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Pathology 2022 May 13. Epub 2022 May 13.

Department of Pathology, APHP, Henri Mondor Hospital, Creteil, France; Paris-Est Creteil University and INSERM U955 Team NFL, Creteil, France. Electronic address:

Mycosis fungoides (MF) and primary cutaneous CD30-positive T-cell lymphoproliferative disorders (CD30LPD) are the most frequent primary cutaneous T-cell lymphomas. Our objective was to study pan-T-cell antigens and PD-1 expression in a large cohort of MF and CD30LPD with a special interest in antigen losses as a diagnostic tool. We retrospectively reviewed 160 consecutive samples from 153 patients over a 3 year period, including 104 with MF and 49 with CD30LPD. Read More

Hematol Rep 2022 Mar 23;14(2):61-66. Epub 2022 Mar 23.

Department of Biomedicine and Prevention, University Tor Vergata, 00133 Rome, Italy.

Primary cutaneous CD30+ lymphoproliferative disorders include primary cutaneous anaplastic large cell lymphoma (pcALCL) and lymphomatoid papulosis. The prognosis of the disease is usually excellent but, in a minority of cases, it presents with extracutaneous involvement and aggressive behavior. The case we present-relapsed after surgical excision, immunosuppressive therapy, and conventional chemotherapy-is the first one treated with Autologous Stem Cell transplant followed by Brentuximab Vedotin consolidation, a scheme already used for high risk Hodgkin Lymphoma. Read More

Virchows Arch 2022 Apr 2. Epub 2022 Apr 2.

Institute of Pathology, Department of Laboratory Medicine and Pathology, Lausanne University Hospital and Lausanne University, Rue du Bugnon 25, CH-1011, Lausanne, Switzerland.

DUSP22 gene rearrangements are recurrent in systemic and cutaneous ALK-negative anaplastic large cell lymphomas, rarely encountered in other cutaneous CD30+ lymphoproliferations, and typically absent in other peripheral T-cell lymphomas. We report the case of a 51-year-old woman, with longstanding celiac disease and a rapidly enlarging leg ulcer, due to a DUSP22-rearranged CD30+ T-cell lymphoproliferation. Subsequent history revealed an intestinal enteropathy-associated T-cell lymphoma (EATL). Read More

Life (Basel) 2022 Mar 11;12(3). Epub 2022 Mar 11.

Department of Laboratory Medicine and Pathology, Saint Louis University, St. Louis, MO 63104, USA.

Peripheral T-Cell lymphoma (PTCL) comprises a heterogenous group of uncommon lymphomas derived from mature, post-thymic or "peripheral" T- and natural killer cells. The World Health Organization (WHO) emphasizes a multiparameter approach in the diagnosis and subclassification of these neoplasms, integrating clinical, morphologic, immunophenotypic, and genetic features into the final diagnosis. Clinical presentation is particularly important due to histologic, immunophenotypic and genetic variations within established subtypes, and no convenient immunophenotypic marker of monoclonality exists. Read More

JAAD Case Rep 2022 Apr 10;22:34-37. Epub 2022 Feb 10.

Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.

Am J Dermatopathol 2022 May;44(5):376-379

Departments of Pathology, and.

Abstract: Primary cutaneous anaplastic large-cell lymphoma (C-ALCL) is a cutaneous CD30-positive lymphoproliferative disorder. The patients usually present with single or multiple cutaneous nodules or papules and about 10% cases present with extracutaneous manifestations, which are predominantly in the form of regional lymph nodal involvement. Visceral involvement especially pulmonary or hepatic involvement in C-ALCL is only rarely described in the scientific literature. Read More

Cells 2022 02 9;11(4). Epub 2022 Feb 9.

Division of Dermatology, McGill University, Montreal, QC H4A 3J1, Canada.

Cutaneous T cell lymphoma (CTCL) is a spectrum of lymphoproliferative disorders caused by the infiltration of malignant T cells into the skin. The most common variants of CTCL include mycosis fungoides (MF), Sézary syndrome (SS) and CD30 Lymphoproliferative disorders (CD30 LPDs). CD30 LPDs include primary cutaneous anaplastic large cell lymphoma (pcALCL), lymphomatoid papulosis (LyP) and borderline CD30 LPD. Read More

JID Innov 2022 Jan 15;2(1):100068. Epub 2021 Nov 15.

Department of Pathology, City of Hope National Medical Center, Duarte, California, USA.

Primary cutaneous CD30 T-cell lymphoproliferative disorders are the second most common cutaneous lymphomas. According to the World Health Organization, CD30 T-cell lymphoproliferative disorders include primary cutaneous anaplastic large cell lymphoma (C-ALCL) and lymphomatoid papulosis (LyP) as well as borderline lesions. C-ALCL and LyP are thought to represent two ends of a spectrum of diseases that have different clinical presentations, clinical courses, and prognoses in their classic forms but share the same histology of medium to large CD30 atypical lymphoid cell infiltrates. Read More

Am J Dermatopathol 2022 Mar;44(3):196-197

Serviço de Anatomia Patológica, Instituto Português de Oncologia de Lisboa Francisco Gentil EPE, Serviço de Anatomia Patológica, Lisboa, Portugal.

Abstract: Primary cutaneous anaplastic large cell lymphoma may harbor a 6p25.3 rearrangement, which has been associated with an epidermotropic small cell component. We report the case of a patient with said lymphoma harboring that rearrangement. Read More

JID Innov 2021 Sep 15;1(3):100034. Epub 2021 Jun 15.

Comprehensive Cancer Center Mainfranken, University of Würzburg, Würzburg, Germany.

The emergence of a common progenitor cell has been postulated for the association of CD30-positive lymphoproliferative disease (LPD) and mycosis fungoides (MF) within the same patient. Up to now, no comprehensive analysis has yet addressed the genetic profiles of such concurrent lymphoma subtypes. We aimed to delineate the molecular alterations of clonally related CD30-positive LPD and MF occurring in the same two patients. Read More

Am J Dermatopathol 2022 Jan;44(1):62-65

Department of Pathology, City of Hope Comprehensive Cancer Center, Duarte, CA; and.

Abstract: Primary cutaneous gamma-delta T-cell lymphoma is a rare and aggressive neoplasm, representing less than 1% of all cutaneous T-cell lymphomas. In this article, we report the case of a 49-year-old woman who presented with a history of generalized skin rash and a recent mass on the left upper extremity, as well as right inguinal soft tissue swelling and splenomegaly. Histologic examination of the mass revealed a diffuse subcutaneous infiltrate of large anaplastic and CD30-positive lymphoid cells with rimming of the adipocytes. Read More

J Cutan Pathol 2022 Mar 8;49(3):306-309. Epub 2021 Nov 8.

Department of Dermatology, University of Virginia, Charlottesville, Virginia, USA.

An 81-year-old male presented with a rapidly growing cheek nodule. Biopsy revealed a dermal infiltrate of large atypical cells, some exhibiting a horseshoe-shaped nucleus. Immunohistochemistry revealed positivity for CD4, CD3, CD45, and CD30 (>95%). Read More

J Cutan Pathol 2022 Mar 7;49(3):299-305. Epub 2021 Nov 7.

Department of Dermatology, University of Michigan, Ann Arbor, Michigan, USA.

The DUSP22-IRF4 gene rearrangement results in downregulation of DUSP22, a presumed tumor suppressor in T-cell lymphomagenesis. It has been described in some cases of primary cutaneous and systemic anaplastic large-cell lymphoma, lymphomatoid papulosis, and transformed mycosis fungoides. Here we describe two patients with clinical lesions resembling patch/plaque mycosis fungoides that did not meet WHO criteria for large-cell transformation on histopathology yet showed a DUSP22 translocation. Read More

JAAD Case Rep 2021 Nov 20;17:34-37. Epub 2021 Sep 20.

Department of Dermatology, The University of Texas MD Anderson Cancer Center, Houston, Texas.

J Cutan Pathol 2022 Feb 20;49(2):187-190. Epub 2021 Oct 20.

Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.

Primary cutaneous anaplastic large-cell lymphoma (pC-ALCL) is distinguished from systemic anaplastic large-cell lymphoma (S-ALCL) with cutaneous involvement. Although pC-ALCL is a unique entity with different genetics, clinical characteristics, and prognosis, its causes are unknown. Herein, we report the case of a Chinese woman with a 4-month history of a gradually enlarged ulcerative mass in her right forearm following an unidentified insect bite. Read More

Cancers (Basel) 2021 Sep 17;13(18). Epub 2021 Sep 17.

Department of Pathology, Hospital Español, Mexico City 11520, Mexico.

Anaplastic large cell lymphoma (ALCL) is a subtype of CD30+ large T-cell lymphoma (TCL) that comprises ~2% of all adult non-Hodgkin lymphomas. Based on the presence/absence of the rearrangement and expression of anaplastic lymphoma kinase (ALK), ALCL is divided into ALK+ and ALK-, and both differ clinically and prognostically. This review focuses on the historical points, clinical features, histopathology, differential diagnosis, and relevant cytogenetic and molecular alterations of ALK- ALCL and its subtypes: systemic, primary cutaneous (pc-ALCL), and breast implant-associated (BIA-ALCL) Recent studies have identified recurrent genetic alterations in this TCL. Read More

Nat Commun 2021 09 22;12(1):5577. Epub 2021 Sep 22.

Division of Haematopathology, European Institute of Oncology IRCCS, Milan, Italy.

Anaplastic large cell lymphoma (ALCL), an aggressive CD30-positive T-cell lymphoma, comprises systemic anaplastic lymphoma kinase (ALK)-positive, and ALK-negative, primary cutaneous and breast implant-associated ALCL. Prognosis of some ALCL subgroups is still unsatisfactory, and already in second line effective treatment options are lacking. To identify genes defining ALCL cell state and dependencies, we here characterize super-enhancer regions by genome-wide H3K27ac ChIP-seq. Read More

Blood Adv 2021 12;5(23):5098-5106

Division of Cancer Medicine, Peter MacCallum Cancer Centre, Sir Peter MacCallum Department of Oncology and Epworth Healthcare, The University of Melbourne, Melbourne, VIC, Australia.

The primary analysis of the phase 3 ALCANZA trial showed significantly improved objective responses lasting ≥4 months (ORR4; primary endpoint) and progression-free survival (PFS) with brentuximab vedotin vs physician's choice (methotrexate or bexarotene) in CD30-expressing mycosis fungoides (MF) or primary cutaneous anaplastic large-cell lymphoma (C-ALCL). Cutaneous T-cell lymphomas often cause pruritus and pain; brentuximab vedotin improved skin symptom burden with no negative effects on quality of life. We report final data from ALCANZA (median follow-up, 45. Read More

Haematologica 2021 08 12. Epub 2021 Aug 12.

Department of Dermatology, Leiden University Medical Center, Leiden.

Primary cutaneous anaplastic large cell lymphoma (pcALCL), a hematological neoplasm caused by skin-homing CD30+ malignant T cells, is part of the spectrum of primary cutaneous CD30+ lymphoproliferative disorders. To date, only a small number of molecular alterations have been described in pcALCL, and so far, no clear unifying theme that could explain the pathogenetic origin of the disease has emerged among patients. To clarify the pathogenetic basis of pcALCL, we performed a high-resolution genetic profiling (genome/transcriptome) of this lymphoma (n=12) by using whole-genome sequencing, whole-exome sequencing and RNA sequencing. Read More

BMJ Case Rep 2021 Jul 21;14(7). Epub 2021 Jul 21.

Dermatology, University of Arkansas for Medical Sciences College of Medicine, Little Rock, Arkansas, USA

Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) is a rare non-Hodgkin's lymphoma that arises as a single, or multiple dome-shaped tumours on the skin. The histology is characterised by the presence of atypical lymphocytes with large irregularly shaped nuclei that express the surface marker CD30. There can be significant heterogeneity in clinical manifestation and histological pattern and in rare cases accurate diagnosis can be a challenge. Read More

J Cutan Pathol 2021 Dec 21;48(12):1463-1470. Epub 2021 Jul 21.

Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing, Jiangsu, China.

Background: Primary cutaneous CD30+ lymphoproliferative diseases are the second most common group of cutaneous T-cell lymphomas, including lymphomatoid papulosis (LyP), primary cutaneous anaplastic large-cell lymphoma (pcALCL), and borderline cases. These diseases form a spectrum and may show overlapping histopathological, phenotypic, and genetic features. In the 2016 WHO classification, LyP with 6p25. Read More

Am J Hematol 2021 08 26;96(8):1027-1046. Epub 2021 Jun 26.

Department of Hematology/Hematopoietic Cell Transplantation, City of Hope Medical Center, Duarte, California, USA.

Introduction: Aggressive T-cell lymphomas continue to have a poor prognosis. There are over 27 different subtypes of peripheral T-cell lymphoma (PTCL), and we are now beginning to understand the differences between the various subtypes beyond histologic variations.

Molecular Pathogenesis Of Various Subtypes Of Ptcl: Gene expression profiling (GEP) can help in diagnosis and prognostication of various subtypes including PTCL-nos and anaplastic large cell lymphoma (ALCL). Read More

Clin Exp Dermatol 2021 Dec 17;46(8):1420-1426. Epub 2021 Jul 17.

Department of Oncology, Houston Methodist Cancer Center, Houston, TX, USA.

Introduction: Primary cutaneous anaplastic large-cell lymphoma (PC-ALCL) is a rare T-cell lymphoma. A prior analysis of the Surveillance, Epidemiology, and End Results (SEER) database reported only 157 cases of localized primary cutaneous CD30+ T-cell lymphoproliferative disorders (PC-ALCL and lymphomatoid papulosis) from 1973 to 2004. Our analysis of the SEER database since 2004 is the largest to date and our results improve our understanding of this disease and their potential prognostic factors. Read More

Med Clin North Am 2021 Jul;105(4):737-755

Department of Dermatology, VU Cutaneous Lymphoma Clinic, Vanderbilt University Medical Center, Vanderbilt Dermatology, One Hundred Oaks, 719 Thompson Lane, Suite 26300, Nashville, TN 37204-3609, USA. Electronic address:

The cutaneous lymphomas are malignancies of T-cell and B-cell lymphocytes in which the skin is the primary organ of involvement. The cutaneous T-cell lymphomas include variants that can mimic the presentation of common skin diseases or arthropod bites. Mycosis fungoides, the most common cutaneous T-cell lymphoma, usually presents as fixed asymptomatic patches or plaques in sun-protected areas. Read More

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 11 30;85(5):1073-1090. Epub 2021 Apr 30.

Memorial Sloan Kettering Cancer Center, New York, New York. 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 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