Intravascular large cell lymphoma revealed by diffuse telangiectasia and cauda equina syndrome

BACKGROUND: Intravascular lymphoma is a rare disease with usually fatal outcome, characterized by the proliferation within the lumen of small blood vessels of neoplastic large lymphoid cells of B-cell origin. We report a case of intravascular lymphoma revealed by diffuse telangiectasia and cauda equina syndrome. CASE REPORT: A 64-year-old Vietnamese woman presented with unexpected fever and weight loss. Three months after the onset of the first symptoms, an oedema appeared on the lower limbs and the trunk, followed by the eruption of diffuse superficial telangiectasia. Neurologic examination revealed a cauda equina syndrome. The diagnosis of intravascular B cell lymphoma was established on cutaneous and muscular biopsy specimen. A moderate hemophagocytic syndrome was observed, confirmed by bone marrow biopsy. Corticosteroid therapy was started, followed by combination chemotherapy yielding complete response. Six months later death occurred, without evidence of relapse of intravascular lymphoma. DISCUSSION: Clinical presentation of intravascular lymphoma is often confusing, mimicking systemic disease, with a predilection for skin and nervous system involvement. Diagnosis is difficult and often an autopsy finding. Prognosis is generally poor, but favourable responses to chemotherapy have been observed after early diagnosis and treatment. The pathogenesis of intravascular lymphoma remains unknown. Dysfunction of cell-endothelial interaction affecting adhesion molecules has been suspected. The implication of Epstein-Barr virus in intravascular lymphoma remains controversial.     

Intravascular large B-cell lymphoma colonizing cutaneous hemangiomas

Intravascular large B-cell lymphoma is a malignant neoplasm characterized by the proliferation of large B cells (rarely of T lymphocytes) confined within the blood vessels. Although the disease can be limited to the skin, involvement of other organs is common. We report a case of intravascular large B-cell lymphoma colonizing the vessels of preexisting cutaneous cherry hemangiomas.     

血管内大B细胞淋巴瘤一例

患者,女,82岁。大腿、腹部皮疹2月余,伴间歇性发热。体检：浅表淋巴结未扪及肿大,肝脾肋下未触及。神经系统检查未见异常。双股及下腹部可见大小不一的暗红色斑块,质地坚实,有触痛,伴有明显的非凹陷性水肿,皮肤表面高低不平,局部呈橘皮样外观。血常规示三系减低。乳酸脱氢酶显著升高。第1次大腿部肿块穿刺和病理活检均未明确诊断。在不同医院诊断过慢性淋巴管炎、皮肤变应性血管炎和发热待查,经抗生素治疗无效,皮疹从大腿渐扩展至下腹部。第2次皮肤组织病理：皮下脂肪组织间隔血管腔内可见异形淋巴样细胞。免疫组化：异形淋巴样细胞抗淋巴细胞毒抗体（LCA）、CD20、CD79α、bcl-2阳性,bcl-6、CD10、CD3、CD45RO、CD30、EMA、AE1/3、CK均阴性,血管内皮细胞示CD34阳性。基于其组织形态学表现,结合免疫组化标记结果,符合血管内大B细胞淋巴瘤的诊断。患者2个月后死亡。     

Intravascular large B-cell lymphoma of the cutaneous variant in Korea

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of diffuse large B-cell lymphoma. It is characterized by proliferation of malignant lymphoid cells within the small vessels of various organs. The skin and central nervous system are commonly involved although it occurs in other organs such as the kidneys, adrenals, lung and liver. In Western patients, there is a cutaneous variant of IVLBCL, which involves only the skin. However, the Asian variant, which rarely involves the skin, is associated with hemophagocytosis. It was reported in the majority of Japanese patients. Here, we report a case of the cutaneous variant of IVLBCL in a Korean individual.     

第四讲 皮肤淋巴瘤皮肤T细胞淋巴瘤向大细胞淋巴瘤转化

阐述皮肤T细胞淋巴瘤向大细胞淋巴瘤转化的临床表现、组织病理、免疫组织化学染色、分子生物学特征、诊断和鉴别诊断与治疗。     

Cutaneous intravascular anaplastic large cell lymphoma

Intravascular lymphoma (IL) is a rare variant of non‐Hodgkin lymphoma with a predilection for skin. Most reported cases are large B cell lymphomas. Intravascular anaplastic large cell lymphoma (IALCL) is extremely rare. Retrospective analysis of a case of cutaneous IALCL was performed. Hematoxylin and eosin stained sections and immunohistochemical staining results were analyzed. The patient was a 47‐year‐old woman who had developed multiple erythematous patches and plaques on her back. The lesions responded well to CHOP (cyclophosphamide, hydroxydoxorubicin, oncovin, prednisone) chemotherapy, but relapsed shortly after therapy. The patient was surviving with the disease for eight years but was ultimately lost to follow up. Histopathologically, the neoplasm evolved from IL to extravascular lymphoma. This was showed in biopsies obtained at different stages of the disease. The lymphoma cells stained positively for CD30, CD45, CD3, CD4, CD5 and Ki67, and lacked expression of anaplastic lymphoma kinase (ALK), CD8, CD45RA, CD45RO, CD20, CD79, CD56, perforin and granzyme B. Our results suggest that IALCL represents a distinct subtype of IL and is histopathologically and biologically different from IL with B, NK or T cell phenotype. Wang L, Li C, Gao T. Cutaneous intravascular anaplastic large cell lymphoma.     

Cutaneous pleomorphic large cell lymphoma.

The case of a primary cutaneous pleomorphic large cell lymphoma occurring in a twenty-one-year-old woman who presented with a blue-reddish nodule on her left cheek of three months' duration is presented. The tumor consisted of pleomorphic blast cells showing high mitotic activity. On immunohistochemical examination, the majority of the tumor cells expressed CD 3 (Leu-4), CD 4 (Leu-3), HLA-DR CD 30 (Ki-1/Ber-H 2), and CD 25 (IL2 receptor). Twenty-two months after excision of the tumor there is no detectable systemic spread of the lymphoma. This case provides further evidence for recent observations that primary cutaneous Ki-1-positive large cell lymphomas without lymph node involvement may have a favorable prognosis after local treatment despite showing histologic pattern of malignancy.     

Primary cutaneous anaplastic large cell lymphoma

Primary cutaneous anaplastic large cell lymphoma (PC‐ALCL) is a CD30+ lymphoproliferative disorder (LPD) of the skin with a relatively good prognosis in the absence of high‐stage disease. CD30+ LPDs comprise approximately 25%‐30% of primary cutaneous lymphomas and as a group represent the second most common clonal T‐cell neoplasm of the skin behind mycosis fungoides. Diagnosis of PC‐ALCL relies strongly on clinicopathologic correlation given the potential morphologic, clinical and molecular overlap with the other cutaneous CD30+ LPD, lymphomatoid papulosis, and more aggressive hematolymphoid neoplasms.     

Primary cutaneous CD30-positive large cell lymphoma

Primary cutaneous CD30-positive large cell lymphoma is a recently described entity, recognized by immunohistochemical criteria. Spontaneous regression may occur and although relapses are frequent, most patients have a favourable prognosis. We describe a 83-year-old woman with a large ulcerated tumour on her right forearm, with 2 months evolution. Histological and immunohistochemical examination revealed a CD30-positive large cell lymphoma. Systemic involvement was not detected. The tumour regressed spontaneously. Cutaneous relapses occurred one year later, all with spontaneous regression. After a 2-year follow-up period, the patient is well, without concurrent extracutaneous disease. This case is in keeping with other reports of spontaneous regression and favourable outcome of this type of cutaneous lymphoma.     

Primary anaplastic large cell lymphoma of the skin

Anaplastic large cell lymphoma is a subtype of nodal and extranodal lymphoproliferative disorder characterized by peculiar histopathological features and the positivity of lymphoid proliferating cells for Ki-1/Ber-H2 monoclonal antibody. A case, misdiagnosed as cutaneous metastasis and treated by surgery, in a woman with a history of previous neoplasms, is reported. In spite of the histopathological malignancy of the tumor, the patient is alive and free from recurrent or metastatic disease after 3 years of follow-up.     

血管内大B细胞淋巴瘤一例

患者女,60岁。反复双下肢结节、溃疡、坏死1年,加重6个月。患者1年前,出现左下肢结节、溃疡、坏死,皮肤瘢痕、硬化,皮损沿血管走行,伴有间断发热。2012年8月16日皮损组织病理检查提示皮肤血管炎,给予甲泼尼龙80 mg/d治疗15 d,皮损好转,但糖皮质激素减量后病情出现反复。既往有慢性肾功能不全病史7年,贫血病史2年及甲状腺结节病史1年……     

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.     

皮肤原发性间变性大细胞淋巴瘤—附1例报告

报道1例皮肤原发性间变性大细胞淋巴瘤。运用HE染色观察组织学形态及LCA、UCHL1、L26、CD30、EMA等抗体的免疫组化染色,以确定本例肿瘤的免疫表型。结果本例瘤细胞表达CD30、EMA及T细胞相关抗原。皮肤原发性间变性大细胞淋巴瘤临床经过缓慢,预后良好。