ABSTRACT
O linfoma anaplásico de células grandes (ALCL) associado a implantes mamários é um distúrbio linfoproliferativo das células T que foi recentemente reconhecido como uma entidade independente na classificação de linfomas da Organização Mundial de Saúde (OMS). Apesar do pequeno número de descrições, o número de casos está crescendo rapidamente. Das poucas centenas de casos que foram publicados até agora, muito poucos vieram do Brasil e nenhum foi relatado às autoridades locais. Encontramos um caso recentemente, e acreditamos que seu relato à comunidade local de cirurgia plástica poderá chamar a sua atenção para essa patologia emergente. O prognóstico é muito bom na maior parte dos casos diagnosticados. Contudo, ainda se sabe pouco sobre como e por que os implantes de silicone poderiam desencadear uma resposta linfoide, culminando num ALCL.
Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a T-cell lymphoproliferative disorder that has recently been recognized as an independent entity in the World Health Organization (WHO) classification of lymphomas. Despite the small number of reports to date, the number of cases is rapidly increasing. Of the few hundred cases that have been reported so far, very few came from Brazil and none have been reported to the local authorities. We encountered a case of BIA-ALCL and believe that its report to the local plastic surgery community could raise awareness to this emerging pathology. The prognosis is very good in most of the diagnosed cases. However, little is known about how and why silicone implants could trigger a lymphoid response that results in ALCL.
Subject(s)
Humans , Female , Adult , History, 21st Century , Breast Neoplasms , Mammaplasty , Lymphoma, Large-Cell, Anaplastic , Breast Implants , Plastic Surgery Procedures , Seroma , Breast Neoplasms/surgery , Breast Neoplasms/therapy , Mammaplasty/methods , Lymphoma, Large-Cell, Anaplastic/surgery , Lymphoma, Large-Cell, Anaplastic/therapy , Breast Implants/adverse effects , Plastic Surgery Procedures/methods , Seroma/surgeryABSTRACT
Ataxia telangiectasia (AT) is a rare autosomal recessive disease resulting in progressive degeneration of multiple systems in the body. Both A-T homozygote and heterozygote are at increased risk of developing malignancy. We report a family in which three generations were affected by this disorder. Our index case is a 12-year-old female child, born of second degree consanguineous marriage diagnosed to have ataxia telangiectasia at the age of four years, now presented with fever and neck swelling of one month duration. Family history suggestive of ataxia telangiectasia in maternal uncle and younger sibling was present. History of premature coronary artery disease and death in paternal grandfather was present. On evaluation, child was diagnosed to have Alk negative anaplastic large T cell lymphoma. Management included genetic counseling, examination of all the family members, identification of A-T homozygote and providing appropriate care, regular surveillance of the heterozygote for malignancy.
Subject(s)
Ataxia Telangiectasia/diagnosis , Ataxia Telangiectasia/genetics , Ataxia Telangiectasia/therapy , Caregivers , Child , Consanguinity , Female , Genetic Counseling/methods , Humans , Lymphoma, Large-Cell, Anaplastic/diagnosis , Lymphoma, Large-Cell, Anaplastic/genetics , Lymphoma, Large-Cell, Anaplastic/therapyABSTRACT
Anaplastic large cell lymphoma [ALCL] accounts for 10 to 15% of childhood non-Hodgkin's lymphomas. It is characterized by a high frequency of extranodal involvement, a wide morphological spectrum and the expression of CD 30. The therapeutic strategy is not yet well established. We report 5 cases of childhood ALCL diagnosed between 1999 and 2002 in the paediatric oncology and haematology department in Casablanca. Three patients presented with a bone lesion while the other 2 patients had both nodal and cutaneous involvement. The phenotype was T in 3 cases and null in 2 cases. All the patients were in stage III according to Murphy classification. The patients were treated according to the SFOP HM 91 protocol. Four patients are in CR with a follow-up of 41, 26, 18 and 11 months respectively. The fifth patient is lost to follow-up in CR. ALCL accounts for 10% of childhood NHL in our department. It is probably underestimated because of the lack of all monoclonal antibodies especially the ALK. All our patients achieved a CR and 4 of them are alive and disease free survivors. In conclusion, this type of childhood NHL seems to have a good prognosis in our context and more efforts have to be done in the diagnosis of this type of NHL