异基因造血干细胞移植治疗耳道复发的急性早幼粒细胞白血病一例并文献复习

目的:探讨急性早幼粒细胞白血病(APL)髓外复发的相关因素及治疗。方法:对1例APL缓解后耳道复发患者的临床资料进行回顾性分析,并复习相关文献。结果:患者2015年8月诊断为APL(低危型),经诱导后达完全缓解,随后进行巩固、维持治疗,并多次行腰椎穿刺术及椎管内注射化疗药物预防中枢神经系统白血病。2017年3月发现左外耳道新生物,活检确诊外耳道髓系肉瘤,示髓外复发。随后出现骨髓复发。经诱导巩固化疗后行异基因造血干细胞移植,存活至今。结论:对于髓外复发的急性早幼粒细胞白血病,其预后较差,异基因造血干细胞移植治疗有较好疗效。     

Kupffer cells support extramedullary erythropoiesis induced by nitrogen-containing bisphosphonate in splenectomized mice

Our previous study indicated that injecting nitrogen-containing bisphosphonate (NBP) induced the site of erythropoiesis to shift from the bone marrow (BM) to the spleen. This was due to the depletion of BM-resident macrophages, which support erythropoiesis. In this study, we examined NBP treatment-induced extramedullary hematopoiesis in splenectomized mice, focusing on hepatic hematopoiesis. NBP-treated mice did not display anemia or significant change in erythropoietin production, while megakaryopoiesis and erythropoiesis were constantly observed in the liver. Erythroblastic islands were detected in the sinusoidal lumen. Kupffer cells expressed VCAM-1 following NBP treatment, which is an important factor for erythroblast differentiation. Cl₂MBP-liposome treatment depleted the erythroblastic islands, and decreased the number of hematopoietic cells in the liver, as determined by colony forming assays. Together, these results indicate that Kupffer cells support erythropoiesis, acting as stromal cells in the liver, and that they might act as a niche for hematopoietic precursor cells in an emergency.     

Anal canal plasmacytoma—An uncommon presentation site

Background

Extramedullary plasmacytomas (EMP) are rare plasma cell tumors that arise outside the bone marrow. They are most often located in the head and neck region, but may also occur in the other locations. The lower gastrointestinal EMP represents less than 5% of all cases, and location in the anal canal is exceedingly rare.

Aim

We present an exceedingly rare case of anal canal plasmacytoma, aiming to achieve a better understanding of this rare entity.

Methods

We report a case of a 61-year-old man with a bulky mass in the anal canal. The lesion measured about 6 cm and invaded in all layers of the anal canal wall. The biopsy was performed and revealed a round and plasmocitoid cell population with a solid growth pattern and necrosis. The tumoral cells have express CD79a and CD138 with lambda chains. There was no evidence of disease in other locations and these features were consistent with the diagnosis of an extra-osseous plasmacytoma. The patient was submitted to conformal radiotherapy 50.4 Gy total dose, 1.8 Gy per fraction. After 24 months, the patient is asymptomatic and the lesion has completely disappeared.

Conclusions

EMP accounts for approximately 3% of plasma cell malignancies. The median age is about 60 years, and the majority of patients are male. The treatment of choice for extramedullary plasmacytoma is radiation therapy in a dosage of about 50 Gy. Patients should be followed-up for life with repeated bone marrow aspiration and protein studies to detect the development of multiple myeloma.     

甲状腺浆细胞瘤1 例及文献复习

目的:探讨甲状腺浆细胞瘤的病理学特点及临床表现。方法:对1例甲状腺浆细胞瘤进行组织学表现、免疫组化染色观察及文献复习。结果:组织学特点:肿瘤细胞成分单一,大多数为分化较为成熟的肿瘤性浆细胞,胞核圆形或卵圆形,大小较一致,常偏位,染色质呈车辐状或钟面状。免疫组化特点:肿瘤细胞表达CD20(++)、CD79a(+)、CD38(++)、CD138(+)、κ链(++)。结论:甲状腺浆细胞瘤是甲状腺一种少见的肿瘤,应同炎症反应的浆细胞增生及低分化癌、淋巴瘤、髓样癌等相鉴别,可以通过免疫组化染色及形态学观察进行鉴别诊断。     

Splenic extramedullary hemopoiesis caused by a dysfunctional mutation in the NF-κB-inducing kinase gene

NF-κB-inducing kinase (NIK) plays critical roles in the development of lymph nodes and Peyer’s patches, and microarchitecture of the thymus and spleen via NF-κB activation. Alymphoplasia (aly/aly) mice have a point mutation in the NIK gene that causes a defect in the activation of an NF-κB member RelB. Here, we developed a novel method to determine the aly mutation by genetic typing using PCR. This method facilitated the easy establishment of a congeneic aly/aly mouse line. Indeed, we generated a mouse line with aly mutation on a BALB/cA background (BALB/cA-aly/aly). BALB/cA-aly/aly mice showed significant splenomegaly with extramedullary hemopoiesis, which was not significant in aly/aly mice on a C57BL/6 background. Interestingly, the splenomegaly and extramedullary hemopoiesis caused by the aly mutation was gender-dependent. These data together with previous reports on extramedullary hemopoiesis in RelB-deficient mice suggest that NIK–RelB signaling may be involved in the suppression of extramedullary hemopoiesis in adult mice.     

Radiotherapy for granulocytic sarcoma of the breast—Case report and review of the literature

Granulocytic sarcomas are rare tumors that can present in innumerous locations; thus there is very little clinical experience with these cases. Therefore there is no consensus on which is the best treatment for patients with this malignancy.The authors present a case of a female with a granulocytic sarcoma of the breast and review the literature for the role of radiotherapy in the management of this clinical entity.