Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma: Transformation from indolent to aggressive phase in association with CCR7-positive conversion.

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma: Transformation from indolent to aggressive phase in association with CCR7-positive conversion.

Oct 2012

Kasuya A, Hirakawa S, Tokura Y.

Source

Hamamatsu University School of Medicine Hamamatsu, Shizuoka, Japan.

Abstract

We report a case of primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma that transformed from the indolent phase to the aggressive phase. In the aggressive phase, the patient developed numerous subcutaneous lesions on the trunk, limbs, and face. The involvement of muscle, right testis, and pharynx were also found. We immunohistochemically analyzed the expression of chemokine receptors, before and after the transformation of aggressive epidermotropic CD8+ T-cell lymphoma. The transformation was accompanied by positive conversion of CCR7. CCR7 is crucial in lymphatic cell migration and chemotaxis to lymph nodes. Therefore, its expression is likely associated with the disseminated behavior of T-cell lymphoma.

PubMed

Mycosis fungoides palmaris et plantaris.

Nov 2012

[Article in German]

Pfaff S, Megahed M.

Source

Klinik für Dermatologie und Allergologie, RWTH Aachen University, Pauwelsstr. 30, 52074, Aachen, Deutschland, spfaff@ukaachen.de.

Abstract

Mycosis fungoides (MF) is a low-grade cutaneous T-cell lymphoma characterized by skin-homing CD4- positive helper T cells. Mycosis fungoides palmaris et plantaris is an uncommon variant primarily involving the palms and soles. An 80-year old man presented with hyperkeratotic erythematous palmoplantar changes. Clinical and histopathologic criteria led to the diagnosis mycosis fungoides palmaris et plantaris. Tumor staging using sonography of the abdomen and lymph nodes, chest x-ray and blood examination is recommended, because extracutaneous manifestations may be present.

Springerlink

PML-IRIS IN A PATIENT TREATED WITH BRENTUXIMAB.

Oct 2012

von Geldern G, Pardo CA, Calabresi PA, Newsome SD.

Source

From the Departments of Neurology (G.v.G., C.A.P., P.A.C., S.D.N.) and Pathology (C.A.P.), The Johns Hopkins Hospital, Baltimore, MD.

Abstract

A 38-year-old woman was diagnosed with cutaneous anaplastic T-cell lymphoma that proved refractory to methotrexate, bexarotene, denileukin diftitox, interferon γ-1b, interferon α-2b, vorinostat, and pralatrexate. She was therefore started on the newly approved monoclonal anti-CD30 antibody brentuximab vedotin. Treatment with brentuximab 1.8 mg/kg IV every 3 weeks quickly led to disappearance of her cutaneous tumors. The day after her second brentuximab infusion she developed word-finding difficulties and unsteady gait. Due to further neurologic deterioration, she was admitted to an outside hospital. Brain MRI revealed multifocal enhancing white matter lesions throughout bilateral cerebral hemispheres and posterior fossa (figure, A-C). Brain biopsy was performed 15 days after her last brentuximab dose to rule out metastases and she was diagnosed with progressive multifocal leukoencephalopathy (PML) (figure, J). The patient was discharged home with hospice care. Upon discharge, she was started on prednisone 50 mg daily to help treat her eczema. Her family brought her to our clinic for a second opinion.

Neurology

Painful and rapidly growing exophytic lesion of the vestibular upper lip

Nov  2012

[Article in German]

Trempler C, Maurer P, Wysluch A.

Source

Abteilung für Mund-, Kiefer- und Gesichtschirurgie, Klinikum Bremerhaven-Reinkenheide gGmbh, Postbrookstr. 103, 27574, Bremerhaven, Deutschland, christina.trempler@klinkum-bremerhaven.de.

Abstract

A 49-year-old healthy woman presented with a painful exophytic-growing mucous lesion on her upper lip that had been primarily noted for 2 weeks. The biopsy showed histological changes of a dense infiltration of lymphoid cell elements. The immunohistological examination presented the diagnosis of primary cutaneous CD30-positive large cell T-cell lymphoma. In the diagnosis of oral lesions cutaneous CD30-positive large cell T-cell lymphoma constitutes a rare but important differential diagnosis.

Springerlink

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