Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 6 de 6
Filtrar
2.
Mod Pathol ; 31(2): 327-336, 2018 02.
Artículo en Inglés | MEDLINE | ID: mdl-28984300

RESUMEN

CD200, a marker currently utilized in the diagnosis of B-cell lymphoma, is uniformly positive in chronic lymphocytic leukemia/chronic lymphocytic leukemia, and is usually absent in mantle cell lymphoma. Over a 6 year-period, of 668 mantle cell lymphoma assessed by flow cytometry, CD200 expression was detected in 25 patients (~4%). All 25 patients had bone marrow involvement; however, 11 (44%) patients had no nodal or extranodal disease and belonged to non-nodal leukemic variant mantle cell lymphoma. Morphologically, bone marrow showed an unusual interstitial infiltrative pattern in 14/25 (56%) and small round cells resembling chronic lymphocytic leukemia in 9/25 (36%). CD23 was positive in 19/25 (76%) patients; and SOX11 was only positive in 5/21(24%). All 4 patients tested showed IGHV mutations. With a median follow-up of 23 months, 12/24 (50%) patients were not treated. These clinicopathological features were significantly different from 154 randomly chosen CD200-negative mantle cell lymphoma patients, in SOX11 positivity (24% versus 74%, P<0.0001), CD23 expression (76% versus 8%, P<0.0001), a non-nodal leukemic presentation (44% versus 2%, P<0.001), and therapy requirement (50% versus 92%, P<0.0001). This is the first study to show that CD200 expression in mantle cell lymphoma, though uncommon, identifies a subgroup of mantle cell lymphoma patients with characteristic pathological features, frequent non-nodal leukemic variant, and an indolent clinical course.


Asunto(s)
Antígenos CD/metabolismo , Linfoma de Células del Manto/metabolismo , Mutación , Proteínas de Fusión Oncogénica/genética , Factores de Transcripción SOXC/metabolismo , Adulto , Anciano , Progresión de la Enfermedad , Femenino , Citometría de Flujo , Humanos , Inmunofenotipificación , Linfoma de Células del Manto/genética , Linfoma de Células del Manto/patología , Masculino , Persona de Mediana Edad , Proteínas de Fusión Oncogénica/metabolismo
3.
Leuk Res ; 127: 107033, 2023 04.
Artículo en Inglés | MEDLINE | ID: mdl-36774789

RESUMEN

The presence of JAK2 exon 12 mutation was included by the 2016 World Health Organization (WHO) Classification as one of the major criteria for diagnosing polycythemia vera (PV). Few studies have evaluated the clinical presentation and bone marrow morphology of these patients and it is unclear if these patients fulfill the newly published criteria of 5th edition WHO or The International Consensus Classification (ICC) criteria for PV. Forty-three patients with JAK2 exon 12 mutations were identified from the files of 7 large academic institutions. Twenty patients had complete CBC and BM data at disease onset. Fourteen patients met the diagnostic criteria for PV and the remaining six patients were diagnosed as MPN-U. At diagnosis, 9/14 patients had normal WBC and platelet counts (isolated erythrocytosis/IE subset); while 5/14 had elevated WBC and/or platelets (polycythemic /P subset). We found that hemoglobin and hematocrit tended to be lower in the polycythemia group. Regardless of presentation (P vs IE), JAK2 deletion commonly occurred in amino acids 541-544 (62 %). MPN-U patients carried JAK2 exon 12 mutation, but did not fulfill the criteria for PV. Half of the patients had hemoglobin/hematocrit below the diagnostic threshold for PV, but showed increased red blood cell count with low mean corpuscular volume (56-60 fL). Three cases lacked evidence of bone marrow hypercellularity. In summary, the future diagnostic criteria for PV may require a modification to account for the variant CBC and BM findings in some patients with JAK2 exon 12 mutation.


Asunto(s)
Trastornos Mieloproliferativos , Policitemia Vera , Policitemia , Humanos , Trastornos Mieloproliferativos/diagnóstico , Trastornos Mieloproliferativos/genética , Trastornos Mieloproliferativos/patología , Policitemia Vera/diagnóstico , Policitemia Vera/genética , Policitemia Vera/patología , Médula Ósea/patología , Policitemia/patología , Janus Quinasa 2/genética , Mutación , Exones/genética
4.
Histol Histopathol ; 30(5): 559-68, 2015 May.
Artículo en Inglés | MEDLINE | ID: mdl-25301266

RESUMEN

Growing evidence supports the involvement of human herpervirus 8, Kaposi's sarcoma associated herpesvirus (KSHV), in the pathology of primary effusion lymphoma, multicentric Castleman's disease, and Kaposi's sarcoma, but the exact mechanism of KSHV contribution to the oncogenic process remains elusive. We studied transgenic mice expressing the ORF K1 of KSHV, whose position in the KSHV genome corresponds to known lymphoproliferative genes of other herpesviruses. K1 protein was previously shown to contain a constitutively active ITAM domain, involved in activation of Akt and pro-survival signaling, and to inhibit Fas-mediated apoptosis by interfering with binding of FasL. All this pointed to a possible role of K1 in the pathogenesis of KSHV-associated cancers. K1 transgenic mice (80-90%) developed lymphoid hyperplasia and splenomegaly at 8 and 10 months of age, 25% had confirmed diagnosis of lymphoma, and 50% developed abdominal and/or hepatic tumors by 18 months of age. Histological examination showed loss of splenic architecture and increased cellularity. Lymph nodes showed disrupted architecture with effaced follicles and other pathological changes, including signs of angiofollicular lymphoid hyperplasia. One of the livers showed signs of angiosarcoma. In summary, our histology results revealed pathological changes in K1 transgenic mice similar to lymphoma, Castleman's disease, and angiosarcoma, suggesting that K1 may contribute to the development of KSHV-associated cancers.


Asunto(s)
Herpesvirus Humano 8 , Linfocitos/citología , Linfoma de Efusión Primaria/virología , Alelos , Animales , Apoptosis , Enfermedad de Castleman/genética , Enfermedad de Castleman/virología , Modelos Animales de Enfermedad , Femenino , Citometría de Flujo , Hiperplasia/genética , Hiperplasia/virología , Ganglios Linfáticos/patología , Linfoma de Efusión Primaria/genética , Masculino , Ratones , Ratones Endogámicos C57BL , Ratones Transgénicos , Sistemas de Lectura Abierta , Sarcoma de Kaposi/genética , Sarcoma de Kaposi/virología , Transducción de Señal , Bazo/citología , Timocitos/citología , Receptor fas/química
5.
Mol Clin Oncol ; 3(6): 1319-1322, 2015 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-26807241

RESUMEN

A 23-year-old woman presented with enlarged right inguinal lymph nodes. The pathological examination of the nodes revealed infiltration by myeloid sarcoma. A bone marrow smear and biopsy revealed cytogenetic abnormalities, with 46,XX,t(9;22) and chronic myeloid leukemia (CML) was diagnosed. The e1a2 BCR-ABL1 fusion transcript was detected. The patient received imatinib-based combined chemotherapy, allogeneic hematopoietic stem cell transplantation, donor lymphocyte infusions and dasatinib treatment. The patient achieved complete response and has remained leukemia-free for >48 months. To the best of our knowledge, this is the first case report of CML with the e1a2 BCR-ABL1 transcript, with extramedullary blast crisis as the initial presentation. The aim of the present study was to discuss this special case with reference to the literature.

SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA