DOCSLIB.ORG

A Review of the Solitary Cutaneous T-Cell Lymphomas

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
A Review of the Solitary Cutaneous T-Cell Lymphomas J Cutan Pathol 2014 © 2014 John Wiley & Sons A/S. doi: 10.1111/cup.12353 Published by John Wiley & Sons Ltd John Wiley & Sons. Printed in Singapore Journal of Cutaneous Pathology Review A review of the solitary cutaneous T-cell lymphomas Cutaneous T-cell lymphomas (CTCL) account for almost 65-92% Mina S. Ally1 and Alistair of all cutaneous lymphomas, many of which usually present with Robson2 multiple lesions. However, a number of well-recognized and rare 1Department of Dermatology, Stanford types of CTCL, including mycosis fungoides, can present in University School of Medicine, Redwood City, isolated fashion. These solitary lesions often run a relatively CA, USA, and indolent clinical course but often pose diagnostic difficulties. We 2St. John’s Institute of Dermatology, review histopathologically challenging solitary cutaneous T-cell St. Thomas’ Hospital, London, UK lymphomas, including criteria for diagnosis, clinical course and prognosis, particularly for primary cutaneous CD4+ small/medium pleomorphic lymphoma and indolent CD8+ lymphoid proliferation of acral sites. In addition, we suggest an algorithm and nomenclature to aid in the diagnosis of such problematic lesions. Keywords: solitary mycosis fungoides, pagetoid reticulosis, indolent Alistair Robson, FRCPath Dip RCPath, CD8+ lymphoid proliferation of acral sites, primary cutaneous St. John’s Institute of Dermatology, St. Thomas’ CD4+ small/medium pleomorphic lymphoma Hospital, Westminster Bridge Road, London SE1 7EH, UK Ally MS, Robson A. A review of the solitary cutaneous T-cell Tel: 0207 1887188 Fax: +44 207 1886382 lymphomas. e-mail: [email protected] J Cutan Pathol 2014. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd Accepted for publication December 16, 2013 Primary cutaneous T-cell lymphomas present run a relatively indolent clinical course, they in the skin with no evidence of extra-cutaneous continue to pose diagnostic difficulty. involvement.1 Cutaneous T-cell lymphomas In this review, we present an overview of the (CTCL) account for almost 65–92% of all cuta- solitary cutaneous T-cell lymphomas that, in neous lymphomas,2 of which, mycosis fungoides our opinion, present the greatest challenge in (MF) is the most common and characteristically diagnosis and that have been emerging over the presents with multiple lesions. However, solitary last 5 years. We have excluded some of the more MF, including and distinct from pagetoid retic- clinically aggressive tumors that may present as ulosis (Woringer-Kolopp), is well documented;3 solitary lesions as they tend to be well recognized similarly, other well-characterized forms of and histopathologically and immunopheno- lymphoma can present as an isolated lesion. typically distinct. We discuss the criteria for Finally, a number of increasingly recognized diagnosis, particularly for primary cutaneous rarer CTCL commonly present with an isolated CD4+ small/medium pleomorphic lymphoma lesion, including CD4+ small/medium pleomor- and indolent CD8+ lymphoid proliferation phic T-cell lymphoma (SMPTCL) and indolent of acral sites. Finally, we suggest an algorithm CD8+ lymphoid proliferation of acral sites.4 to aid in the diagnosis of such problematic Although it seems that these isolated lesions lesions. 1 Review Cutaneous lymphoma classification and review single erythematous scaly patch or plaque, in non-sun-exposed areas.1 Solitary MF has been The recent détente and concord between 3,13,14 the World Health Organization (WHO) and reported to have an excellent prognosis, European Organization for the Research and although recurrence of treated lesions has been 2,13,15–18 Treatment of Cancer (EORTC) classifications reported six of which occurred distant acknowledges the unique features of primary from the site of the original lesion (Table 1). Two cutaneous lymphomas, which differ greatly from of these distant recurrences presented as multi- the nodal and other extranodal lymphomas.1,5 ple lesions, but in both cases, there was complete An accurate diagnosis of primary cutaneous resolution with further treatment and no further 2 lymphoma requires the correlation of the clini- disease at follow-up. The other four cases pre- 11,17 cal findings with the microscopic, immunophe- sented as a unilesional recurrence. In addi- notypic and genetic features. Given that the tion, four cases of solitary MF have progressed to further cutaneous involvement, three of which majority of lymphomas usually present with mul- , , , tiple lesions it is difficult to make a diagnosis had large cell transformation.2 8 16 19 of a specific cutaneous lymphoma when faced Solitary lesions of follicular and syringotropic with the clinical presentation of a solitary lesion. MF are also clinically and pathologically indis- Moreover, in the absence of multiple lesions the tinguishable from lesions seen in classical dis- surgical pathologist is frequently in difficulty in ease. Solitary folliculotropic MF presents as an determining whether an infiltrate is neoplastic infiltrated patch, plaque or an isolated areaof or reactive. follicular papules without prominent scaling, , , To date, of the solitary cutaneous T-cell usually in the head and neck area.2 6 8 Solitary lymphomas reported in the literature, approxi- syringotropic MF usually presents with a single mately 166 cases represent MF,3 231 represent erythematous, indurated plaque, often studded SMPTCL, 22 represent indolent CD8+ lym- with small pinhead-sized papules, in the trunk, , phoid proliferation and 62 represent pagetoid hip and thigh area,11 20 although cases have reticulosis (Table 1). been reported in the head and neck area.11,12 Alopecia is a prominent feature in both cases.11 Solitary MF There is still uncertainty about the relation- ship between these two MF subtypes, but they MF accounts for almost 50% of all primary cuta- often co-exist.6,11,20 When compared with clas- neous lymphomas and is characterized by mul- 1 sic MF, folliculotropic MF is typically associated tiple patches and plaques. Indeed, given that with a poorer prognosis.21 This is sometimes,8 the finding of multiple patches and plaques but not always the case for solitary lesions.2,6 is a defining feature it is somewhat contradic- Syringotropic MF, however, has a prognosis sim- tory to consider a solitary variant. Nevertheless, ilar to that of classic MF.22 Out of the nine soli- since 1981, 166 solitary cases of MF have been tary cases of syringotropic MF reported, one has described that are distinct from pagetoid retic- shown recurrence of the lesion distant from the ulosis, including 10 of the folliculotropic variant 11 , , original site. (follicular MF)2 3 6–8 and 9 cases of syringotropic Finally, the report of an EORTC workshop con- MF.9–12 cerning granulomatous MF and granulomatous slack skin (GSS) detailed one solitary example Clinical features of GSS.23 Recently, at the senior author’s insti- Solitary MF, clinically and histopathologically tution, a case of GSS presented with a single resembles classic MF. Clinically, it presents as a pendulous axillary skin fold, which was followed Table 1. Number of reports of each solitary cutaneous T-cell lymphoma and reported instances of recurrence or disease progression Cutaneous Cases with cutaneous/ Cases with lesion recurrence lymphoma type Total cases systemic progression post-treatment* Solitary MF 166 4 (Cutaneous spread, 3 × large cell transformation) 15 (six distant from original site) Pagetoid reticulosis (Woringer-Kolopp) 62 1 (Cutaneous spread) 8 (1 distant from original site) SMPTCL 231 5 (Extracutaneous spread, one died of lymphoma) 12 Indolent CD8+ lymphoid proliferation of 22 0 4 (one distant from original site) acral sites *Recurrence occurred at the same site of the original lesions unless stated otherwise. 2 Review Fig. 2. ’Lining up’ of atypical lymphocytes at the dermoepider- mal junction and fibrotic changes in the papillary dermis are Fig. 1. A solitary plaque of granulomatous slack skin, affecting noted in a solitary patch of mycosis fungoides involving the the groin. thigh of a 75-year male (hematoxylin/eosin, ×100). several years later with involvement of the groin 24 3 loss of T-cell associated antigens, T-cell clonality (Fig. 1). A recent report by Ally et al. of 15 and appropriate clinical appearance. cases suggested that the observation of solitary MF might be expected, albeit exceptional, in Immunocytochemistry which the disease is simply found at an uncom- monly early stage. The unsurprising excellent Immunophenotypically, solitary MF, like classic prognosis of such cases indicates that therapy MF, is usually a neoplasm of CD4+ T-helper shouldaimtobecurative. cells, although CD8+ and double negative CD4−/CD8− variants have been described.28 In addition, there may be variable antigen loss of Histopathologic features CD2, CD5 and CD7.3 These immunophenotypes In solitary MF, microscopic sections show a super- do not seem to affect prognosis.28,29 ficial lymphoid infiltrate of epidermotropic and atypical lymphocytes.20,25 Atypia is usually more pronounced in the epidermotropic lymphocytes T-cell receptor gene analysis than those in the dermis.26,27 Other histopatho- T-cell receptor (TCR) gene rearrangements are logic features include lining up of atypical detected in approximately 57–71% of cases of lymphocytes at the dermoepidermal junction, early MF,29 so solitary lesions might be expected Pautrier microabscesses and fibrotic changes in to have a similar or perhaps lower clonal preva- the papillary dermis, none of which
Load more

Recommended publications
  • Lymphomatoid Papulosis and Recurrent Transitional Cell Carcinoma
    Volume 20 Number 4 April 2014 Letter Lymphomatoid papulosis and recurrent transitional cell carcinoma of the bladder: a paraneoplastic association WMS Leong1, CWD Aw1, KB Tan2 Dermatology Online Journal 20 (4): 17 1Division of Dermatology, National University Hospital, Singapore 2Department of Pathology, National University Hospital, Singapore Correspondence: Wai Mun Sean Leong, M.B.B.S. University Medicine Cluster National University Hospital 5 Lower Kent Ridge Road Singapore 119074 Phone: 67795555 Email: [email protected] Abstract Lymphomatoid papulosis is a rare, papulonodular skin eruption with histologic features of a CD30+ T cell lymphoma. We present a 79-year-old man with lymphomatoid papulosis and transitional cell carcinoma of the bladder. Introduction Lymphomatoid papulosis (LyP) is a rare, recurrent, self-healing papulonodular skin eruption with histologic features of a CD30+ malignant T cell lymphoma [1]. It belongs to a group of cutaneous CD30+ lymphoproliferative disorders, which includes cutaneous T cell lymphoma [2]. Patients with LyP are at risk of developing a second cutaneous or nodal lymphoma [3,4]. However, an association with transitional cell carcinoma (TCC) of the bladder has not been reported in the literature. This case illustrates a possible association between these two conditions. Case synopsis A 79-year-old man complained of a slightly itchy rash on the arms, neck, and shoulders for one year. Betamethasone- gentamicin cream previously afforded partial improvement. Approximately 9 months prior to presentation at the dermatology clinic, he was diagnosed to have TCC of the bladder (T1G3) and had undergone a trans-urethral resection of bladder cancer (TURBT) in December 2006 and January 2007.
    [Show full text]
  • Comorbidities in Mycosis Fungoides and Racial Differences In
    medicines Article Comorbidities in Mycosis Fungoides and Racial Differences in Co-Existent Lymphomatoid Papulosis: A Cross-Sectional Study of 580 Patients in an Urban Tertiary Care Center Subuhi Kaul 1,*, Micah Belzberg 2, John-Douglas Matthew Hughes 3, Varun Mahadevan 2 , Raveena Khanna 2, Pegah R. Bakhshi 2, Michael S. Hong 2, Kyle A. Williams 2, 2 2, 2, Annie L. Grossberg , Shawn G. Kwatra y and Ronald J. Sweren y 1 Department of Medicine, John H. Stroger Hospital of Cook County, Chicago, IL 60612, USA 2 Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA; [email protected] (M.B.); [email protected] (V.M.); [email protected] (R.K.); [email protected] (P.R.B.); [email protected] (M.S.H.); [email protected] (K.A.W.); [email protected] (A.L.G.); [email protected] (S.G.K.); [email protected] (R.J.S.) 3 Section of Dermatology, University of Calgary, Alberta, AB T2N 1N4, Canada; [email protected] * Correspondence: [email protected]; Tel.: +1-312-864-7000 Considered co-senior authors. y Received: 26 October 2019; Accepted: 24 December 2019; Published: 26 December 2019 Abstract: Background: Mycosis fungoides (MF) is a cutaneous T-cell lymphoma. Previous reports have suggested MF is associated with inflammatory conditions such as psoriasis, increased cardiovascular risk factors as well as secondary neoplasms. Methods: A cross-sectional study of MF patients seen from 2013 to 2019 was performed. Comorbidities were selected based on the 2015 Medicare report highlighting the most common chronic medical illnesses in the United States.
    [Show full text]
  • Primary Cutaneous Anaplastic Large Cell Lymphoma / Lymphomatoid
    Primary Cutaneous CD30-Positive T-cell Lymphoproliferative Disorders Definition A spectrum of related conditions originating from transformed or activated CD30-positive T-lymphocytes May coexist in individual patients Clonally related Overlapping clinical and/or histological features Clinical, histologic, and phenotypic characteristics required for diagnosis Types 1. Primary cutaneous anaplastic large cell lymphoma (C-ALCL) 2. Lymphomatoid papulosis 3. Borderline lesions C-ALCL: Definition T-cell lymphoma, presenting in the skin and consisting of anaplastic lymphoid cells, the majority of which are CD30- positive Distinction from: (a) systemic ALCL with cutaneous involvement, and (b) secondary high-grade lymphomas with CD30 expression In nearly all patients disease is limited to the skin at the time of diagnosis Assessed by meticulous staging Patients should not have other subtypes of lymphoma C-ALCL: Synonyms Lukes-Collins: Not listed (T-immunoblastic) Kiel: Anaplastic large cell Working Formulation: Various categories (diffuse large cell; immunoblastic) REAL: Primary cutaneous anaplastic large cell (CD30+) lymphoma Related terms: Regressing atypical histiocytosis; Ki-1 lymphoma C-ALCL: Epidemiology 25% of the T-cell lymphomas arising primarily in the skin. Predominantly in adults/elderly and rare in children. The male to female ratio is 1.5-2.0:1. C-ALCL: Sites of Involvement The disease is nearly always limited to the skin at the time of diagnosis Extracutaneous dissemination may occur Mainly regional lymph nodes Involvement of other organs is rare C-ALCL: Clinical Features Most present solitary or localized skin lesions which may be tumors, nodules or (more rarely) papules Multicentric cutaneous disease occurs in 20% Lesions may show partial or complete spontaneous regression (similar to lymphomatoid papulosis) Cutaneous relapses are frequent Extracutaneous dissemination occurs in approximately 10% of the patients.
    [Show full text]
  • Bexarotene (Targretin) Capsules Reference Number: PA.CP.PHAR.75 Effective Date: 09/11 Revision Log Last Review Date: 04/18
    Clinical Policy: Bexarotene (Targretin) Capsules Reference Number: PA.CP.PHAR.75 Effective Date: 09/11 Revision Log Last Review Date: 04/18 Description The intent of the criteria is to ensure that patients follow selection elements established by Pennsylvania Health and Wellness ® clinical policy for bexarotene (Targretin®) capsules FDA Approved Indication(s) Targretin is indicated for the treatment of cutaneous manifestations of cutaneous T-cell lymphoma (CTCL) in patients who are refractory to at least one prior systemic therapy. Policy/Criteria It is the policy of Pennsylvania Health and Wellness that Targretin is medically necessary when the following criteria are met: I. Initial Approval Criteria A. Cutaneous T-Cell Lymphoma (must meet all): 1. Diagnosis of cutaneous T-cell lymphoma (CTCL) (see Appendix B for CTCL subtypes); 2. Prescribed by or in consultation with an oncologist; 3. Age ≥ 18 years; 4. Dose does not exceed 400mg/m2 daily; Approval duration: 6 months B. Other diagnoses/indications: Refer to PA.CP.PHAR.57 - Global Biopharm Policy. II. Continued Approval A. Cutaneous T-Cell Lymphoma (must meet all): 1. Currently receiving medication via Pennsylvania Health and Wellness benefit or member has previously met all initial approval criteria; or the Continuity of Care policy (PA.LTSS.PHAR.01) applies; 2. Member is responding positively to therapy; 3. Dose does not exceed 400mg/m2 daily; Approval duration: 6months B. Other diagnoses/indications (must meet 1 or 2): 1. Currently receiving medication via Pennsylvania Health and Wellness benefit and documentation supports positive response to therapy; or the Continuity of Care policy (PA.LTSS.PHAR.01) applies; Approval duration: Duration of request or 6 months (whichever is less); or 2.
    [Show full text]
  • 18F-FDG Avidity in Lymphoma Readdressed: a Study of 766 Patients
    Journal of Nuclear Medicine, published on December 15, 2009 as doi:10.2967/jnumed.109.067892 18F-FDG Avidity in Lymphoma Readdressed: A Study of 766 Patients Michal Weiler-Sagie1, Olga Bushelev2, Ron Epelbaum2,3, Eldad J. Dann2,4,5, Nissim Haim2,3, Irit Avivi2,5, Ayelet Ben-Barak6, Yehudit Ben-Arie2,7, Rachel Bar-Shalom1,2, and Ora Israel1,2 1Department of Nuclear Medicine, Rambam Health Care Campus, Haifa, Israel; 2Ruth and Bruce Rappaport Faculty of Medicine, Technion–Israel Institute of Technology, Haifa, Israel; 3Department of Oncology, Rambam Health Care Campus, Haifa, Israel; 4Blood Bank and Apheresis Unit, Rambam Health Care Campus, Haifa, Israel; 5Department of Hematology and Bone Marrow Transplantation, Rambam Health Care Campus, Haifa, Israel; 6Department of Pediatric Hematology-Oncology, Meyer Children’s Hospital, Rambam Health Care Campus, Haifa, Israel; and 7Department of Pathology, Rambam Health Care Campus, Haifa, Israel PET/CT with 18F-FDG is an important noninvasive diagnostic tool for management of patients with lymphoma, and its use may sur- Lymphoma is a heterogeneous group of diseases rep- pass current guideline recommendations. The aim of the present resenting the fifth most common malignancy in the United study is to enlarge the growing body of evidence concerning 18F- FDG avidity of lymphoma to provide a basis for future guidelines. States, with an estimated 74,340 new cases predicted for Methods: The reports from 18F-FDG PET/CT studies performed 2008 (1). Subtypes of lymphoma differ in molecular char- in a single center for staging of 1,093 patients with newly diag- acteristics and biologic behavior. Based on clinical charac- nosed Hodgkin disease and non-Hodgkin lymphoma between teristics, this entity is divided into aggressive and indolent 2001 and 2008 were reviewed for the presence of 18F-FDG avid- types (2,3).
    [Show full text]
  • Landispiwowar.Kristin.Genetics of Select Lymphoproliferative
    3/21/16 Objecves Gene+cs of Select • Define tumor suppressor gene and oncogene Lymphoproliferave Neoplasms: • Correlate genotypes Pathophysiology, Prognos+caon, and Therapy with prognosis in lymphoproliferave neoplasms Kris+n Landis-Piwowar PhD, MLS(ASCP)CM • Recognize the targets of lymphoproliferave Clinical Laboratory Hematology, 3/E, McKenzie • Williams neoplasm therapies 2 Leukemia Lymphoma WHO 2008: Mature B-Cell Neoplasms • Chronic lymphocy+c leukemia/small lymphocy+c • Mantle cell lymphoma lymphoma • Diffuse large B-cell lymphoma (DLBCL), NOS • Primarily BM and • Primarily lymph • B-cell prolymphocyc leukemia • T-cell/his+ocyte rich large B-cell lymphoma • Primary DLBCL of the CNS • Splenic marginal zone lymphoma • Primary cutaneous DLBCL, leg type blood involvement node / solid ssue • Hairy cell leukemia • EBV-posi+ve DLBCL of the elderly • Splenic lymphoma/leukemia, unclassifiable • DLBCL associated with chronic inflammaon • Splenic diffuse red pulp small B-cell lymphoma • Lymphomatoid granulomatosis • Hairy cell leukemia variant involvement • Primary medias+nal (thymic) large B-cell lymphoma • Myeloid or • Lymphoplasmacy+c lymphoma • Intravascular large B-cell lymphoma • Waldenström macroglobulinemia • ALK-posi+ve large B-cell lymphoma lymphoid origin • Lymphoid origin • Heavy chain diseases • α Heavy chain disease • Plasmablas+c lymphoma • γ Heavy chain disease • Large B-cell lymphoma arising in HHV8-associated • May secondarily • May secondarily • μ Heavy chain disease mul+centric Castleman disease • Plasma cell myeloma • Primary
    [Show full text]
  • Romidepsin (Istodax) Reference Number: ERX.SPA
    Clinical Policy: Romidepsin (Istodax) Reference Number: ERX.SPA. 267 Effective Date: 12.01.18 Last Review Date: 11.20 Line of Business: Commercial, Medicaid Revision Log See Important Reminder at the end of this policy for important regulatory and legal information. Description Romidepsin (Istodax®) is a histone deacetylase inhibitor. FDA Approved Indication(s) Istodax is indicated for the treatment of: • Cutaneous T-cell lymphoma (CTCL) in adult patients who have received at least one prior systemic therapy; • Peripheral T-cell lymphoma (PTCL) in adult patients who have received at least one prior therapy. o This indication is approved under accelerated approval based on response rate. Continued approval for this indication may be contingent upon verification and description of clinical benefit in confirmatory trials. Policy/Criteria Provider must submit documentation (such as office chart notes, lab results or other clinical information) supporting that member has met all approval criteria. Health plan approved formularies should be reviewed for all coverage determinations. Requirements to use preferred alternative agents apply only when such requirements align with the health plan approved formulary. It is the policy of health plans affiliated with Envolve Pharmacy Solutions™ that Istodax and romidepsin injection solution are medically necessary when the following criteria are met: I. Initial Approval Criteria A. Cutaneous T-Cell Lymphoma (must meet all): 1. Diagnosis of CTCL (see Appendix D for examples of CTCL subtypes); 2. Prescribed by or in consultation with an oncologist or hematologist; 3. Age ≥ 18 years; 4. Request meets one of the following (a or b):* a. Dose does not exceed 14 mg/m2 for three days of a 28-day cycle; b.
    [Show full text]
  • Successful Treatment of CD30+Lymphomatoid Papulosis
    ooggeenneessii iinn ss && rrcc aa MM CC uu tt ff aa Journal ofJournal of oo gg ll ee ee aa aa nn nn nn nn ee ee rr rr ss ss uu uu ii ii Watabe et al., J Carcinog Mutagen 2014, 5:3 ss ss oo oo JJ JJ ISSN: 2157-2518 CarCarcinogenesiscinogenesis & Mutagenesis DOI: 10.4172/2157-2518.1000174 Case Report Open Access Successful Treatment of CD30+Lymphomatoid Papulosis using a 308-nm Excimer Light Akiko Watabe, Taku Fujimura*, Sadanori Furudate and Setsuya Aiba Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan *Corresponding author: Taku Fujimura, Department of Dermatology, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, 980-8574, Japan, Tel:+81 (22) 717-7271; Fax: +81 (22) 717-7361; E-mail: [email protected] Received date: Mar 11, 2014, Accepted date: May 03, 2014, Published date: May 07, 2014 Copyright: © 2013 Watabe A, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited Abstract We describe a 61-year-old Japanese patient with Lymphomatoid papulosis (LYP) successfully archived complete remission, using a 308-nm Excimer light. Interestingly, immunohistochemical staining revealed that CD30+ anaplastic tumor cells were surrounded by CD163+ macrophages and CCL18 producing cells, both of which were reported to correlate with the prognosis of CTCL. Our present study sheds light on the possible pathogenesis of LYP and the possibility of a 308-nm Excimer light phototherapy for LYP.
    [Show full text]
  • Message from the President
    DermNewsletter of the American OsteopathicLine College of Dermatology Spring 2016 Vol. 32, No. 1 Message from the President Greetings from Houston, Texas - again! As President of the AOCD, I welcome you to another edition of DermLine. As I think about our recent past and the dramatic changes afoot, I recall Suzanne Sirota Rozenberg, DO, FAOCD, Past President of AOCD, commenting in the Spring 2014 edition of DermLine about the coming AOA/ACGME merger. As I stated previously, “Today, at this moment, we are living in yesterday’s future.” Our future has arrived. Because the AOA, along with the Accreditation Council for Graduate Medical Education and the American Association of Colleges of Osteopathic Medicine, have agreed to a single accreditation system for graduate medical education programs in the United States, our graduates of osteopathic institutions, along with graduates of allopathic medical schools will complete their residency and/or fellowship education in ACGME-accredited programs and demonstrate achievement of common milestones and competencies side-by-side. I hope you realize the power in the previous statement. Trained together, osteopathic and allopathic graduates will no longer be divided. Perhaps the most exciting news to share is the AAD vote that just passed last month. This has been a battle we have been fighting for generations. The vote suffered defeat in 2004 and 2010. On both occasions, a majority of the membership voted in favor, but the required two-thirds majority required to approve a change to the bylaws fell short. I was told I would never see this vote go through in my lifetime.
    [Show full text]
  • Lymphomatoid Papulosis Development in Acute Lymphoblastic Leukemia
    Case Report J Med Cases. 2021;12(8):306-309 Lymphomatoid Papulosis Development in Acute Lymphoblastic Leukemia Kazumi Ouraa, Tomonobu Satoa, d , Akihiro Iguchib, Naohisa Toriumic, Takeo Sarashinac Abstract LyP is a rare disease with an incidence of 1.2 - 1.9 per million [1] and accounts for 16.0-47.0% of pediatric cutaneous lym- Lymphomatoid papulosis (LyP) is a chronic, recurrent benign skin phoproliferative disorders [2]. It has been reported that LyP disease characterized by histological features of a CD 30-positive cu- often disappears spontaneously and has a benign course [3, taneous T-cell lymphoproliferative disorder. It is rare, with an annual, 4]. However, a relationship between LyP and certain hemato- worldwide incidence of 1.2 - 1.9 per million, and accounts for 16-47% logical malignancies has also been reported [5-7]. Herein, we of pediatric cutaneous lymphoproliferative disorders. It often occurs describe a case in which tumors appeared in the left scrotum on the extremities or the trunk and rarely affects the face or genitals. and under the left lip during maintenance therapy for pre- Its onset may be triggered by irradiation therapy, immunomodulating cursor B-cell acute lymphoblastic leukemia (BCP-ALL). We agents, infection or atopic dermatitis. It has a benign course but is as- needed to distinguish the tumor from extramedullary recur- sociated with certain hematological malignancies. Mycosis fungoides rence of ALL or de novo other cutaneous lymphoma; how- and primary cutaneous anaplastic large cell lymphoma are the most ever, the histological findings of a tumor biopsy resulted in a commonly associated hematological malignancies.
    [Show full text]
  • Evaluation of Melanocyte Loss in Mycosis Fungoides Using SOX10 Immunohistochemistry
    Article Evaluation of Melanocyte Loss in Mycosis Fungoides Using SOX10 Immunohistochemistry Cynthia Reyes Barron and Bruce R. Smoller * Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, NY 14642, USA; [email protected] * Correspondence: [email protected] Abstract: Mycosis fungoides (MF) is a subtype of primary cutaneous T-cell lymphoma (CTCL) with an indolent course that rarely progresses. Histologically, the lesions display a superficial lymphocytic infiltrate with epidermotropism of neoplastic T-cells. Hypopigmented MF is a rare variant that presents with hypopigmented lesions and is more likely to affect young patients. The etiology of the hypopigmentation is unclear. The aim of this study was to assess melanocyte loss in MF through immunohistochemistry (IHC) with SOX10. Twenty cases were evaluated, including seven of the hypopigmented subtype. The neoplastic epidermotropic infiltrate consisted predominantly of CD4+ T-cells in 65% of cases; CD8+ T-cells were present in moderate to abundant numbers in most cases. SOX10 IHC showed a decrease or focal complete loss of melanocytes in 50% of the cases. The predominant neoplastic cell type (CD4+/CD8+), age, race, gender, histologic features, and reported clinical pigmentation of the lesions were not predictive of melanocyte loss. A significant loss of melanocytes was observed in 43% of hypopigmented cases and 54% of conventional cases. Additional studies will increase our understanding of the relationship between observed pigmentation and the loss of melanocytes in MF. Citation: Barron, C.R.; Smoller, B.R. Evaluation of Melanocyte Loss in Keywords: mycosis fungoides; hypopigmented mycosis fungoides; SOX10; cutaneous T-cell lymphoma Mycosis Fungoides Using SOX10 Immunohistochemistry.
    [Show full text]
  • Primary Cutaneous Lymphoma: ESMO Clinical Recommendations for Diagnosis, Treatment and Follow-Up R
    Annals of Oncology 19 (Supplement 2): ii72–ii76, 2008 clinical recommendations doi:10.1093/annonc/mdn095 Primary cutaneous lymphoma: ESMO Clinical Recommendations for diagnosis, treatment and follow-up R. Dummer1 & M. Dreyling2 On behalf of the ESMO Guidelines Working Group* 1Department of Dermatology, University Hospital, Zu¨rich, Switzerland; 2Department of Medicine III, University Hospital Grosshadern, Munich, Germany introduction the quality of life due to their impact on skin appearance and annoying symptoms such as pruritus. In some cases they can Cutaneous lymphomas (CL) are the second most common already be disfiguring in early disease stages. In advanced stages, extranodal non-Hodgkin’s lymphomas. Their incidence is local skin problems are accompanied by systemic immune estimated at 1/100 000 yearly. Primary CL develop by definition suppression which results in an increased risk of infections and in the skin and remain confined to the skin for a long period of secondary malignancies. Some of the late-stage problems in time, while secondary CL reflect cutaneous spread from MF/SS patients might have been aggravated by earlier disseminated primary nodal or extranodal lymphomas. Primary therapeutic interventions, e.g. radiotherapy or phototherapy CL include a wide spectrum of clinically and histologically may contribute to mutations that increase the proliferative heterogeneous lymphoproliferative neoplasms: 75% of CL are and invasive capacity of the tumor cell populations. Cytotoxic cutaneous T-cell lymphomas (CTCL); 25% cutaneous B-cell drugs favor infectious complications. Most patients with lymphomas (CBCL); and a few percent other uncommon advanced disease may die due to secondary problems such as forms. CL and nodal or extracutaneous lymphomas with the infections.
    [Show full text]