Publications by authors named "Neha Jariwala"

16 Publications

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Low-Dose Total Skin Electron Beam Therapy as Part of a Multimodality Regimen for Treatment of Sézary Syndrome: Clinical, Immunologic, and Molecular Analysis.

JAMA Dermatol 2021 01;157(1):90-95

Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia.

Importance: Sézary syndrome (SS) is an advanced form of cutaneous T-cell lymphoma with few long-term remissions observed.

Objective: To profile 3 patients with SS who have experienced long-term remission following the addition of low-dose total skin electron beam therapy (TSEBT) to systemic regimens of extracorporeal photopheresis, bexarotene, and interferon-γ.

Design, Setting, And Participants: This is a retrospective case series with additional investigations of patient-donated samples to assess therapeutic response. The study was conducted at the University of Pennsylvania Cutaneous Lymphoma Clinic and follows 3 patients with stage IVA1 CD4+ SS who presented to the clinic between November 1, 2009, and November 1, 2017, and who had a history of SS that was refractory to multimodality systemic therapy prior to receiving low-dose TSEBT.

Interventions: Patients were treated in a multimodality fashion with combined extracorporeal photopheresis, bexarotene, interferon-γ, and low-dose TSEBT.

Main Outcomes And Measures: To characterize treatment responses in these patients, the extent of skin disease was measured with the modified severity weighted assessment tool. Blood disease was measured with flow cytometric assessments of Sézary cell count, CD4:CD8 ratio, and high throughput sequencing of the T-cell receptors. To assess for restoration of immune function, we measured markers of immune exhaustion, including PD-1 (programmed cell death 1), TIGIT (T-cell immunoreceptor with immunoglobulin and ITIM domains), CTLA4 (cytotoxic T-lymphocyte-associated protein 4), TOX (thymocyte selection-associated high mobility group box protein), and Foxp3 (forkhead box P3) on circulating CD4 and CD8 T cells, along with production capacity of interferon-γ by lymphocytes following activation stimuli.

Results: Following administration of low-dose TSEBT and maintenance of the other therapies, remissions ranged from 24 to 30 months, with complete responses in 2 patients ongoing. Markers of immune exhaustion including PD-1, TIGIT, CTLA4, TOX, and Foxp3 were significantly reduced from baseline following TSEBT, along with enhanced production capacity of interferon-γ by lymphocytes following activation stimuli. High throughput sequencing demonstrated near-complete eradication of the circulating clone among 2 of 3 patients with stable levels in 1.

Conclusions And Relevance: We describe 3 patients who achieved long-term clinical and molecular remissions following low-dose TSEBT as part of a multimodality regimen for treatment of SS. As long-term remissions in SS are uncommon, this approach demonstrates promise, and clinical trials should be considered.
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http://dx.doi.org/10.1001/jamadermatol.2020.3958DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7593882PMC
January 2021

Cutaneous T-cell lymphoma and concomitant atopic dermatitis responding to dupilumab.

Cutis 2020 Sep;106(3):131-132

Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, USA.

Cutaneous T-cell lymphoma (CTCL) represents a diagnostic challenge because of its large symptomatic overlap with other common skin conditions such as atopic dermatitis (AD) and psoriasis. Dupilumab has offered promising results in AD treatment; however, concerns exist that its use may exacerbate undiagnosed CTCL. We present a patient with CTCL and concomitant AD who experienced improvement in both CTCL blood involvement and AD following the addition of dupilumab therapy.
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http://dx.doi.org/10.12788/cutis.0066DOI Listing
September 2020

Urethral involvement is associated with higher mortality and local recurrence in vulvar melanoma: a single institutional experience.

Hum Pathol 2020 10 20;104:1-8. Epub 2020 Jul 20.

Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, 19104, USA. Electronic address:

Vulvar malignant melanoma (VMM), although uncommon, comprises 5-10% of all vulvar malignancies. Local control is notoriously poor in VMM with recurrence rates of 30-50% compared with approximately 3% in cutaneous melanomas. We studied clinicopathologic features of 37 women with VMM, after reviewing three decades of clinical follow-up data in our institutional databases. Most patients were Caucasian (n = 35) with an average age at diagnosis of 60.6 years (range 23-83). The most common subtype was mucosal lentiginous melanoma (n = 25). We compared Kaplan-Meier survival curves of 31 patients defined by clinical and microscopic attributes using exact log-rank tests. Younger patients at diagnosis (23-64 years), those with thin melanomas (≤1 mm), and those with Clark's level II or III tumors had better 5-year survival rates than older patients (65-83 years) and those with thick melanomas (>1 mm) and those with Clark's level IV or V (P ≤ 0.05), respectively, by exact log-rank test. Local recurrence of melanoma occurred in 15 patients. Nine patients (24%) had eventual urethral involvement by malignant melanoma, and this feature was associated with significantly shorter survival (P = 0.036). Patients with urethral involvement had shorter median time to death and worse 5-year survival rates. Given that spread to the urethra is common in VMM and urethral recurrence is also associated with mortality, pathology excision specimens should be carefully reviewed with attention to urethral involvement as a potentially important prognostic factor.
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http://dx.doi.org/10.1016/j.humpath.2020.07.017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7669565PMC
October 2020

Pilot Study of a Novel Therapeutic Approach for Refractory Advanced Stage Folliculotropic Mycosis Fungoides.

Acta Derm Venereol 2020 06 18;100(13):adv00187. Epub 2020 Jun 18.

Department of Dermatology, University of Pennsylvania, 19104 Philadelphia, USA.

Folliculotropic mycosis fungoides is a variant of cutaneous T-cell lymphoma characterized as having a folliculocentric infiltrate of malignant T cells along with a worse prognosis in comparison to the epidermotropic variants. Patients with advanced forms of folliculotropic mycosis fungoides are often poorly responsive to both skin-directed as well as to systemic therapies. We report here a high response rate using a novel therapeutic regimen combining interferon gamma, isotretinoin in low dose and topical carmustine, and in some cases concomitant skin-directed therapies, among 6 consecutive patients with refractory folliculotropic mycosis fungoides with stages IB through IIIB who had previously failed both topical and systemic therapies. The potential mechanisms of this multimodality approach are discussed.
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http://dx.doi.org/10.2340/00015555-3443DOI Listing
June 2020

Scrotal ulceration: a complication of hyperthermic intraperitoneal chemotherapy and subsequent treatment with dimethyl sulfoxide.

Cutis 2019 Jul;104(1):E1-E3

Department of Dermatology, Hofstra Northwell School of Medicine, New York, USA.

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July 2019

Clinical registries: Should physicians accept payments for enrolling patients?

J Am Acad Dermatol 2017 07;77(1):183-185

Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address:

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http://dx.doi.org/10.1016/j.jaad.2017.01.021DOI Listing
July 2017

Skin Disease as Art.

JAMA Dermatol 2017 05;153(5):448

Department of Dermatology, University of Pennsylvania, Philadelphia.

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http://dx.doi.org/10.1001/jamadermatol.2017.0967DOI Listing
May 2017

Establishment of a dermatology global health outreach and residency partnership program in Guatemala.

J Am Acad Dermatol 2017 05;76(5):993-994.e1

Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1016/j.jaad.2016.11.047DOI Listing
May 2017

Eyebrow and Eyelash Loss.

JAMA 2017 Jan;317(1):81-82

Drexel College of Medicine, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1001/jama.2016.17688DOI Listing
January 2017

Topical Carmustine as Monotherapy or as Multimodality Therapy for Folliculotropic Mycosis Fungoides.

Acta Derm Venereol 2017 03;97(3):373-374

Department of Dermatology, Johns Hopkins University, 601 North Caroline Street, 8 Floor, Baltimore, Maryland, 21287, USA.

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http://dx.doi.org/10.2340/00015555-2551DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5363058PMC
March 2017

TIGIT and Helios Are Highly Expressed on CD4 T Cells in Sézary Syndrome Patients.

J Invest Dermatol 2017 01 1;137(1):257-260. Epub 2016 Sep 1.

Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

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http://dx.doi.org/10.1016/j.jid.2016.08.016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5358509PMC
January 2017

CD164 identifies CD4 T cells highly expressing genes associated with malignancy in Sézary syndrome: the Sézary signature genes, FCRL3, Tox, and miR-214.

Arch Dermatol Res 2017 Jan 20;309(1):11-19. Epub 2016 Oct 20.

Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, 421 Curie Blvd, 1049 BRB, Philadelphia, PA, 19104, USA.

Sézary syndrome (SS), a leukemic variant of cutaneous T-cell lymphoma (CTCL), is associated with a significantly shorter life expectancy compared to skin-restricted mycosis fungoides. Early diagnosis of SS is, therefore, key to achieving enhanced therapeutic responses. However, the lack of a biomarker(s) highly specific for malignant CD4 T cells in SS patients has been a serious obstacle in making an early diagnosis. We recently demonstrated the high expression of CD164 on CD4 T cells from Sézary syndrome patients with a wide range of circulating tumor burdens. To further characterize CD164 as a potential biomarker for malignant CD4 T cells, CD164 and CD164CD4 T cells isolated from patients with high-circulating tumor burden, B2 stage, and medium/low tumor burden, B1-B0 stage, were assessed for the expression of genes reported to differentiate SS from normal controls, and associated with malignancy and poor prognosis. The expression of Sézary signature genes: T plastin, GATA-3, along with FCRL3, Tox, and miR-214, was significantly higher, whereas STAT-4 was lower, in CD164 compared with CD164CD4 T cells. While Tox was highly expressed in both B2 and B1-B0 patients, the expression of Sézary signature genes, FCRL3, and miR-214 was associated predominantly with advanced B2 disease. High expression of CD164 mRNA and protein was also detected in skin from CTCL patients. CD164 was co-expressed with KIR3DL2 on circulating CD4 T cells from high tumor burden SS patients, further providing strong support for CD164 as a disease relevant surface biomarker.
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http://dx.doi.org/10.1007/s00403-016-1698-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5357118PMC
January 2017

The Keloid Scars of Slavery.

JAMA Dermatol 2016 10;152(10):1121

Department of Dermatology, University of Pennsylvania, Philadelphia.

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http://dx.doi.org/10.1001/jamadermatol.2015.6263DOI Listing
October 2016

Spiradenocylindroma: an uncommon morphologic entity.

Int J Dermatol 2016 Jul 5;55(7):801-3. Epub 2016 Mar 5.

Department of Dermatology, Drexel University College of Medicine, Philadelphia, PA, USA.

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http://dx.doi.org/10.1111/ijd.13233DOI Listing
July 2016

Foreign body injuries in children: Are the younger siblings doomed?

Int J Pediatr Adolesc Med 2016 Mar 27;3(1):7-11. Epub 2016 Jan 27.

Dept. of Emergency Medicine, St Christopher's Hospital for Children, Drexel University College of Medicine, Philadelphia, PA, United States.

Background And Objectives: Foreign body injury (FBI) is a considerable public health issue for children. Although the relationships of FBI with age, gender, and objects of injury have been studied, the extent to which other demographic factors influence FBI is unclear. We hypothesized that the risk for FBI increases with the number of children in the household.

Design And Settings: This was a retrospective analysis of 223 patients aged 2-10 years who presented to the emergency department of an inner-city pediatric hospital and who were found to have FBI.

Patients And Methods: The guardians were contacted via phone to examine the associations of FBI with income, parental educational level, number of children in the household, and birth order while controlling with a matched population of 250 patients. Statistical analyses using frequencies and univariate and multivariate analyses were performed.

Results: For each increase in the number of children, the risk of FBI increased 1.44-fold (OR = 1.442). With each increase in the number of caregivers, the risk of a FBI decreased 33% (OR = 0.673). With each increase in income category, the risk of a FBI decreased 59% (OR = 0.413).

Conclusion: The results suggest that an increase in the number of children in a household is associated with a greater risk of FBI.
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http://dx.doi.org/10.1016/j.ijpam.2015.12.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6372414PMC
March 2016