Spontaneous Regression of Primary Diffuse Large B-Cell Lymphoma, Leg Type.

Oct 2012

[Article in English, Spanish]

Alcántara-González J, González-García C, Fernández-Guarino M, Jaén-Olasolo P.

Source

Servicio de Dermatología, Hospital Universitario Ramón y Cajal, Madrid, España. Electronic address: jalcantarag@hotmail.es.

Abstract

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL LT) accounts for approximately 20% of all primary cutaneous B-cell lymphomas and tends to present as infiltrated nodules, tumors, and plaques on the legs in the elderly. Unlike other primary cutaneous large B-cell lymphomas, it has a poor prognosis and tends to require treatment with systemic chemotherapy. We present the case of an 82-year-old patient with a 1-year history of nodules and plaques on her right leg. Biopsy led to a diagnosis of PCLBCL LT and the lesions resolved without treatment within 1 month of the first visit. This is an atypical course of PCLBCL LT and we believe that it is the first such case to be reported in the literature.

ACTAS Dermo Sifiliograficas

Cutaneous γδ T-cell Lymphomas: A Spectrum of Presentations With Overlap With Other Cytotoxic Lymphomas.

Nov 2012

Guitart J, Weisenburger DD, Subtil A, Kim E, Wood G, Duvic M, Olsen E, Junkins-Hopkins J, Rosen S, Sundram U, Ivan D,Selim MA, Pincus L, Deonizio JM, Kwasny M, Kim YH.

Source

*Departments of Dermatology and Pathology ††Department of Hematology and Oncology, Northwestern University Feinberg Medical School, Chicago, IL †Department of Pathology, University of Nebraska Medical Center, NE ‡Department of Dermatology, Yale University, New Haven, CT §Department of Dermatology, University of Pennsylvania, Philadelphia, PA ∥Department of Dermatology, University of Wisconsin, WI ¶Department of Dermatology, MD Anderson, Houston, TX #Departments of Dermatology and Pathology, Duke University, Durham, NC **Department of Dermatology, Johns Hopkins University, Baltimore, MA ‡‡Department of Pathology, Stanford University, Stanford §§Department of Dermatology, University of California in San Francisco, San Francisco, CA ¶¶Department of Preventive Medicine, Northwestern University Feinberg Medical School, Chicago, IL.

Abstract

We reviewed our multicenter experience with gamma-delta (γδ) T-cell lymphomas first presenting in the skin. Fifty-three subjects with a median age of 61 years (range, 25 to 91 y) were diagnosed with this disorder. The median duration of the skin lesions at presentation was 1.25 years (range, 1 mo to 20 y). The most common presentation was deep plaques (38 cases) often resembling a panniculitis, followed by patches resembling psoriasis or mycosis fungoides (10 cases). These lesions tended to ulcerate overtime (27 cases). Single lesions or localized areas of involvement resembling cellulitis or pyoderma were reported in 8 cases. The most common anatomic site of involvement was the legs (40 cases), followed by the torso (30 cases) and arms (28 cases). Constitutional symptoms were reported in 54% (25/46) of the patients, including some with limited skin involvement. Significant comorbidities included autoimmunity (12 cases), other lymphoproliferative disorders (5 cases), internal carcinomas (4 cases), and viral hepatitis (2 cases). Lymphadenopathy (3/42 cases) and bone marrow involvement (5/28 cases) were uncommon, but serum lactose dehydrogenase (LDH) was elevated in 55% (22/39) of the patients. Abnormal positron emission tomography and/or computed tomography scans in 20/37 subjects mostly highlighted soft tissue or lymph nodes. Disease progression was associated with extensive ulcerated lesions resulting in 27 deaths including complications of hemophagocytic syndrome (4) and cerebral nervous system involvement (3). Median survival time from diagnosis was 31 months. Skin biopsies varied from a pagetoid pattern to purely dermal or panniculitic infiltrates composed of intermediate-sized lymphocytes with tissue evidence of cytotoxicity. The most common immunophenotype was CD3/CD4/CD5/CD8/BF1/γ-M1/TIA-1/granzyme-B/CD45RA/CD7, and 4 cases were Epstein-Barr virus positive. This is the largest study to date of cutaneous γδ T-cell lymphomas and demonstrates a variety of clinical and pathologic presentations with a predictable poor outcome.

Lippincott Williams & Wilkins

Topical Chemotherapy in Cutaneous T-cell Lymphoma: Positive Results of a Randomized, Controlled, Multicenter Trial Testing the Efficacy and Safety of a Novel Mechlorethamine, 0.02%, Gel in Mycosis Fungoides.

Lessin SR, Duvic M, Guitart J, Pandya AG, Strober BE, Olsen EA, Hull CM, Knobler EH, Rook AH, Kim EJ, Naylor MF,Adelson DM, Kimball AB, Wood GS, Sundram U, Wu H, Kim YH.

Abstract

OBJECTIVE To evaluate the efficacy and safety of a novel mechlorethamine hydrochloride, 0.02%, gel in mycosis fungoides. 

DESIGN Randomized, controlled, observer-blinded, multicenter trial comparing mechlorethamine, 0.02%, gel with mechlorethamine, 0.02%, compounded ointment. Mechlorethamine was applied once daily for up to 12 months. Tumor response and adverse events were assessed every month between months 1 and 6 and every 2 months between months 7 and 12. Serum drug levels were evaluated in a subset of patients. 

SETTING Academic medical or cancer centers. 

PATIENTS In total, 260 patients with stage IA to IIA mycosis fungoides who had not used topical mechlorethamine within 2 years and were naive to prior use of topical carmustine therapy. 

MAIN OUTCOME MEASURES Response rates of all the patients based on a primary clinical end point (Composite Assessment of Index Lesion Severity) and secondary clinical end points (Modified Severity-Weighted Assessment Tool and time-to-response analyses). 

RESULTS Response rates for mechlorethamine gel vs ointment were 58.5% vs 47.7% by the Composite Assessment of Index Lesion Severity and 46.9% vs 46.2% by the Modified Severity-Weighted Assessment Tool. By the Composite Assessment of Index Lesion Severity, the ratio of gel response rate to ointment response rate was 1.23 (95% CI, 0.97-1.55), which met the prespecified criterion for noninferiority. Time-to-response analyses demonstrated superiority of mechlorethamine gel to ointment (P < .01). No drug-related serious adverse events were seen. Approximately 20.3% of enrolled patients in the gel treatment arm and 17.3% of enrolled patients in the ointment treatment arm withdrew because of drug-related skin irritation. No systemic absorption of the study medication was detected. 

CONCLUSION The use of a novel mechlorethamine, 0.02%, gel in the treatment of patients with mycosis fungoides is effective and safe. TRIAL REGISTRATION clinicaltrials.gov Identifier: NCT00168064.

Archives Dermatology

Romidepsin: A Histone Deacetylase Inhibitor for Refractory Cutaneous T-Cell Lymphoma

Romidepsin: A Histone Deacetylase Inhibitor for Refractory Cutaneous T-Cell Lymphoma (October).

Kim M, Thompson LA, Wenger SD, O'Bryant CL.

Source

< Skaggs School of Pharmacy and Pharmaceutical Sciences, University of Colorado, Aurora.

Abstract

OBJECTIVE:

To evaluate the efficacy and safety of romidepsin in refractory cutaneous T-cell lymphoma (CTCL).

DATA SOURCES:

An English-language literature search of PubMed and MEDLINE (Nov 2011-April 2012) was performed using the terms romidepsin, CTCL, and depsipeptide (FK228). The National Comprehensive Cancer Network guidelines, American Society of Clinical Oncology abstracts, American Society of Hematology abstracts, clinical trial registry, and prescribing information from the manufacturer were reviewed for additional information.

STUDY SELECTION AND DATA EXTRACTION:

Phase 1 and 2 trials evaluating the efficacy and safety of romidepsin were reviewed with a specific focus on its use in cutaneous T-cell lymphoma. All peer-reviewed articles with clinically relevant information were evaluated for inclusion.

DATA SYNTHESIS:

In advanced stage CTCL, single or combination chemotherapy regimen responses are variable and lack durability. Romidepsin is a histone deacetylase inhibitor approved for refractory cutaneous T-cell lymphoma. Romidepsin has shown an improvement in duration of response and pruritus over traditional therapy. In 2 independent Phase 2 trials, romidepsin showed an overall response rate of 34% and durable response of 13-15 months in patients with refractory CTCL. The most frequent toxicities of romidepsin include nausea, vomiting, fatigue, or myelosuppression. Clinically insignificant QT interval changes have been observed but did not correlate with a decrease in left ventricular ejection fraction, or elevated laboratory markers of myocardial damage.

CONCLUSIONS:

Romidepsin is an effective, durable, and well-tolerated single-agent therapy in patients with refractory CTCL and should be considered for formulary addition in this population.

Annals of Pharmacotherapy

Long-term follow-up and survival of cutaneous T-cell lymphoma patients treated with extracorporeal photopheresis.

Oct 2012

Knobler R, Duvic M, Querfeld C, Straus D, Horwitz S, Zain J, Foss F, Kuzel T, Campbell K, Geskin L.

Source

Department of Dermatology, Medical University of Vienna, Vienna, Austria.

Abstract

PURPOSE:

Extracorporeal photopheresis (ECP) is effective for treating cutaneous T-cell lymphoma. In 1987, a pivotal trial showed 81% overall response rate (ORR) using outdated criteria. No long-term follow-up was available for assessing survival. This study applies modern criteria to the 1987 trial to assess the impact of ECP on skin responses and also updates overall survival of the cohort.

METHODS:

Generalized erythroderma (GE, stage T4, n = 31) or extensive patch-plaque (EPP, stage T2, n = 8) patients received ECP (mean 3.9 years' duration). Patients achieving ≥ 50% partial skin response, ≥ 90% near-complete skin response, treatments required, and duration of response (DOR) were determined. Overall survival (OS) from diagnosis and first ECP treatment was determined for all patients and the GE cohort.

RESULTS:

Patients showed 74% skin ORR using modern criteria; 33% of patients achieved ≥ 50% partial skin response (after median 7.1 months, mean 23 ECP treatments); 41% achieved ≥ 90% improvement (after median 19.6 months, mean 40 ECP treatments). Mean DOR was 14 months for ≥ 50% improvement and 8.9 months for ≥ 90% improvement. Response rates were comparable for GE and EPP cohorts. Median OS was 9.2 years from diagnosis and 6.6 years from ECP initiation (71.6 months follow-up).

CONCLUSION:

Analysis of long-term follow-up confirmed durable responses and prolonged survival of patients treated with ECP.

Wiley OnLine

Prevalence and Severity of Pruritus and Quality of Life in Patients With Cutaneous T-Cell Lymphoma.

Aug 2012

Wright A, Wijeratne A, Hung T, Gao W, Whittaker S, Morris S, Scarisbrick J, Beynon T.

Source

St. Thomas' Hospital, Kings Healthcare Partners, London, United Kingdom.

Abstract

CONTEXT:

Cutaneous T-cell lymphoma (CTCL), although rare, is associated with a significant symptom burden. Pruritus appears to be one of the most prominent and disturbing symptoms.

OBJECTIVES:

To describe the prevalence and severity of pruritus and quality of life (QOL) in patients with CTCL.

METHODS:

Patients with CTCL able to complete two questionnaires were invited to complete a visual analogue scale for itch (VAS(itch)) and the Skindex-29. Prevalence of pruritus, mean score, and SD were estimated for the VAS(itch) and Skindex-29, and the Pearson's correlation coefficient was calculated to evaluate the relationship between severity of pruritus and QOL.

RESULTS:

One hundred patients were recruited (mean [SD] age 57.9 [12.9] years, range 30-86 years). Eighty-eight percent reported pruritus in the preceding four weeks, 46% indicating that it was often or always a problem. The mean (SD) of VAS(itch) (n=92) was 3.2 (3.2), range zero to 10. The mean (SD) total Skindex-29 score was 43.3 (27.7). More advanced disease stage was associated with poorer QOL. The Skindex-29 correlated strongly with the VAS(itch) (Pearson's correlation coefficient=0.72, P<0 .001=".001" b="b">

CONCLUSION:

All aspects of QOL are affected in CTCL. Pruritus is a common and troublesome symptom. A more advanced disease stage and more severe pruritus symptoms were associated with poorer QOL in this study.

 Elsevier Inc.

Radiotherapy in the Management of Localized Primary Cutaneous B-Cell Lymphoma.

August 2012

Itai Pashtan, Peter M. Mauch, Yu-Hui Chen, David M. Dorfman, Barbara Silver, Andrea K. Ng

Corresponding author: Itai Pashtan, MD, Department of Radiation Oncology, 

Brigham and Women's Hospital, 75 Francis Street, ASB1-L2, Boston, MA 02115

, USA. Tel: +1-617-732-6231; Fax: +1-617-975-0932; E-mail: ipashtan@partners.org

Keywords

Primary cutaneous B-cell lymphoma, prognosis, radiation, dose, local control

Abstract

The optimal therapy and radiation dose for patients with localized primary cutaneous B-cell lymphoma (PCBCL) are unknown. We retrospectively identified 23 patients with localized (T1-T2) PCBCL treated with definitive radiation to doses ranging from 30–44 Gy (median, 36 Gy). With median follow-up of 4.8 years, the 5-year overall survival rate was 100%, the relapse-free survival rate was 71% (95% CI, 46-86%) and there were no local recurrences, suggesting that radiotherapy to a dose of 30 Gy may be sufficient for cure.

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