皮肤B细胞淋巴瘤

报告２例皮肤Ｂ细胞淋巴瘤。例１．５６岁，男性，躯干、四肢多数皮肤结节，伴有淋巴结肿大５个月。例２，５１岁，男性胸部皮肤斑块３个月入院。２例皮损活检，组织病 理均示为淋巴瘤性浸润。侵犯皮肤附属器及血管周围，乳头层浸润稀疏，有无浸润滞。免疫组化均证实为Ｂ细胞性淋巴瘤。便１免疫球蛋白标记双重链（ＩｇＧ，ＩｇＭ）和双轻链（κ，γ）均呈阳性。     

原发性皮肤小B细胞淋巴瘤

报告1例原发性皮肤小B细胞淋巴瘤。患者男，62岁。反复低热4年，躯干暗红斑、斑匠疹1年。外周血白细胞和淋巴细胞计数正常，IgM56100mg／L，κ轻链（free κ）21，73g/k尿本-周蛋白阳性。3次骨髓穿刺均显示增生性骨髓像。皮损组织病理检查：真皮层及胶原束间密集淋巴样细胞浸润，免疫组化染色结果示肿瘤细胞CD20、CD79a、CD5、CD10、部分CD23、bcl-2均（＋），UCHL1、CD4、CD8、CD38、cyclin D1均（-）。组织病理改变符合小B细胞淋巴瘤。诊断：原发性皮肤小B细胞淋巴瘤。     

原发性皮肤大B细胞淋巴瘤的研究进展

阐述原发性皮肤大B细胞淋巴瘤的分类、原发性皮肤富于T细胞的B细胞淋巴瘤、血管内大B细胞淋巴瘤和B-免疫母细胞性淋巴瘤的临床表现、组织病理变化、免疫组化、基因重排与诊断和鉴别诊断，以及皮肤大B细胞淋巴瘤的形态学变异的研究进展。     

皮肤弥漫大B细胞淋巴瘤1例

我科于2013年9月16日收治1例皮肤弥漫大B细胞淋巴瘤-腿型患者,现报告如下。1病历摘要患者女,68岁。因左小腿内侧红色包块伴疼痛3个月余于2013年9月16日就诊。患者自述3个月前无明显诱因自觉左小腿内侧中上段出现疼痛不适,于局部触及约3 cm×2 cm梭形疼痛性皮下包块,表面皮肤正常,四五天后于该部位皮肤表面出现一2 cm×4 cm红色斑块,未予重视,约2周后于该包块下方约5 cm处出现类     

皮肤淋巴瘤第五讲 B细胞-皮肤淋巴样增生

阐述B细胞-皮肤淋巴样增生的命名、临床表现、组织病理、免疫组织化学染色及其与B-小淋巴细胞性淋巴瘤/慢性淋巴细胞性白血病、原发性皮肤滤泡中心细胞性淋巴瘤、原发性皮肤边缘区淋巴瘤等鉴别诊断和治疗。     

继发性皮肤弥漫大B细胞淋巴瘤1例

报告1例继发性皮肤弥漫大B细胞淋巴瘤。患者男,76岁,因左小腿结节1月余就诊。皮肤专科检查:左小腿可见多个2~5cm大小淡褐色结节,压痛明显,左侧腹股沟可扪及一大小约2cm×2cm大小淋巴结,质软,活动度一般,无压痛,余全身浅表淋巴结未扪及肿大。皮损组织病理示:真皮中下层弥漫较大淋巴细胞样细胞浸润,肿瘤细胞形态不规则,异形明显,核大深染,胞浆丰富,病理核分裂象易见。免疫组化:CD20弥漫阳性(+++);Bcl-6(+++);MUM-1部分阳性(++);Ki-6780%~90%(+)。FISH基因检测:BCL6基因发生断裂。诊断:弥漫大B细胞淋巴瘤ⅣB期。予R(利妥昔单抗)+miniCHOP方案(美罗华+环磷酰胺+长春地辛+表柔比星+地塞米松)化疗1次,后患者家属放弃治疗,患者2个月后死亡。     

皮肤B细胞性恶性淋巴瘤1例

患者女，73岁。右下肢大小形态不五之暗红色结节、肿块，部分皮损表面溃破、结痂。组织病理示真皮及皮下组织内大片弥漫性淋巴细胞浸润，细胞有异形性改变，可见核分裂现象；免疫组化染色CD79a呈强阳性表达，CD3呈阴性表达。确诊为皮肤B细胞性恶性淋巴瘤     

皮肤B细胞淋巴瘤一例

原发于皮肤的Ｔ细胞淋巴瘤（ＣＴＣＬ）多见，如蕈样肉芽肿（ＭＦ）和Ｓｅｚａｒｙ综合征。而皮肤Ｂ细胞淋巴（ｃｕｔａｎｅｏｕｓＢ－ｃｅｌｌｌｙｍｐｈｏｍａ，ＣＢＣＬ）则少见［１～３］，皮肤损害酷似ＭＦ的更少见。在此我们报告一例，并附以皮肤病理、免疫组化及超微结构观察。一、临床资料患者男，５５岁。因全身皮肤的浸润性斑片、斑块性损害１年半于１９９３年２月４日入院。患者于１９９１年９月开始于右上肢出现约钱币大淡红色斑片，略痒，１０余天后自行消退，但一周后，局部出现同样损害，且逐渐增多，发展到躯干及四肢、皮疹…     

原发性皮肤弥漫性大B细胞淋巴瘤

报告1例原发性皮肤弥漫性大B细胞淋巴瘤.患者男,87岁.右下肢结节1年余.皮肤科检查:右下肢内侧可见8 cm×8 cm肿块,周围可见数个大小不等黄豆至蚕豆大的散在褐色结节.皮损组织病理示:表皮萎缩,真皮内可见单一核细胞呈团块状浸润,细胞体积大,胞质丰富,胞核淡染,并见核异形性和较多核分裂象.免疫组化结果:Bcl-2(+)、MUM-1(+).诊断:原发性皮肤弥漫性大B细胞淋巴瘤(腿型)(PCLBCL-LT).     

原发性皮肤弥漫性大B细胞淋巴瘤一例

患者,男,51岁。左小腿红斑、结节、斑块伴疼痛3个月。组织病理及免疫组化确诊为原发性皮肤弥漫性大B细胞淋巴瘤,腿型。     

Primary cutaneous marginal zone B-cell lymphoma: a clinical,histopathological, immunophenotypic and molecular genetic study of 22 cases

BACKGROUND: Primary cutaneous marginal zone B-cell lymphoma (MZCL) has recently been described. Differentiation from follicular centre cell lymphomas and lymphocytomas is often difficult due to insufficient experience and a lack of large series of patients. OBJECTIVES: To characterize primary cutaneous MZCL better, we report clinical, histopathological, immunophenotypic and molecular genetics features in a series of 22 patients. METHODS: All patients were treated and followed up at the same institution. Diagnosis of MZCL was based on the World Health Organization classification criteria. All samples were routinely tested with a wide panel of monoclonal antibodies. DNA was extracted from every sample following standard methods. IgH rearrangement and t(14;18)(q32;q21) studies were performed in all samples. RESULTS: Twenty-two patients (20 men, two women; mean age 50 years, range 24-77) were included. The mean follow-up was 43 months. Seventy per cent of patients presented with characteristic skin lesions on the trunk or extremities, consisting of deep red to violaceous infiltrated plaques, nodules or tumours frequently surrounded by diffuse or annular erythema. Four patients presented with lesions on the head and neck area. Two patients had disseminated skin lesions. The main histopathological features were non-epidermotropic, dense lymphocytic infiltrates mainly distributed in a nodular pattern. Adnexal involvement was usually present, with eventual formation of lymphoepithelial complexes. Cytologically, the infiltrate was polymorphous with marginal zone B cells and B-monocytoid cells. Blastoid CD30+ cells were often observed. Colonized reactive germinal centres and lymphoplasmocytoid differentiation were frequently present. Neoplastic cells were CD20+, CD79a+, CD5- and CD10-. Monotypic expression of light chains was observed in 18 cases (13 kappa; five lambda). Clonal IgH rearrangements were detected in 14 cases. The bcl-2 mutation t(14;18)(q32;q21) was demonstrated in two cases. Most patients were treated with local radiotherapy. Complete response rate with this approach was 100%. Six patients (27%) had skin recurrences from 6 months to 8 years after first treatment. Five patients (23%) had extracutaneous involvement. Two of them had a large cell transformation and one died of lymphoma. Three of four patients with head and neck presentation developed extracutaneous disease. CONCLUSIONS: MZCL appears to be a well recognizable entity, clinically, histologically and immunophenotypically. Although prognosis is generally good, the disease has potential for skin as well as extracutaneous recurrences. Large cell transformation and head and neck presentation may be associated with a worse prognosis.     

Intralesional rituximab for cutaneous B-cell lymphoma

Rituximab, a chimeric anti-CD20 monoclonal antibody, has been approved for systemic treatment of relapsed or refractory CD20-positive B-cell non-Hodgkin's lymphoma. As cutaneous B-cell lymphoma (CBCL) also expresses the CD20 molecule, three patients with histologically and immunohistochemically confirmed CBCL without systemic involvement were treated with low-dose intralesional rituximab in a pilot study. Single doses applied ranged from 10 to 30 mg per lesion, according to lesion extent, with a cumulative dose of up to 350 mg. Injections were given two or three times weekly for 3-5 weeks, with a second cycle after 6 weeks in one patient with incomplete remission. Complete and lasting remission was achieved in each patient; this has persisted for up to more than 1 year. The observed adverse events were of grade 1 severity. Results suggest that intralesional rituximab may be a safe and effective new therapy modality for CBCL.     

Cutaneous spindled follicle center cell lymphoma with abundant mucin: A diagnostic pitfall

Primary cutaneous follicle center cell lymphoma is the most prevalent type of primary cutaneous B-cell lymphoma and usually portends a favorable prognosis. Typically, the diagnosis can be rendered based on characteristic histopathologic features and immunohistochemical profile. Rarely, a diagnostically challenging variant with a predominant spindle morphology mimicking other malignant spindle cell neoplasms may be encountered. Even more unusual is the presence of a prominent myxoid stroma in this rare sarcomatoid variant of follicle center cell lymphoma. Herein, we present a case of a 52-year-old man with a slowly enlarging cyst-like lesion on the chin with histopathologic examination revealing a malignant, predominantly spindled neoplasm within an abundant myxoid stroma. Following a broad panel of immunohistochemical stains, the strong positive staining of the spindle cells for LCA (CD45), CD20, and Bcl-6 confirmed the diagnosis of follicle center cell lymphoma. We present this distinctive rare subtype of cutaneous follicle center cell lymphoma to increase awareness of this variant and to discuss challenging histopathologic mimics to consider while highlighting the utility of immunohistochemistry stains to avoid misdiagnosis.     

Concurrent mycosis fungoides and intravascular large B-cell lymphoma in a single patient

Mycosis fungoides (MF) is an indolent, uncommon, non-Hodgkin T-cell lymphoma of the skin. It classically presents with patches, plaques, and tumors and may rarely show spread to internal organs or bone marrow. Up to 7.5% of MF patients may be diagnosed with a second malignancy. Intravascular large B-cell lymphoma (IVLBCL) is an exceedingly rare non-Hodgkin B-cell lymphoma characterized by predominant growth of large neoplastic cells in the lumina of blood vessels. This case presents with an unusual confluence of two rare diagnoses, MF and IVLBCL, made more remarkable by the presence of both diagnoses on a single skin biopsy sample.     

Cutaneous large B-cell lymphoma of the leg masquerading as a chronic venous ulcer

We report on a 74-year-old female patient with a primary cutaneous CD20+, diffuse large cell B-cell lymphoma of the lower leg resembling a chronic non-healing leg ulcer. There was no evidence of systemic involvement on computed tomography (CT) scans of the chest, abdomen and pelvis; a slightly enlarged lymph node in the right groin showed dermatopathic lymphadenopathy on histology and immunohistochemistry. Involvement of the bone marrow and peripheral blood was ruled out by punch biopsy and fluorescent activated cell sorter (FACS) analysis of the blood, respectively. Therapeutic anti-CD20 monoclonal antibody rituximab was given at 375 mg m(-2) i.v. once weekly for 7 weeks, without adverse effects, resulting in a minor improvement in the centre of the ulcerated tumour. Unfortunately, the response was not maintained, and after 7 weeks of treatment the patient started to develop new tumour lesions at the border of the ulcer. Local radiotherapy was started and combined photon and electron beam irradiation induced complete remission of the B-cell lymphoma.     

Scalp tumour as a sign of systemic B-cell lymphoma

An 86-year-old man presented with a painful reddish tumour on the scalp with a 3-month history, mental confusion with recent onset and lymphadenopathies. Histological examination of the lymph node and cutaneous lesion revealed a dense infiltrate of atypical and large B cells. There was no evidence of bone marrow invasion. According to REAL (Revised European-American Classification of Lymphoid Neoplasms), this lymphoma was considered as a diffuse large B-cell lymphoma with concurrent cutaneous and nodal involvement. Cerebral computerized tomography (CT) scan showed bone and dura mater invasion in the right parieto-occipital region with collapse of lateral ventricle. The patient was submitted to systemic chemotherapy with cyclophosphamide, vincristine and prednisolone (CVP). There was a good response with regression of the cutaneous lesion, but the patient died after the third cycle. We point out the unusual clinical presentation and aggressive behaviour of this lymphoma.     

Primary cutaneous B-cell lymphoma associated with actinic prurigo

We describe two patients with a diagnosis of actinic prurigo who subsequently developed cutaneous B-cell lymphoma. This is the first report, to our knowledge, of this association. We propose that chronic antigenic stimulation by ultraviolet radiation, in the context of actinic prurigo, may have been causal in the development of these unusual lymphomas.     

Intravascular B-cell lymphoma diagnosed by skin biopsy

A 58-year-old man presented with unexplained fever, constitutional symptoms, worsening respiratory failure and gross, generalized oedema. He was eventually diagnosed with intravascular B- cell lymphoma on a random skin biopsy. Examination of the skin showed patchy erythema and induration, with peau d'orange. Despite multiorgan failure requiring intensive care, he responded dramatically to multiagent chemotherapy (six cycles of cyclophosphamide, doxorubicin, vincristine and prednisone) and is currently in clinical remission.