primary cutaneous b-cell lymphoma: a case report
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P6645Plasmablastic lymphoma of the oral cavity: A case report
Janeth Cristina Cardona-Alzate, MD, Complejo Hospitalario de Toledo, Toledo,Spain; Blas Alexis Gomez-Dorado, MD, Complejo Hospitalario de Toledo, Toledo,Spain; Cristina Schoendorff-Ortega, MD, Complejo Hospitalario de Toledo,Toledo, Spain; Fabienne Robuschi-Lestouquet, MD, Complejo Hospitalario deToledo, Toledo, Spain; Silvia Honorato-Guerra, MD, Complejo Hospitalario deToledo, Toledo, Spain
Background: Plasmablastic lymphoma is a rare form of non-Hodgkin lymphoma thatmost frequently affects the oral cavity of HIV patients. It is recognized by WHO’sclassification of lymphoproliferative disorders as a subtype of diffuse large B-celllymphoma, HIV related and AIDS defining illness.
Case report: A 56-year-old man presented with a 1-month history of rapidly growingmass in his tongue. He denied fever, chills, night sweats or weight loss. His medicalhistory was significant for VIH (stage B3). Physical examination revealed a 33 4 cmpink-withe, ulcerated, exophytic lesion involving the left half of the dorsal surface ofthe tongue. Biopsy sample of the lesion showed atypical neoplastic infiltrates ofplasmocytoid cells with abundant cytoplasm and prominent nucleoli.Inmunostaining was negative for CD2O, CD3, HHV8, BCL2, BCL6 and positive forCD38, CD138, EBER, MUM1. These finding were consistent with a diagnosis ofplasmablastic lymphoma. A bone marrow aspirate and biopsy were done, and theseconfirmed infiltration of bone marrow (stage IV-A). CT imaging of the brain, neck,chest, abdomen and pelvis didn’t show lymph node involvement. The patient wastransferred to the oncology service and treated with EPOCH (etoposide, predni-sone, vincristine, cyclophosphamide and doxorubicin) in association with HAART(highly active antiretroviral therapy). The disease progress and the patient wasswitched to bortezomib with poor clinical response.
Discussion: Plasmablastic lymphoma accounts for 2.6% of all HIV-related non-Hodgkin lymphomas, it was first described by Delecluse et al as highly malignant B-cell lymphomawith predilection for the oral cavity in advanced immunosuppressedpatients, nevertheless extraoral localizations and immunocompetent patientsaffected had been reported. It has been associated with EpsteineBarr virus in hispathogenesis, the association with HHV-8 is not clear. The histology is characterizedby immunoblastic morphology and plasma cell phenotype (negativity for typical B-cell antigens CD20 and positivity for plasma cell markers such as CD38, CD138 andVS38c). The differential diagnosis includes primarily infectious and others malignantprocesses like squamous cell tumor, metastatic tumor and Kaposi sarcoma. Theprognosis is very poor despite chemotherapy and HAART. Dermatologists should beaware of this oral pathology in the diagnostic evaluation of HIV patients with rapidlygrowing oral mass.
AB148
cial support: None identified.
CommerP6785Primary cutaneous anaplastic large cell lymphoma: A diagnostic challenge
Andreia Almeida, MD, Instituto de Dermatologia Professor Rubem David Azulay,Rio de Janeiro, Brazil; Flavia Bonini, MD, Instituto de Dermatologia ProfessorRubem David Azulay, Rio de Janeiro, Brazil; Flavia Brito, MD, Instituto deDermatologia professor Rubem David Azulay, Rio de Janeiro, Brazil; MercedesPockstaller, MD, Instituto de Dermatologia professor Rubem David Azulay, Rio deJaneiro, Brazil; Patricia Rezende, MD, Instituto de Dermatologia Professor RubemDavid Azulay, Rio de Janeiro, Brazil; Tullia Cuzzi, MD, Instituto de DermatologiaProfessor Rubem David Azulay, Rio de Janeiro, Brazil
Primary cutaneous CD30 lymphoproliferative disorders (LPDs) are the second mostcommon group of cutaneous T-cell lymphomas (CTCLs), accounting for approxi-mately 30% of CTCLs. This group includes primary cutaneous anaplastic large celllymphoma (C-ALCL), lymphomatoid papulosis (LyP), and borderline cases. It is nowgenerally accepted that C-ALCL and LyP form a spectrum of disease, and thathistologic criteria alone are often insufficient to differentiate between these twoends of this spectrum. The clinical appearance and course are used as decisivecriteria for the definite diagnosis and choice of treatment, thus requiring amultidisciplinary approach and expertise to ensure them. A 53-year-old manpresented with a history of painless erythematous nodule on the right foot,progressing to increase the number of lesions and anatomical areas involved, in 9months, such as trunk, thighs, legs and feet. He denied systemic symptoms. Onexamination, he had erythematous infiltrated plaques and nodules with centralulceration in the right lumbar region, bilateral popliteal fossa and the medial aspectof left foot. The diagnostic hypothesis was skin cancer and incisional biopsy andsubsequently immunohistochemistry were necessary for definitive diagnosis. Theyrevealed cutaneous CD30+ T cells, with CD3 e granzima Immunohistochemicalexpression, and after clinicopathological correlation to differentiate the type C LyPand C-ALCL, we diagnose C-ALCL. In the next moment, we excluded the systemicmanifestation of the disease with normal carried out laboratory tests and imaging.The patient was treated with cyclophosphamide, doxorubicin, vincristine andprednisone regimen (CHOP). Currently, after six sessions of chemotherapy thepatient is in remission of the disease. The differential diagnosis between PC-ALCLand LyP is important, since patients with LyP have a 10% to 20% increased risk fordeveloping lymphoid malignancy in comparison to PC-ALCL and because thetherapeutic strategy among these conditions differs. In C-ALCL, individual lesionsare usually [2 cm in diameter and partial or complete spontaneous regressionoccurs in approximately 25% of patients, different from LyP. The prognosis is usuallyfavorable, but the progression to extracutaneous disease is reportedly and occursmore frequently in patients with multifocal skin involvement. For this reason,systemic chemotherapy has generally been recommended for that subset ofpatients.
cial support: None identified.
CommerJ AM ACAD DERMATOL
P6801Primary cutaneous B-cell lymphoma: A case report
Asfa Akhtar, DO, Cleveland Clinic Florida, Weston, FL, United States; RoyaGhorsriz, DO, Nova Southeastern University Department of Dermatology, Davie,FL, United States
The classification of primary cutaneous B-cell lymphoma (PCBCL) is controversial.PCBCL is divided into 3 major subtypes based on the World Health Organization-European Organization for Research and Treatment of Cancer (EORTC). Theseinclude primary cutaneous follicle center lymphoma (PCFCL), primary cutaneousmarginal zone B-cell lymphoma (PCMZL), and primary cutaneous diffuse large B-celllymphoma, leg type (PCLBCL, LT). A combination of clinical, histologic, immuno-logic, and molecular criteria is required in the diagnosis of PCBCL. Primarycutaneous B-cell lymphomas are generally located on the head and neck, with thetrunk and extremities affected to a lesser degree. PCFCLs present with plaques ornodules localized to the scalp, face, or trunk followed by the legs. The upperextremities tend to be spared. PCMZLs comprise cases with erythematous papules,nodules or plaques on the trunk or extremities. PCLBCL, LTs present as rapidlygrowing violaceous-red tumors on the lower extremities. Immunohistologically all 3PCBCLs are positive for the B-cell markers CD20 and CD79a. There has also been anassociated relationship with infectious triggers to include Borrelia burgdorferi,Helicobacter pylori, and EpteineBarr virus. Systemic lymphoma workup iswarranted in all cases of cutaneous B-cell lymphoma. Treatment involves multiplemodalities to include specific treatment aimed at concurrent or suspected infection.We present a case of a 56-year-old Israeli man who presented to the deparment ofdermatology with an asymptomatic localized eruption on the right posteriorshoulder of unknown duration. His medical history was significant for hyperlipid-emia. Physical examination revealed a circumscribed erythematous patch on theright posterior shoulder. Biopsies revealed an atypical intradermal nodular anddiffuse lymphocytic proliferation consistent with primary cutaneous follicularcenter lymphoma with diffuse large cell pattern. A comprhehensive evaluation bythe department of oncology was negative for systemic involvement. The patientunderwent radiation therapy. Six months later, he developed a similar erythematouspatch on his left mid back. Physical examination of the left mid back demonstrated apoorly circumscribed erythematous patch. Biopsies revealed cutaneous B-celllymphoma, follicle center cell type. The patient was referred back to the departmentof oncology, where he was treated with 3 cycles of rituximab. He is pending follow-up.
cial support: None identified.
CommerP6567Primary cutaneous diffuse large B-cell lymphoma, leg type: Two Bcl-2negative cases
Nicole Yi Zhen Chiang, MBChB, Birmingham, United Kingdom; Aleem Uddin,MBBS, Birmingham, United Kingdom; Anthony Abdullah, MBChB, Birmingham,United Kingdom; Donna Thompson, MBBS, Birmingham, United Kingdom;Parveen Abdullah, Birmingham, United Kingdom; Shireen Velangi, MBChB,Birmingham, United Kingdom
Primary cutaneous diffuse large B-cell lymphoma, leg lype (PCLBCL, LT) is a rarecutaneous B-cell lymphoma with a predominance or confluent sheets of centro-blasts and immunoblasts. Bcl-2 is strongly expressed inmost cases.We report 2 casesof PCLBCL, LTwith negative Bcl-2. One of these cases was associated with the use ofimmunosuppressants. Patient 1, a 76-year-old man with multiple sclerosis andbullous pemphigoid, presented with a 6-week history of asymptomatic lesions onhis left leg. He had been taking azathioprine (75 mg twice daily) and prednisolone(7.5 mg daily) for bullous pemphigoid for the last 18 months. Examination showedmultiple hard, purplish nodules on his left leg. Skin biopsy showed heavy infiltrationof lymphoid cells in the dermis with a mixture of mature lymphoid cells and blastscells. The cells were stained positive for CD79a, but negative for CD3, CD10, CD23,and Bcl-2. Patient 2, an 85-year-old woman with limited mobility caused by severeosteoarthritis, presented with a small red patch on her left lower leg, whichdeveloped into multiple nodules covering the whole of her left lower leg over thecourse of 4 months. The lesions were occasionally pruritic. Examination showedmultiple large, tumid, red-purple, coalescing nodules with some areas of ulcerationand yellow crusting. Skin biopsy showed large neoplastic cells infiltration in thedermis constituting mainly of immunoblasts and centroblasts with prominentmitosis and tangible-body macrophages. The cells were stained positive for CD20,CD79a, Bcl-6, CD10 and MUM1, but negative for Bcl-2. Clinical and histologicfindings in both cases were diagnostic of PCLBCL, LT. Both patients did not have anyextracutaneous dissemination. Patient 1 died shortly after diagnosis with a bron-chopneumonia. Patient 2 was commenced on low dose oral chemotherapy(Prednisolone and etoposide) with adjuvant radiotherapy as a palliative treatment.PCLBCL, LT is an aggressive lymphoma. Both our cases were Bcl-2 negative, anuncommon feature in PCLBCL, LT. Prognosis for PCLBCL, LT is generally poor withan overall 5-year survival rate of 41%. PCLBCL, LT has been associated withimmunodeficiency before, and immunosuppression may have played a role ininducing PCLBCL, LT in patient 1.
cial support: None identified.
CommerAPRIL 2013