RESUMEN
<p><b>OBJECTIVE</b>To study the clinicopathologic features, immunophenotype and molecular genetic changes of T lymphoblastic lymphoma (T-LBL) associated with Langerhans cell histiocytosis (LCH).</p><p><b>METHODS</b>Three cases of T-LBL associated with LCH were included. The morphologic characteristics were reviewed along with immunohistochemical profiling using EnVision method and TCR gene rearrangement by PCR. A review of composite lymphoma previously reported in the literature was performed.</p><p><b>RESULTS</b>All three patients were male with the mean age of 61.7 years. One was Hans and the other 2 were Uyguers. All presented with superficial lymph node enlargement. Biopsy of lymph node showed two abnormal cell populations: distended sinus by large, pale histiocytes with nuclear grooves, and the interfollicular region containing immature-appearing cells with irregular nuclei slightly larger than that of small lymphocyte, dispersed chromatin, inconspicuous nucleoli, scant cytoplasm, and scattered mitotic figures. These cells presented in aggregates and small sheets interspersed with normal-appearing lymphocyte. The histiocytes were positive for CD1a, S-100 protein and CD68. The lymphoma cells were positive for CD3, CD7, TdT and CD34. TCR-γ gene rearrangement was detected in one case by PCR technology. One case involved bone marrow with double phenotype acute leukemia. Amongst the 8 including 5 reported cases, there were 4 males and 4 females. The mean age of the patients and the median age were 54 years. Lymphoadenopathy was the most common presentation. Bone marrow was involved in 4 cases. The time of follow-up was 2 to 27 months. The median survival was 5.5 months and the one-year survival rate was 33.3%.</p><p><b>CONCLUSIONS</b>Diagnosis of T-LBL and LCH should be based on typical morphology, immunophenotype and molecular genetic findings, with differential diagnoses including Langerhans cell hyperplasia originated from dermatopathic lymphadenopathy. When involving lymph node, extensive sampling supplemented by immunohistochemical staining is important to reach a correct diagnosis. Although coexistent T-LBL and LCH is clonally related, the understanding of its pathogenesis requires further investigation.</p>
Asunto(s)
Femenino , Humanos , Masculino , Persona de Mediana Edad , Médula Ósea , Patología , Reordenamiento Génico , Histiocitosis de Células de Langerhans , Genética , Patología , Inmunofenotipificación , Leucemia , Genética , Ganglios Linfáticos , Patología , Leucemia-Linfoma Linfoblástico de Células Precursoras , Genética , Patología , Leucemia-Linfoma Linfoblástico de Células T Precursoras , Genética , Patología , PronósticoRESUMEN
In order to investigate the occurrence of hyperleukocytosis in treating acute promyelocytic leukemia (APL) patients with all trans retinoic acid (ATRA) and to explore the influence of the level of leucocyte on curative effect of ATRA, the APL patients were divided into three different groups according to the count of leucocyte in peripheral blood. Patients with WBC count less than 30x10(9)/L were administered with ATRA alone (the first group), patients with WBC count more than 30x10(9)/L were administered with ATRA alone (the second group) and patients with WBC count more than 30x10(9)/L were treated with ATRA+cytotoxic drugs (the third group). The results showed that hyperleukocytosis were found in 23 out of 39 patients (58.97%). Total remission rates in the second group and in the third group were 91.3%. The remission rates in the first, second and third groups were 100%, 87.5% and 90.9%, respectively. It is concluded that the ATRA in combination with cytotoxic drugs can efficiently control the occurrence of hyperleukocytosis during ATRA-treating APL and reduce the early mortality.
Asunto(s)
Adolescente , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Antineoplásicos , Protocolos de Quimioterapia Combinada Antineoplásica , Usos Terapéuticos , Leucemia Promielocítica Aguda , Quimioterapia , Recuento de Leucocitos , Leucocitosis , Quimioterapia , Resultado del Tratamiento , TretinoinaRESUMEN
To explore CD34(+) antigen expression in new diagnosed acute myeloid leukemia (AML) and analyze the prognosis for CD34(+) AML patients, the expression of antigen CD34 in 238 AML patients was detected by indirect immunofluorescence assay. The results showed that CD34 in 92 out of the 238 patients (38.7%) were positive, there was relationship between the CD34(+) expression and FAB subtypes (M(0), M(1)), and no CD34(+) expression was observed in M(3) subtypes. The complete remission rate of CD34(+) AML patients was 32%, which was lower than that of CD34(-) AML (61%). The lymphoid-associated antigen (CD7) was significantly increased in CD34(+) AML patients, compared with CD34(-) patients (P < 0.05). It is concluded that CD34(+) AML patients show poor prognosis and lower CR rate. The detection of CD34 expression is of some value in predicting prognosis in AML.