Hemophagocytic syndrome and neurological involvement in a case of intravascular large B-cell lymphoma

Intravascular large B-cell lymphoma (IVLBCL) is a rare form of non-Hodgkin’s lymphoma, and is divided into Western and Asian variants. The latter is rarely found to have neurological system involvement. In China, there have only been a few diagnosed cases of IVLBCL. Here, we present a Chinese case of Asian-variant IVLBCL with neurological symptoms. A 32-year-old Chinese man presented with bilateral lower limb numbness and persistent fever. He also complained of difficulties in urination and defecation. In addition, splenomegaly and pancytopenia were observed. We identified 3% dysplastic lymphocytes in his peripheral blood film, and his bone marrow biopsy led to a diagnosis of Asian-variant IVLBCL. Lumbar spine magnetic resonance imaging, which revealed an edematous spinal cord, further confirmed neurological involvement. The patient refused treatment from the time of diagnosis, and died 2 months after being discharged. IVLBCL is a highly aggressive but nonspecific clinical manifestation that is difficult to diagnose; therefore, a greater understanding of the disease is needed. The current first-line therapy involves R-CHOP combination therapy (cyclophosphamide, doxorubicin, vincristine, prednisone, and rituximab); however, the overall prognosis of IVLBCL remains poor.     

眼眶NK／T细胞淋巴瘤误诊2例分析并文献复习

目的：综述眼眶NK／T细胞淋巴瘤的临床诊断、治疗及常见误诊原因。方法：结合文献和2例个案，对眼眶NK／T细胞的临床诊断、治疗及鉴别诊断进行分析。结果：眼眶NK／T细胞淋巴瘤是临床罕见的一类结外淋巴瘤，易误诊为眼结膜炎、眼非特异性炎症、眼眶周围软组织炎或蜂窝织炎。病理改变、免疫表型符合鼻型NK／T细胞淋巴瘤。结论：眼眶NK／T细胞淋巴瘤临床表现、免疫表型、影像学特征独特，容易误诊，临床进展快，死亡率高。     

Osteoblast differentiation; Hyperglycemia; Osmotic stress; Diabetes 散发性伯基特及伯基特样淋巴瘤临床病理分析

目的：探讨伯基特淋巴瘤（BL）的临床病理特征、免疫表型及鉴别诊断。方法：运用组织病理学、免疫组织化学和EBER原位杂交，对13例BL及7例伯基特样淋巴瘤（BLL）患者的肿瘤组织进行检测．并复习相关文献。结果：20例散发性病例中，病变部位在淋巴结内者，位于颈部淋巴结6例．颌下淋巴结4例，锁骨下淋巴结1例，腋下淋巴结1例；病变部位在淋巴结外者，位于回盲部3例，精索、睾丸、结肠、肌肉、前列腺各1例。光镜下可见瘤组织由单一的、弥漫浸润的中等大小圆细胞组成，其中散在分布着吞噬细胞碎片的组织细胞，呈“星空”样改变。免疫表型检测示，瘤细胞弥漫表达CD20和CD79α，部分表达CD10，〉90％的瘤细胞Ki-67阳性，而CD3、CD43、bcl-2和TdT呈阴性。EBER原位杂交发现，3例瘤细胞呈阳性。结论：散发性BL属高度恶性肿瘤，确诊依赖于病理组织学与免疫组化标记。     

嗜碱性粒细胞白血病的诊断与治疗进展

嗜碱性粒细胞增多常见于慢性髓细胞白血病(CML)加速期或急变期,其变化可作为CML的预后评价指标.嗜碱性粒细胞白血病(BL)是以嗜碱性粒细胞异常增多为主要临床表现的独立白血病类型.临床在排除其他血液系统疾病后,依据骨髓检查及外周血检查结果将嗜碱性粒细胞异常增多引起的BL分为急性BL和慢性BL.因BL发病率较低,目前尚无明确的诊断标准.随着检测技术和诊疗水平的不断提高,基于形态学、免疫学、细胞及分子遗传学(MICM)的诊断模式,可使越来越多单独发病的BL被识别和发现.笔者拟就目前国内外发现的BL的诊断和治疗进展进行综述.     

Marked hyperuricemia with acute renal failure: need to consider occult malignancy and spontaneous tumour lysis syndrome

Spontaneous tumour lysis syndrome (TLS) is an extremely uncommon cause for acute renal failure (ARF). ARF presenting with hyperkalemia, hyperuricemia and hyperphosphatemia should lead to further work up for occult haematological malignancy. We describe two cases of lymphoma presenting with ARF secondary to spontaneous TLS. The ARF in the first case appeared to be due to intravenous volume depletion but eventually lead to the diagnosis of gastric lymphoma. The second patient is interesting as he is probably the first reported case of lymphoma with HIV infection presenting as spontaneous TLS. Early diagnosis and prompt renal replacement therapy does not necessarily reverse the ARF.     

甲状腺原发性Burkitt淋巴瘤临床病理观察

目的甲状腺原发性Burkitt淋巴瘤非常罕见,对其临床病理特点进行探讨。方法对1例患有甲状腺Burkitt淋巴瘤的9岁男童进行详细的临床资料和病理形态学观察,并采用MaxVision法进行免疫组化染色、EBER原位杂交和c-myc基因FISH检测。结果甲状腺组织内见中等大小异型淋巴细胞弥漫浸润,可见明显"星空"现象。肿瘤细胞CD20、CD10、bcl-6、CD43和CD38(+),CD3、CD5、bcl-2和TDT均(-),Ki-67阳性率>95%。FISH检测有c-myc基因位点的染色体断裂重组。EBER分子原位杂交(-)。结论本例为原发于甲状腺的散发型Burkitt淋巴瘤,与EBV感染无关。需要与弥漫性大B细胞性淋巴瘤、结外黏膜相关淋巴组织边缘区淋巴瘤和B淋巴母细胞性淋巴瘤/白血病进行鉴别。     

以纵隔肿块为表现的慢性粒细胞白血病髓外急变

目的探讨慢性粒细胞白血病髓外急变(extramedullary blast crisis of chronic myelogenous leukemia,CML-EBC)的诊断及治疗要点。方法对1例CML-EBC的临床资料进行回顾性分析并复习相关文献。结果本例因确诊慢性粒细胞白血病3年,胸背痛伴呼吸困难2个月入院。行胸部CT扫描示胸腹主动脉旁椎前梭形软组织密度影;复查骨髓涂片+骨髓流式细胞检测符合慢性粒细胞白血病(慢性期);行胸腔镜下纵隔病变活检及免疫组织化学检查,诊断考虑恶性肿瘤浸润,淋巴造血系来源可能;组织病理切片荧光原位杂交(FISH)技术分析示BCR/ABL融合基因阳性。明确诊断为慢性粒细胞白血病髓外B淋巴细胞急性变,予伊马替尼等药物化疗后病情好转出院,继续予伊马替尼维持治疗。结论 CML-EBC易误诊为慢性粒细胞白血病并淋巴瘤,临床应注意鉴别,治疗可选用化疗、局部放疗、造血干细胞移植等综合措施。     

p63阳性的弥漫大B细胞淋巴瘤1例并文献复习

弥漫大B细胞淋巴瘤是最常见的非霍奇金淋巴瘤,p63在弥漫大B细胞淋巴瘤中可表达,因此在临床上易被误诊为p63阳性的上皮源性恶性肿瘤,尤其在淋巴结发生转移时。本研究报道1例以呼吸道症状为首发症状的弥漫大B细胞淋巴瘤患者,临床高度怀疑肺恶性肿瘤并淋巴结转移。行右腋窝淋巴结穿刺活检病理诊断,结果显示p63阳性,提示为鳞状细胞癌,而完善免疫组织化学检查后,最终诊断为弥漫大B细胞淋巴瘤。因此,认识p63阳性的弥漫大B细胞淋巴瘤很有必要,可减小临床误诊的可能性。     

T-lymphoblastic lymphoma with cutaneous involvement

To study dermatological manifestation of T-lymphoblastic lymphoma and to help clinicians in the diagnosis, we report here the case of a 75-year-old patient who presented with violaceous nodules acquired during the last 4 wk and affecting the scalp and right arm. The diagnosis of systemic lymphoma was suggested upon the appearance of cutaneous tumors, palpable lymph nodes and general symptoms including asthenia and weight-loss. The pathology features: positive immunostaining for CD3 and terminal deoxynucleotidyl transferase(Td T) and staging, led us to the final diagnosis of T-lymphoblastic lymphoma(T-LBL) with cutaneous involvement. He received a CHOP regimen as first-line treatment. Unfortunately, the patient relapsed and died 8 mo after the treatment initiation. T-LBL may be diagnosed by skin lesions. Additional immunostaining including Td T and experienced histopathologists are needed to correctly classify this aggressive disease and discuss the correct management including bone-marrow transplantation where appropriate.     

Muscular involvement of extranodal natural killer/T cell lymphoma misdiagnosed as polymyositis: A case report and review of literature

BACKGROUND Natural killer(NK)/T cell lymphoma is a rare and highly aggressive malignant tumor,and is a special form of non-Hodgkin's lymphoma.Although extranodal involvement is frequently found in tissues such as the skin,testicular and gastrointestinal tract etc,its presence in skeletal muscle has scarcely been reported in the literature.CASE SUMMARY We report a case of extranodal NK/T cell lymphoma with muscle swelling as the first clinical manifestation.A 42-year-old man,who initially presented with localized swelling in the double lower extremities,demonstrated gradual facial and eyelid swelling,and his imaging results showed multiple sites of muscle damage throughout the body.The final pathological results suggested NK/T cell lymphoma,and immunohistochemistry showed CD20(-),CD3(+),CD30(+),CD56(-),EBER(+),Ki67(60%),TIA-1(+)and CD68(±)staining.The muscle swelling significantly improved after treatment with chemotherapy regimens.CONCLUSION This disease is difficult to diagnose and highly invasive,and should be included in the differential diagnosis of unexplained muscle swelling.     

套细胞淋巴瘤发病机制的研究进展

套细胞淋巴瘤(MCL)是一种侵袭性的非霍奇金淋巴瘤(NHL),该病的经典遗传学标志为t(11;14)(q13;q32)移位和细胞周期蛋白(cyclin) D1过表达,以难治和易复发为临床特征,并且总体预后不良.近年,随着对MCL细胞遗传学及分子发病机制研究的不断深入,靶向药物的开发与应用,使得该病的临床疗效有所提高.本文就近年来MCL发病机制中细胞遗传学、信号通路、转录因子、肿瘤微环境等改变的研究最新进展作一综述,旨在为MCL的早期诊断和靶向治疗提供新的方向.     

Recurrent inverted papilloma coexisted with skull base lymphoma: A case report

BACKGROUNDInverted papilloma is an uncommon neoplasm in the nasal cavity. It is a histologically benign tumor, but has a high recurrence and local invasion rate. In addition, nasal or skull base lymphoma is another rare neoplasm. The coexistence of these two tumors in one case makes the diagnosis and related treatment difficult.CASE SUMMARYWe report a case of an immunocompetent patient, who had a history of inverted papilloma 20 years ago. The patient presented with an infiltrated mass lesion in the nasal cavity with extension to the frontal base. The repeated biopsies revealed inverted papilloma without any malignant transformation. After the patient underwent a frontobasal craniotomy with total tumor excision, the final pathological examination revealed nasal inverted papilloma coexisting with diffuse large B-cell lymphoma of the skull base.CONCLUSIONBased on this case report, while managing a case of an aggressive recurrent inverted papilloma, not only squamous cell carcinoma transformation, but also other invasive malignancy, such as lymphoma, should be considered.     

Adult T-cell leukemia/lymphoma in the Middle East: first report of two cases from Lebanon

BACKGROUND: Adult T-cell leukemia/lymphoma (ATL) is an aggressive lymphoproliferative disorder caused by human T-cell leukemia virus type I (HTLV-I). HTLV-I is endemic in southern Japan, the Caribbean, Central and South America, certain areas of Africa, and the southeastern United States. In the Middle East, North East Iran, particularly the region of Mashhad, has been recognized as an endemic region.
CASE REPORTS: In this report, the first two cases of ATL diagnosed in Lebanon are described. The first patient of Lebanese origin presented with acute ATL. The second patient of Romanian origin developed acute ATL in early relapse after autologous transplantation for ATL. Both patients had lymphocytosis, severe hypercalcemia, and CD25+ T-cell immunophenotype on peripheral blood. In both patients, HTLV-I serology was positive by enzyme-linked immunosorbent assay and confirmed by Western blot and HTLV-I oncoprotein Tax expression was documented in the leukemic cells. Upon screening, seven direct family members of the first patient were HTLV-I positive; four of them were regular blood donors.
CONCLUSIONS: Screening blood donors for HTLV-I seropositivity is not currently performed in Lebanon. A large screening study in Lebanon is needed to confirm whether South Lebanon is a new endemic region for HTLV-I infection and to recommend mandatory screening of blood donors for HTLV-I infection.     

Hodgkin's and non-Hodgkin's hepatic lymphoma: Sonographic findings

The sonographic studies of 72 patients with pathologically proven Hodgkin's or non-Hodgkin's hepatic lymphoma were retrospectively reviewed. Sixty-eight patients (94%) had secondary hepatic lymphoma (nine of them had AIDS-related lymphoma) and four patients (6%) had primary lymphoma of the liver. Forty-six of 72 patients (64%) had diffuse hepatic involvement, and 26 of 72 patients (36%) had focal liver lesions as demonstrated by sonography. Four patterns of disease were identified: (a) hepatomegaly was depicted by sonography in 26 of the 59 patients with secondary hepatic lymphoma not related to AIDS, in two of the nine patients with AIDS-related secondary hepatic lymphoma, and in one of the four patients with primary hepatic lymphoma; (b) multiple rounded well-delineated hypoechoic liver lesions were found in 22 of the 68 patients with secondary hepatic lymphoma; (c) a large heterogeneous echoic mass, which was an evocating clue to the diagnosis of primary lymphoma of the liver, was found in the four patients with primary lymphoma of the liver; and (d) an absence of sonographic abnormalities was found in 20 of the 59 patients with secondary lymphoma not related to AIDS. Liver involvement with lymphoma should be considered in any patient who develops multiple homogeneous hypoechoic liver masses, even in the absence of known underlying lymphomatous disease.     

循环肿瘤DNA在弥漫大B细胞淋巴瘤中的临床应用

弥漫大B细胞淋巴瘤(DLBCL)作为一种侵袭性淋巴瘤,目前尚未有较好的分子标志物检测方法用于评估其疗效及预后。本文通过与传统检测方法相比较,发现循环肿瘤DNA(ct DNA)可以作为一种非侵入性的、能动态反映淋巴瘤信息的特异性生物标志物;DLBCL细胞中的ct DNA可以通过二代测序结合PCR技术获取,从而进行基因标记,淋巴瘤相关基因的靶向测序,淋巴瘤体细胞突变的d PCR及异常甲基化检测;ct DNA与DLBCL的诊断、疗效以及预后息息相关,可以应用于DLBCL的早期检测、中期监测、预后监测等方面,使得ct DNA在DLBCL的使用中具有广阔的应用前景。     

Primary colonic lymphoma: report of two cases and a literature review

Primary colonic lymphoma is a very rare malignant tumor with no standard treatment. We report two cases of primary colonic lymphoma successfully treated with surgery and chemotherapy, and chemotherapy alone, respectively. The first case was a 61-year-old woman who presented with abdominal pain of more than 1 month. The patient was diagnosed with a colonic tumor, and immunohistochemical examinations confirmed the initial diagnosis of colonic lymphoma. The patient underwent laparoscopic-assisted right hemicolectomy followed by postoperative adjuvant chemotherapy with the cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) regimen, combined with targeted therapy with rituximab (R-CHOP). The second case was a 78-year-old man who presented with a complaint of abdominal distention for more than 1 year. Diffuse large B-cell lymphoma was definitively diagnosed by immunohistochemical examinations, and the patient underwent systemic chemotherapy with the R-CHOP regimen. Primary colonic lymphoma is a rare type of non-Hodgkin''s lymphoma (NHL), and the clinical treatment is not standardized, unlike for many other types of lymphoma. Therefore, treatment is mainly based on the patient’s symptoms to determine whether surgery or systemic chemotherapy is appropriate. Rituximab is effective in some patients and may play an important role in the treatment of unresectable or asymptomatic colonic lymphoma.     

Primary T-lymphoma of the breast with bilateral involvement, unusual presentation

Primary or secondary involvement of the breast is a rare form of extranodal lymphoma. Most reported primary non-Hodgkin lymphomas of the breast have a B-cell phenotype, those of T-cell phenotype are even more rare. Bilateral breast involvement at diagnosis also is very rare. We herein report a young female patient with bilateral breast involvement by low-grade T-cell malignant lymphoma.     

Primary non-hodgkin lymphoma of the larynx

A rare case of primary large B-cell non-Hodgkin lymphoma of the larynx is reported. The patient was an 80-year-old female who presented with pharyngeal discomfort and dysphagia. Radiotherapy was instituted with complete remission of the tumor. Nine months afterward, she presented with aspiration pneumonia and dysphagia. Indirect laryngoscopy disclosed laryngeal and esophageal edema, which was probably related to radiotherapy. No signs of tumor recurrence were observed. Three months later, she was entirely asymptomatic. Although cases of aggressive course and poor response to therapy exist, primary large B-cell non-Hodgkin lymphoma of the larynx is generally considered a relatively benign and radiosensitive tumor.     

皮肤原发NK/T细胞淋巴瘤1例报告并文献复习

目的 从临床和病理方面研究1例皮肤结外NK/T细胞淋巴瘤,以引起对此病的重视.方法 对1例NK/T细胞淋巴瘤进行临床和病理分析,并进行文献复习.结果 普通病理结合免疫组织化学支持皮肤的结外NK/T细胞淋巴瘤.结论 皮肤的结外NK/T细胞淋巴瘤恶性度高,易误诊,预后差,需依据临床表现、组织学特点、免疫组化表达尽早诊断.     

Primary angiosarcoma of the spleen—CT, MR, and sonographic characteristics: report of two cases

Primary angiosarcoma of the spleen is a rare entity, but it is the most common primary splenic malignancy. These tumors demonstrate an aggressive growth pattern and can be single or multiple. The diagnosis should be suspected in a patient who presents with splenomegaly but without evidence of lymphoma, malaria, leukemia, or portal hypertension. The tumor may also present with acute abdominal symptoms secondary to spontaneous splenic rupture. We describe two cases of primary angiosarcoma of the spleen with computed tomographic, magnetic resonance, and sonographic features.