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2.
Cancer Rep (Hoboken) ; 5(10): e1658, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-35715969

RESUMO

BACKGROUND AND CASE: We herein present a case of the co-occurrence of JAK2-mutated essential thrombocythemia (ET) with chronic lymphocytic leukemia (CLL) harboring the recurrent and rare whole-arm translocation, der(8;17)(q10;q10). The co-existence of lymphoproliferative neoplasms and myeloproliferative neoplasms is suggested to be a rare event. Under this condition, the lymphoproliferative disorder presents a clinically indolent course with a low-risk biological profile. However, the present case showed aggressive disease progression, reflecting a poor prognostic factor; that is, the loss of 17p caused by the whole-arm der(8;17)(q10;q10) translocation. CONCLUSION: The present case report emphasizes the importance of considering the involvement of a genetically poor prognostic factor, regardless of the co-occurrence of CLL and ET.


Assuntos
Leucemia Linfocítica Crônica de Células B , Trombocitemia Essencial , Humanos , Janus Quinase 2/genética , Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/diagnóstico , Leucemia Linfocítica Crônica de Células B/genética , Trombocitemia Essencial/complicações , Trombocitemia Essencial/diagnóstico , Trombocitemia Essencial/genética , Translocação Genética
5.
Gan To Kagaku Ryoho ; 48(12): 1511-1513, 2021 Dec.
Artigo em Japonês | MEDLINE | ID: mdl-34911921

RESUMO

We report the case of an 84-year-old man who developed primary diffuse large B-cell lymphoma of the testes during the course of mycosis fungoides treated with topical medication. He was referred to our hospital due to bilateral testicular masses, and bilateral high orchiectomy was performed. A pathological diagnosis of diffuse large B-cell lymphoma was made after an examination of the surgical specimen. Rituximab-combined miniCHOP chemotherapy with prophylactic intrathecal injection resulted in complete remission without recurrence 1 year after diagnosis. People with mycosis fungoides are known to be at a higher risk of secondary malignancies than healthy individuals; hence, a pathological examination is important to confirm the diagnosis.


Assuntos
Linfoma Difuso de Grandes Células B , Micose Fungoide , Neoplasias Cutâneas , Idoso de 80 Anos ou mais , Humanos , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Masculino , Micose Fungoide/tratamento farmacológico , Rituximab , Neoplasias Cutâneas/tratamento farmacológico , Testículo
6.
Transplant Cell Ther ; 27(5): 437.e1-437.e9, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33965190

RESUMO

Post-transplantation acute limbic encephalitis (PALE) is a rare, severe inflammatory disorder in the bilateral limbic system, including the hippocampus. To date, only a few studies have reported details, including risk factors for PALE; however, further clinical evidence of PALE, especially in cerebrospinal fluid human herpesvirus 6-negative cases, is warranted. In addition, data are sparse regarding the risk factors for calcineurin inhibitor (CNI)-induced encephalopathy (CNIE) following allogeneic hematopoietic cell transplantation (allo-HCT) in adults. Therefore, we examined the risk factors for and clinical details of PALE and CNIE. We retrospectively analyzed consecutive patients who underwent allo-HCT between January 2005 and November 2017. A total of 485 patients age 46 years (median) were eligible. In total, 14 PALE cases and 11 CNIE cases were identified. Multivariable analyses identified older age, use of an HLA-mismatched unrelated donor (URD), graft-versus-host disease (GVHD) prophylaxis with CNI and mycophenolate mofetil, and grade II-IV acute GVHD as significantly associated with an increased risk of PALE. In 13 patients who received high-dose methylprednisolone (mPSL) therapy, 6 (46%) responded to mPSL therapy, and 3 (23%) achieved complete remission at day 90 after mPSL administration. Furthermore, myelodysplastic syndrome (MDS), HLA-mismatched URD, and grade II-IV acute GVHD were significantly associated with an increased risk of CNIE. The 5-year nonrelapse mortality rate was 50% in PALE and 63% in CNIE, suggesting a very poor prognosis. In conclusion, this study provides evidence that HLA-mismatched URD and acute GVHD may independently contribute to the development of PALE, possibly in part through HLA-mismatch-derived alloimmune responses. Other than acute GVHD, we have identified MDS and HLA-mismatched URD as novel predictors of CNIE after allo-HCT.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Encefalite Límbica , Adulto , Idoso , Inibidores de Calcineurina/efeitos adversos , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco
7.
Intern Med ; 59(21): 2751-2756, 2020 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-32641652

RESUMO

A 53-year-old man presented with uncontrolled bleeding caused by acquired platelet dysfunction accompanied by calreticulin-mutated primary myelofibrosis. Based on the detection of abnormal platelets, including large gray platelets, under light microscopy and the loss of the second wave of aggregation observed by light transmission aggregometry, the patient was diagnosed with platelet dysfunction accompanied by myeloproliferative neoplasms (MPNs). In addition, the absence of platelet α-granules was confirmed by electron microscopy. Therefore, this condition may be termed "acquired gray platelet syndrome." Acquired platelet dysfunction must be ruled out when abnormal platelets are observed in patients with MPNs.


Assuntos
Calreticulina/sangue , Síndrome da Plaqueta Cinza/complicações , Síndrome da Plaqueta Cinza/terapia , Hemorragia/etiologia , Hemorragia/terapia , Mielofibrose Primária/complicações , Mielofibrose Primária/terapia , Síndrome da Plaqueta Cinza/diagnóstico , Síndrome da Plaqueta Cinza/fisiopatologia , Hemorragia/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Transfusão de Plaquetas/métodos , Mielofibrose Primária/diagnóstico , Mielofibrose Primária/fisiopatologia , Resultado do Tratamento
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