from January 2005 to December 2015. Read More

Arch Soc Esp Oftalmol 2020 Sep 6;95(9):459-462. Epub 2020 Jun 6.

Servicio de Oftalmología, Hospital Virgen de los Lirios, Alcoy, Alicante, España.

Cutaneous anaplastic large-cell lymphoma (cALCL) is a condition within CD30 lymphoid proliferations spectrum. Involving the eyelid is unusual and all cases found in the literature are located in the upper eyelid. In this case we report an cALCL atypical presentation. Read More

Curr Hematol Malig Rep 2020 08;15(4):333-342

City of Hope National Medical Center, 1500 E. Duarte Road, Duarte, CA, 91010, USA.

Purpose Of Review: Primary cutaneous CD30+ T-cell lymphoproliferative disorders (CD30+ LPDs) are the second most common cutaneous lymphomas after mycosis fungoides and Sezary syndrome. They include primary cutaneous anaplastic large cell lymphoma (pcALCL), lymphomatoid papulosis (LyP), and borderline lesions. The purpose of this literature review is to consolidate the available evidence on the primary cutaneous CD30+ LPD in order to define the tools for correct diagnosis and appropriate treatment. Read More

J Am Acad Dermatol 2021 Jan 22;84(1):185-187. Epub 2020 Apr 22.

Department of Clinical Haematology, Peter MacCallum Cancer Centre and Royal Melbourne Hospital, Melbourne; Faculty of Medicine, Nursing, and Health Sciences, Monash University, Clayton; Sir Peter MacCallum Department of Oncology, University of Melbourne, Parkville, Victoria, Australia. Electronic address:

Radiographics 2020 May-Jun;40(3):609-628. Epub 2020 Apr 17.

From the Departments of Radiology (B.S., E.P.), Hematopathology (A.D.A., A.C.W.), Oncology (C.B.), and Hematology (S.S., S.I., D.E.S.), Royal Marsden Hospital, Fulham Road, London SW3 6JJ, England; Department of Medical Oncology, Royal Marsden Hospital Chelsea, London, England (A.J.R.); and University of Exeter Medical School, Exeter, England (R.S.).

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a new provisional category in the 2016 World Health Organization (WHO) classification of lymphoid neoplasms, and its incidence is rising owing to increasing recognition of this complication of breast implant insertion. At a median of 10 years after implant insertion, the typical presenting features are sudden-onset breast swelling secondary to peri-implant effusion and less frequently mass-forming disease. Histologic features comprise pleomorphic cells expressing CD30 and negative anaplastic lymphoma kinase (ALK) receptor, similar to systemic and cutaneous ALK-negative anaplastic large cell lymphoma (ALCL). Read More

J Hematol 2019 Sep 30;8(3):132-136. Epub 2019 Sep 30.

Department of Medicine, Division of Medical Oncology and Hematology, Mayo Clinic Health System, 1221 Whipple St., Eau Claire, WI, 54703, USA.

Primary cutaneous anaplastic large cell lymphoma (pcALCL) is a rare form of non-Hodgkins lymphoma. Current frontline treatments for pcALCL include surgical resection, anthracycline-based chemotherapy, and/or radiation therapy (RT) depending on disease severity. While brentuximab vedotin (BV) has been used for refractory/relapsed cases, it recently received Food and Drug Administration (FDA) approval for use in combination with chemotherapy for peripheral T-cell lymphomas. Read More

Case Rep Dermatol Med 2020 25;2020:9435242. Epub 2020 Mar 25.

Skin Cancer Center, Department of Dermatology, Ruhr-University Bochum, Bochum, Germany.

Actinic reticuloid (AR)-a subtype of chronic actinic dermatitis-clinically and histopathologically shows lymphoma-like features. We report a male patient initially diagnosed with erythrodermic cutaneous T cell lymphoma (CTCL) who developed severe broadband photosensitivity. Clinical evaluation, histopathology, and phototesting were consistent with AR. Read More

Pharmacoecon Open 2020 Dec;4(4):563-574

Faculty of Health and Life Sciences, University of Liverpool, Thompson Yates Building, Liverpool, L69 3GB, UK.

As part of the single technology appraisal process, the National Institute for Health and Care Excellence invited Takeda UK Ltd to submit clinical- and cost-effectiveness evidence for brentuximab vedotin (BV) for treating relapsed or refractory CD30-positive (CD30+) cutaneous T-cell lymphoma (CTCL). The Liverpool Reviews and Implementation Group at the University of Liverpool was commissioned to act as the evidence review group (ERG). This article summarises the ERG's review of the company's submission for BV and the appraisal committee (AC) decision. Read More

PLoS One 2020 12;15(2):e0228751. Epub 2020 Feb 12.

Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, MD, United States of America.

Background: Primary cutaneous CD30+ lymphoproliferative disorders (CD30CLPD) are the second most common type of cutaneous T cell lymphoma (CTCL) and include lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (pcALCL). Case reports and small patient series suggest an association of CD30CLPD with atopic disorders. However, the prevalence of atopy in patients with CD30CLPD in retrospective studies depends on patients' recall which is not always reliable. Read More

Dermatol Online J 2019 Nov 15;25(11). Epub 2019 Nov 15.

Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, PA.

CD30+ T cell pseudolymphomas (CD30+ PSL) are a group of benign inflammatory cutaneous disorders that can develop in settings of viral infections or drug reactions. Owing to their histological similarities to malignant lymphomas, these benign infiltrates are occasionally misdiagnosed as malignant, causing significant concerns for patients and physicians. Herein, we report a patient with CD30+ PSL associated with molluscum contagiosum whose initial biopsy revealed atypical large CD30-expressing cells, leading to a misdiagnosis of primary cutaneous anaplastic large cell lymphoma and referral to our cutaneous lymphoma clinic. Read More

J Dermatol Sci 2020 Mar 25;97(3):187-193. Epub 2020 Jan 25.

Department of Dermatology, Kyoto Prefectural University of Medicine, Kyoto, Japan.

Background: The types of cutaneous lymphoma (CL) and their incidences can vary among geographic areas or ethnic groups.

Objective: This study aimed to investigate the incidence of various CL types in Japan using epidemiological data from a nationwide registration system for CL.

Methods: A questionnaire was sent to participating hospitals, all of which had been approved to conduct residency programs for board-certified dermatologists by the Japanese Dermatological Association. Read More

Curr Hematol Malig Rep 2020 02;15(1):9-19

Division of Oncology, Department of Medicine, Washington University School of Medicine, 660 S. Euclid, Box 8056, St. Louis, MO, 63110, USA.

Purpose Of Review: The recent development of brentuximab vedotin (BV), an antibody-drug conjugate targeting CD30-positive cells, has led to therapeutic advances in the treatment of T cell lymphomas. In this review, we discuss key studies of BV in peripheral T cell lymphoma (PTCL) and cutaneous T cell lymphoma (CTCL) and highlight important questions for further investigation.

Recent Findings: Monotherapy with BV has proven to be effective and well tolerated in patients with relapsed/refractory (R/R) CD30-positive CTCL. Read More