RESUMEN
Traumatic ulcerative granuloma with stromal eosinophilia (TUGSE) is an ulcerative lesion of the oral mucosa with unknown pathogenesis. A few recent case reports have demonstrated molecular evidence of T-cell clonality in TUGSE and CD30 immunoreactivity in the large atypical mononuclear cells, raising the possibility that a TUGSE subset may represent the oral counterpart of primary cutaneous CD30+ T-cell lymphoproliferative disorders. We examined the immunoreactivity for CD30 and T-cell receptor (TCR) gamma gene rearrangement in 37 TUGSE cases. Clonal TCR gene rearrangements were demonstrated in 7 (24%) of 29 cases with amplifiable DNA, and the morphologic features and CD30 immunoreactivity of these cases did not differ from those with polyclonal TCR gene rearrangements. Clinical follow-up was available for 5 of 7 TUGSE cases with clonal TCR gene rearrangement for an average period of 1.75 years after the initial biopsy or excision, and there was no evidence of local recurrence or development of systemic T-cell lymphoproliferative disorder. Without morphologic and/or clinical evidence of lymphoma, T-cell clonality and/or CD30 positivity in these lesions is not indicative of malignancy and should be interpreted with caution.
Asunto(s)
Eosinofilia/genética , Reordenamiento Génico de Linfocito T , Granuloma/genética , Antígeno Ki-1/biosíntesis , Enfermedades de la Boca/genética , Úlceras Bucales/genética , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Células Clonales , Eosinofilia/metabolismo , Eosinofilia/patología , Femenino , Genes Codificadores de los Receptores de Linfocitos T/genética , Granuloma/metabolismo , Granuloma/patología , Humanos , Inmunohistoquímica , Linfoma/genética , Linfoma/patología , Masculino , Persona de Mediana Edad , Enfermedades de la Boca/metabolismo , Enfermedades de la Boca/patología , Úlceras Bucales/metabolismo , Úlceras Bucales/patología , Reacción en Cadena de la PolimerasaRESUMEN
We report a case of compound heterozygous hemoglobins S [beta6(A3)Glu6Val] and Korle-Bu [beta73(E17)Asp73Asn] in a 2-year-old girl. This hemoglobin genotype is associated with a benign clinical course, much like the sickle cell trait; however, its laboratory characteristics are very similar to compound heterozygous hemoglobin S and hemoglobin D-Los Angeles [beta121(GH4)Glu121Gln], which produces severe sickling hemolytic anemia. We describe laboratory data used to resolve this important differential diagnosis and review the interactions between hemoglobin S and the variant hemoglobins that may account for the different clinical phenotypes in compound heterozygotes.
