Administrarea Topica a Carmustinei in Micoza Fungoida. Eficacitate Si Efecte Adverse
1
P2704 Generalized subcutaneous panniculitis-like T-cell lymphoma following rituximab for hemolytic anemia in a patient with chronic lymphocytic leukemia Matthew Hall, MD, Mayo Clinic, Jacksonville, FL, United States; Jason Sluzevich, MD, Mayo Clinic, Jacksonville, FL, United States; John Snow, MD, Mayo Clinic, Jacksonville, FL, United States A 76-year-old man with a decade long history of chronic lymphocytic leukemia, recently complicated by Coombs-positive hemolytic anemia, was treated with four monthly infusions of rituximab (375mg/m 2 ) in combination with cyclophospha- mide (750mg/m 2 ). The anemia resolved, but 2 months later the patient developed rapidly progressive, generalized, asymptomatic, red-brown indurated cutaneous nodules and plaques. Histopathology revealed infiltration of the subcutaneous fat with atypical lymphocytes that rimmed adipocytes, as well as zonal lobular fat necrosis. Immunohistochemical studies were positive for CD3, CD8, granzyme B, and beta F1, and negative for CD4, CD30, and CD56. In situ hybridization studies for Epstein-Barr virus were negative. T cell gene rearrangement studies by polymerase chain reaction revealed monoclonality. A diagnosis of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) was rendered. The development of B-cell lymphoprolifer- ative disorders in the setting of iatrogenic immunosuppression is well recognized. This case represents the unique development of a clonal cutaneous T-cell lymphoma in the setting of an immunosuppressive milieu characterized by systemic B cell depletion. To our knowledge, this is the first reported case of SPTCL associated with previous treatment with rituximab. Commercial support: None identified. P2705 Sarcoid-like granulomatous mycosis fungoides presenting with markedly hyperpigmented plaques Steven Nelson, MD, Mayo Clinic, Scottsdale, AZ, United States; David DiCaudo, MD, Mayo Clinic, Scottsdale, AZ, United States; Jennifer Ray, MD, Mayo Clinic, Scottsdale, AZ, United States A 69-year-old white woman presented with a 10-year history of asymptomatic, enlarging, hyperpigmented plaques on the lower extremities, upper extremities, and trunk. Cutaneous sarcoidosis was diagnosed 2 years earlier based on skin biopsy findings, and hydroxychloroquine was started shortly after receiving the diagnosis. Minocycline was used for a 2-month period during the treatment course. Physical examination revealed multiple, large, well marginated, black patches and indurated plaques bilaterally on the lower legs. On the buttocks, trunk, and arms were violaceous plaques with overlying laxity and wrinkling of the skin. No skin changes were present in the axillae or groin. Incisional biopsy showed dense dermal and subcutaneous infiltrate composed of small lymphocytes admixed with numerous histiocytes and large multinucleate giant cells. The infiltrate expressed CD2, CD3, CD4, and CD5, with diminished expression of CD7, and absent expression of CD30 and CD56. Throughout the infiltrate, numerous intracellular and extracellular pigment granules stained positively with the Fontana and iron stains. Clonal T-cell receptor gene rearrangement was detected by PCR analysis. The patient has discontinued the hydroxychloroquine and is scheduled to be seen in hematology to discuss treatment options. Sarcoid-like granulomas are present in approximately 2% of primary cutaneous lymphomas; granulomatous mycosis fungoides (GMF) is the most common lymphoma to demonstrate this feature. Sarcoidosis has been reported to precede the diagnosis of GMF. Many of these cases have been shown actually to be early GMF masked by the sarcoid-like reactions. The black hyperpig- mentation makes this patient’s presentation unique. The hydroxychloroquine is suspected to have contributed to the impressive amount of both melanin and iron deposition. Although several of the plaques have overlying wrinkled, lax skin, the term granulomatous slack skin (GSS) is reserved for similar changes in the axillae and groin. GSS and GMF are differentiated by clinical features because they cannot reliably be distinguished histopathologically. Recent studies suggest that the prognosis of GMF is worse than that of classic mycosis fungoides. Commercial support: None identified. P2706 Topical carmustine in the treatment of mycosis fungoides: Efficacy and side effects Diana Santiago-et-Sa ´nchez-Mateos, PhD, Hospital Universitario de La Princesa, Department of Dermatology, Madrid, Spain; A ´ frica Jua ´rez, PhD, Hospital Universitario de La Princesa, Department of Dermatology, Madrid, Spain; Amaro Garcı ´a-Diez, MD, Hospital Universitario de La Princesa, Department of Dermatology, Madrid, Spain; Maximiliano Aragu ¨e ´s, PhD, Hospital Universitario de La Princesa, Madrid, Spain; Rebeca Goiriz, PhD, Hospital Universitario de La Princesa, Department of Dermatology, Madrid, Spain Introduction: Carmustine (BCNU) is a nitrosourea alkilating agent topically used for mycosis fungoides (MF) for more than 3 decades. According to the EORTC consensus recommendations, it is included in the skin-directed therapy as a first- line recommendation for stages IA, IB, IIA, and III. Methods: Retrospective study of the patients diagnosed clinically and histologically with MF who have ever received topical BCNU at the Derpartment of Dermatology in Hospital Universitario de La Princesa from January of 1982 to February of 2009. It was used in alcoholic solution (2mg/mL) diluted in water once daily on involved areas, or undiluted if \3% of the body surface area was involved. Definitions of response are as follows: complete response (CR), disappearance of all clinical evidence of disease; partial response (PR), 50% or more improvement, both for at least 4 weeks; no response (NR), less than 50% of improvement. We have analyzed response, time of treatment to get CR, time of freedom from relapse, evolution and side effects. Results: This study includes 19 patients ranging in age from 40 to 83 years. The time from diagnosis to start of treatment ranged from \1 to 17 years. Fourteen patients were in initial stages (3 in IA, 10 in IB, and 1 in IIA) and five were in advanced stages of the disease (1 in IIB, 1 in III, and 3 in IVA). The patients in IVA stage were receiving other systemic treatments. They were followed up for 7 to 142 months. Eighteen patients responded (94.7%): 13 patients achieved CR (72.2%) and five patients got PR (27.7%). Only in one case this treatment was withdrawn for the acute cutaneous side effects. The CR was reached after a period of treatment from 2 to 10 months. Two patients have maintained the CR. The time of freedom from relapse ranged from 3 to 130 months. Although the majority of patients have suffered from relapses of the disease, they most have maintained a stable stage. Four patients have experienced progression of the disease. We have observed frequent cutaneous side effects, such as erythema after application and persistent poikilodermatous changes with striking telangiectasias. Neither hematologic side effects nor skin cancer were observed. Conclusions: Topical BCNU is an effective treatment for MF in initial stages and even in advanced stages when used as a coadjuvant therapy. It is a remarkable safe modality of treatment because the side effects observed are usually cutaneous without secondary malignancy. Commercial support: None identified. P2707 Se ´zary syndrome and lung cancer association: Case report Ana Rita Travassos, Clı ´nica Universita ´ria de Dermatologia, Hospital de Santa Maria, Lisboa, Portugal; Luı ´s Soares de Almeida, Clı ´nica Universita ´ria de Dermatologia, Hospital de Santa Maria, Lisboa, Portugal; Marisa Andre ´, Clı ´nica Universita ´ria de Dermatologia, Hospital de Santa Maria, Lisboa, Portugal; Paulo Leal Filipe, Clı ´nica Universita ´ria de Dermatologia, Hospital de Santa Maria, Lisboa, Portugal Background: Cutaneous T-cell lymphoma (CTCL) has been associated with in- creased risk for secondary malignancies, namely other lymphomas and lung cancer. Se ´zary syndrome (SS) is a leukemic variant of CTCL and is classically defined by the triad of erythroderma, lymphadenopathy, and the presence of Se ´zary cells in the skin, lymph nodes, and peripheral blood. Case report: A 66-year-old white man, a heavy smoker, with a dermatosis charac- terized by erythematous patches and plaques confined to the lower limbs for 7 years, which were devalued. The patient was subsequently diagnosed of squamous cell carcinoma of the left lung (4 years later), and underwent neoadjuvant chemotherapy and lobectomy followed by chemotherapy. During this lung-guided treatment period, there was also a significant improvement of the skin lesions. Thereafter, there was a progressive worsening of the dermatosis and the patient came to our outpatient unit with erythroderma and generalized pruritus. Laboratory examina- tions showed peripheral leukocytosis with the presence of circulating Se ´zary cells (4600 cells/L); an expanded CD4 1 population and T-cell receptor (TCR) gene rearrangement was detected on peripheral blood and bone marrow aspirate. Skin punch biopsies were compatible with mycosis fungoides/CTCL. Computed tomog- raphy scan of chest, abdomen and pelvis revealed small bilateral axillary and inguinal enlarged lymph nodes. On the basis of these clinical, histopathologic, and molecular biologic findings, the patient fulfilled the WHO-EORTC criteria for SS. Conclusion: This clinical case reports the association of SS and lung cancer of the squamous cell carcinoma type. Although we do not know why there is an increased risk of lung cancer in CTCL patients, an appropriate monitoring for the early detection of second cancers might be warranted in patients with CTCL. Commercial support: None identified. AB96 JAM ACAD DERMATOL MARCH 2010
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Transcript of Administrarea Topica a Carmustinei in Micoza Fungoida. Eficacitate Si Efecte Adverse
P2704Generalized subcutaneous panniculitis-like T-cell lymphoma followingrituximab for hemolytic anemia in a patient with chronic lymphocyticleukemia
Matthew Hall, MD, Mayo Clinic, Jacksonville, FL, United States; Jason Sluzevich,MD, Mayo Clinic, Jacksonville, FL, United States; John Snow, MD, Mayo Clinic,Jacksonville, FL, United States
A 76-year-old man with a decade long history of chronic lymphocytic leukemia,recently complicated by Coombs-positive hemolytic anemia, was treated with fourmonthly infusions of rituximab (375mg/m2) in combination with cyclophospha-mide (750mg/m2). The anemia resolved, but 2 months later the patient developedrapidly progressive, generalized, asymptomatic, red-brown indurated cutaneousnodules and plaques. Histopathology revealed infiltration of the subcutaneous fatwith atypical lymphocytes that rimmed adipocytes, as well as zonal lobular fatnecrosis. Immunohistochemical studies were positive for CD3, CD8, granzyme B,and beta F1, and negative for CD4, CD30, and CD56. In situ hybridization studies forEpstein-Barr virus were negative. T cell gene rearrangement studies by polymerasechain reaction revealed monoclonality. A diagnosis of subcutaneous panniculitis-likeT-cell lymphoma (SPTCL) was rendered. The development of B-cell lymphoprolifer-ative disorders in the setting of iatrogenic immunosuppression is well recognized.This case represents the unique development of a clonal cutaneous T-cell lymphomain the setting of an immunosuppressive milieu characterized by systemic B celldepletion. To our knowledge, this is the first reported case of SPTCL associated withprevious treatment with rituximab.
AB96
cial support: None identified.
CommerP2705Sarcoid-like granulomatous mycosis fungoides presenting with markedlyhyperpigmented plaques
Steven Nelson, MD, Mayo Clinic, Scottsdale, AZ, United States; David DiCaudo,MD, Mayo Clinic, Scottsdale, AZ, United States; Jennifer Ray, MD, Mayo Clinic,Scottsdale, AZ, United States
A 69-year-old white woman presented with a 10-year history of asymptomatic,enlarging, hyperpigmented plaques on the lower extremities, upper extremities,and trunk. Cutaneous sarcoidosis was diagnosed 2 years earlier based on skin biopsyfindings, and hydroxychloroquine was started shortly after receiving the diagnosis.Minocycline was used for a 2-month period during the treatment course. Physicalexamination revealed multiple, large, well marginated, black patches and induratedplaques bilaterally on the lower legs. On the buttocks, trunk, and arms wereviolaceous plaques with overlying laxity and wrinkling of the skin. No skin changeswere present in the axillae or groin. Incisional biopsy showed dense dermal andsubcutaneous infiltrate composed of small lymphocytes admixed with numeroushistiocytes and large multinucleate giant cells. The infiltrate expressed CD2, CD3,CD4, and CD5, with diminished expression of CD7, and absent expression of CD30and CD56. Throughout the infiltrate, numerous intracellular and extracellularpigment granules stained positively with the Fontana and iron stains. Clonal T-cellreceptor gene rearrangement was detected by PCR analysis. The patient hasdiscontinued the hydroxychloroquine and is scheduled to be seen in hematologyto discuss treatment options. Sarcoid-like granulomas are present in approximately2% of primary cutaneous lymphomas; granulomatous mycosis fungoides (GMF) isthe most common lymphoma to demonstrate this feature. Sarcoidosis has beenreported to precede the diagnosis of GMF. Many of these cases have been shownactually to be early GMF masked by the sarcoid-like reactions. The black hyperpig-mentation makes this patient’s presentation unique. The hydroxychloroquine issuspected to have contributed to the impressive amount of both melanin and irondeposition. Although several of the plaques have overlying wrinkled, lax skin, theterm granulomatous slack skin (GSS) is reserved for similar changes in the axillae andgroin. GSS and GMF are differentiated by clinical features because they cannotreliably be distinguished histopathologically. Recent studies suggest that theprognosis of GMF is worse than that of classic mycosis fungoides.
cial support: None identified.
CommerJ AM ACAD DERMATOL
P2706Topical carmustine in the treatment of mycosis fungoides: Efficacy andside effects
Diana Santiago-et-Sanchez-Mateos, PhD, Hospital Universitario de La Princesa,Department of Dermatology, Madrid, Spain; Africa Juarez, PhD, HospitalUniversitario de La Princesa, Department of Dermatology, Madrid, Spain;Amaro Garcıa-Diez, MD, Hospital Universitario de La Princesa, Department ofDermatology, Madrid, Spain; Maximiliano Aragues, PhD, Hospital Universitariode La Princesa, Madrid, Spain; Rebeca Goiriz, PhD, Hospital Universitario de LaPrincesa, Department of Dermatology, Madrid, Spain
Introduction: Carmustine (BCNU) is a nitrosourea alkilating agent topically used formycosis fungoides (MF) for more than 3 decades. According to the EORTCconsensus recommendations, it is included in the skin-directed therapy as a first-line recommendation for stages IA, IB, IIA, and III.
Methods: Retrospective study of the patients diagnosed clinically and histologicallywith MF who have ever received topical BCNU at the Derpartment of Dermatologyin Hospital Universitario de La Princesa from January of 1982 to February of 2009. Itwas used in alcoholic solution (2mg/mL) diluted in water once daily on involvedareas, or undiluted if \3% of the body surface area was involved. Definitions ofresponse are as follows: complete response (CR), disappearance of all clinicalevidence of disease; partial response (PR), 50% or more improvement, both for atleast 4 weeks; no response (NR), less than 50% of improvement. We have analyzedresponse, time of treatment to get CR, time of freedom from relapse, evolution andside effects.
Results: This study includes 19 patients ranging in age from 40 to 83 years. The timefrom diagnosis to start of treatment ranged from \1 to 17 years. Fourteen patientswere in initial stages (3 in IA, 10 in IB, and 1 in IIA) and five were in advanced stagesof the disease (1 in IIB, 1 in III, and 3 in IVA). The patients in IVA stage were receivingother systemic treatments. They were followed up for 7 to 142 months. Eighteenpatients responded (94.7%): 13 patients achieved CR (72.2%) and five patients gotPR (27.7%). Only in one case this treatment was withdrawn for the acute cutaneousside effects. The CR was reached after a period of treatment from 2 to 10 months.Two patients have maintained the CR. The time of freedom from relapse rangedfrom 3 to 130 months. Although the majority of patients have suffered from relapsesof the disease, they most have maintained a stable stage. Four patients haveexperienced progression of the disease. We have observed frequent cutaneous sideeffects, such as erythema after application and persistent poikilodermatous changeswith striking telangiectasias. Neither hematologic side effects nor skin cancer wereobserved.
Conclusions: Topical BCNU is an effective treatment for MF in initial stages and evenin advanced stages when used as a coadjuvant therapy. It is a remarkable safemodality of treatment because the side effects observed are usually cutaneouswithout secondary malignancy.
cial support: None identified.
CommerP2707Sezary syndrome and lung cancer association: Case report
Ana Rita Travassos, Clınica Universitaria de Dermatologia, Hospital de SantaMaria, Lisboa, Portugal; Luıs Soares de Almeida, Clınica Universitaria deDermatologia, Hospital de Santa Maria, Lisboa, Portugal; Marisa Andre, ClınicaUniversitaria de Dermatologia, Hospital de Santa Maria, Lisboa, Portugal; PauloLeal Filipe, Clınica Universitaria de Dermatologia, Hospital de Santa Maria,Lisboa, Portugal
Background: Cutaneous T-cell lymphoma (CTCL) has been associated with in-creased risk for secondary malignancies, namely other lymphomas and lung cancer.Sezary syndrome (SS) is a leukemic variant of CTCL and is classically defined by thetriad of erythroderma, lymphadenopathy, and the presence of Sezary cells in theskin, lymph nodes, and peripheral blood.
Case report: A 66-year-old white man, a heavy smoker, with a dermatosis charac-terized by erythematous patches and plaques confined to the lower limbs for 7 years,which were devalued. The patient was subsequently diagnosed of squamous cellcarcinoma of the left lung (4 years later), and underwent neoadjuvant chemotherapyand lobectomy followed by chemotherapy. During this lung-guided treatmentperiod, there was also a significant improvement of the skin lesions. Thereafter,there was a progressive worsening of the dermatosis and the patient came to ouroutpatient unit with erythroderma and generalized pruritus. Laboratory examina-tions showed peripheral leukocytosis with the presence of circulating Sezary cells(4600 cells/�L); an expanded CD41 population and T-cell receptor (TCR) generearrangement was detected on peripheral blood and bone marrow aspirate. Skinpunch biopsies were compatible with mycosis fungoides/CTCL. Computed tomog-raphy scan of chest, abdomen and pelvis revealed small bilateral axillary and inguinalenlarged lymph nodes. On the basis of these clinical, histopathologic, and molecularbiologic findings, the patient fulfilled the WHO-EORTC criteria for SS.
Conclusion: This clinical case reports the association of SS and lung cancer of thesquamous cell carcinoma type. Although we do not know why there is an increasedrisk of lung cancer in CTCL patients, an appropriate monitoring for the earlydetection of second cancers might be warranted in patients with CTCL.
cial support: None identified.
CommerMARCH 2010
