Primary cutaneous anaplastic large cell lymphoma with intralymphatic involvement associated with chronic lymphedema

Chronic lymphedema predisposes to develop malignant cutaneous tumors, including angiosarcoma, Kaposi's sarcoma and B‐cell lymphoma. T‐cell malignancy has rarely been associated with chronic lymph stasis. Here, we report a case of primary cutaneous anaplastic large cell lymphoma (pcALCL) with lymphatic spread associated with chronic lymphedema. The patient is a 56‐year‐old man who received orchiectomy and right inguinal lymphadenectomy for malignant seminoma 10 years ago, which led to prominent lymphedema of the right leg. He developed extensive skin nodules on the lymphedematous area for 3 months. Histopathology findings confirmed a diagnosis of pcALCL, which is a subtype of cutaneous T‐cell lymphoma characterized by the presence of CD30+ T cells. Intralymphatic infiltration of malignant cells is prominent. The pathogenesis of intralymphatic cutaneous anaplastic large cell lymphoma is largely unknown. Our case suggests that chronic lymphedema resulted in persistent CD4+ T‐cell inflammation, which then may contribute to the development of pcALCL.     

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.     

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.     

红皮病性银屑病合并原发性皮肤间变性大细胞淋巴瘤一例

患者男,56岁.因"全身反复出现皮疹伴瘙痒26年,伴发脓疱2个月"入院.26年前无明显诱因全身反复出现散发鳞屑性皮疹,伴瘙痒,冬重夏轻,具体治疗不详.2002年12月开始皮疹明显加重,全身皮肤变红,脱皮.当地医院诊断为"红皮病性银屑病",给予的最高糖皮质激素剂量为泼尼松15mg/d,皮疹反复.     

Primary cutaneous anaplastic large cell lymphoma with subsequent leg involvement

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is regarded as an indolent type of cutaneous T-cell lymphoma. However, a few recent publications revealed that C-ALCL patients with initial leg involvement had significantly worse survival than those without initial leg involvement. Herein, we report a case of C-ALCL with subsequent leg involvement, which led to death after chemoradiation therapy. A 75 years old Japanese man presented with multiple erythematous nodules in his left arm and the side of his left chest. Histopathological and immunohistochemical studies led to the diagnosis of primary C-ALCL. At the initial diagnosis, no leg lesion was found. One year after the initial diagnosis, C-ALCL appeared in his right lower thigh and left hip. Radiation therapy, low-dose etoposide and CHOP therapy were performed; however, the patient died of malignant lymphoma 4 years after the initial diagnosis. We speculated that the occurrence of subsequent leg involvement may also be indicative of a worse prognosis, as in the case with initial leg involvement in C-ALCL. Therefore, we propose that C-ALCL patients with initial or subsequent leg involvement should be classified as a distinct clinicopathological variant of C-ALCL (“leg-type” involvement) and that they may require intense therapy.     

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.     

Dermatitis herpetiformis in a Japanese patient with anaplastic large cell lymphoma

We report a 73-year-old Japanese man with dermatitis herpetiformis which developed after diagnosis of anaplastic large cell lymphoma. The patient suffered fever, sweating, shivering, and multiple enlarged cervical lymph nodes. The diagnosis of anaplastic large cell lymphoma was confirmed by the histologic features of a biopsied cervical lymph node. The patient underwent combination chemotherapy. However, one month after the initial therapy, pruritic erythematous skin lesions with peripheral vesicles appeared on his buttocks. A skin biopsy showed subepidermal blister formation associated with polymorphonuclear and mononuclear cell infiltrates. Direct immunofluorescence examination of the area adjacent to the lesion showed granular deposits of IgA at the dermoepidermal junction. While it is well-known that dermatitis herpetiformis can develop into lymphoma, there have been only a few reports of its appearance after a diagnosis of lymphoma. This case suggests that dermatitis herpetiformis may be induced by anaplastic large cell lymphoma.     

Neutrophil-rich anaplastic large cell lymphoma presenting in the skin

A neutrophil-rich anaplastic large cell lymphoma (ALCL) presented in the skin of a 47-year-old Japanese woman. The solitary cutaneous lesion was an eroded, 10-mm, dome-shaped nodule involving the skin of her left upper arm. Histologically, it showed a proliferation of pleomorphic, anaplastic, large tumor cells with nuclei of various shapes, including embryo-shaped, Reed-Sternberg cell-like binucleated, and wreath-shaped multiple nuclei, in the dermis and subcutaneous tissues. There was an admixture of neutrophils ranging from about 30% to more than 50% per field. Immunophenotypically, the neoplastic cells were positive for CD30, CD4, leukocyte common antigen, anaplastic lymphoma kinase-1, epithelial membrane antigen, and granzyme B. She had no peripheral neutrophilia. The lesson was excised, and the site on the left upper arm was irradiated. Six and a half months after diagnosis, however, swelling of a left axillary lymph node appeared; it also showed a proliferation of anaplastic large tumor cells admixed with numerous neutrophils ranging from about 25% to more than 60% per field. Southern blot analysis of T-cell receptor gene rearrangement revealed a clonal band. The patient was treated with six courses of cyclophosphamide, doxorubicin, vincristine, and prednisolone chemotherapy with complete remission. Seventeen cases of neutrophil-rich ALCL arising in the skin, lymph node, muscle, testis, and skull bone were reviewed. This form may possibly be induced by trauma or irritation of conventional ALCL, although the true mechanism for the infiltration of neutrophils is still unclear.     

原发性皮肤间变性大细胞淋巴瘤一例

［摘要］报告1例原发性皮肤CD30+大细胞性淋巴瘤。右前臂皮肤红肿浸润、溃疡结痂1月,左侧头面部红肿浸润10天。右前臂皮损组织病理检查：真皮深层以及皮下脂肪组织间瘤细胞浸润,密集分布。瘤细胞胞质丰富,呈嗜酸性,部分呈空泡状,细胞核大,异型性明显,核仁明显,染色质呈粗颗粒状,并可见病理性核分裂象;有少量成熟淋巴细胞浸润。行免疫组化检查示： 瘤细胞CD45R0（+）、CD30（+）,CD68（－）、CD20（-）、EMA（-）及ALK（-）。诊断为原发性皮肤间变性大细胞淋巴瘤     

Primary cutaneous anaplastic CD30+ large cell lymphoma

Primary cutaneous anaplastic CD30+ large cell lymphoma (PCALCL) is part of the spectrum of primary cutaneous CD30+ lymphoproliferative disorders together with lymphomatoid papulosis. It affects mainly elderly patients and presents as skin nodules that tend to ulcerate. Histological and immunohistochemical study show the expression of CD30 antigen in more than 75 % of neoplastic cells. Currently it is considered a low grade lymphoma with favourable prognosis and good response to treatments such as local radiotherapy, methotrexate or surgery. We report a 93-year-old patient with ulcerated nodules in her right leg. Histological and immunohistochemical study confirmed the diagnosis of PCALCL, of non-B, non-T origin. The patient was treated with local radiotherapy with progressive resolution of skin nodules and absence of relapse at 6 months follow-up.     

原发性皮肤间变性大细胞淋巴瘤1例

报告1例原发性皮肤间变性大细胞淋巴瘤。患者女,49岁。右小腿结节1年,溃烂5个月。皮损组织病理检查:真皮内有密集的淋巴样细胞浸润,瘤细胞大、核呈肾形或不规则形、核分裂像多见,免疫组化示瘤细胞约70?30阳性、约20?45Ro阳性,而CD3、CD20、MPO、TIA-1、ALK-1均为阴性。诊断为原发性皮肤间变性大细胞淋巴瘤。     

Co-existent primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis

We describe the case of a 37-year-old female with a history of psoriasiform dermatitis who presented with multicentric primary cutaneous CD30-positive anaplastic large T cell lymphoma (ALCL). Despite aggressive systemic therapy, the patient suffered multiple relapses and the lymphoma spread to cervical and inguinal lymph nodes. Later in her clinical course it was appreciated that she was also suffering from lymphomatoid papulosis (LyP). The case illustrates the overlapping clinical, histological and immunophenotypic features of ALCL and LyP, conditions which represent a spectrum of CD30-positive lymphoproliferative disease. A multidisciplinary approach between dermatologist, oncologist and pathologist is essential for the optimal management of these complex conditions.     

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。     

原发性皮肤CD30+间变性大细胞淋巴瘤临床及组织病理分析

目的：探讨原发性皮肤CD30 间变性大细胞淋巴瘤(ALCL)的临床及组织病理学特征。方法：对3例患者的皮损行组织病理学及免疫组化检查,观察原发性皮肤CD30 ALCL组织病理学特征及CD45、CD3、CD20、CD30、EMA、HMB45等抗体染色结果。结果：3例患者临床上均表现为皮下结节。组织病理学特征为肿瘤细胞异形性明显,呈多形性,胞质丰富,核大,核仁明显。CD30标记阳性,不表达T细胞标记CD45RO及CD3。结论：原发性皮肤CD30 ALCL是具有独特形态特点及临床特征的肿瘤,根据组织学特征及免疫组化CD30阳性,可与其他恶性肿瘤进行鉴别。     

高IgE综合征一例

患儿女,4岁,因咳嗽、发热20余天,颜面、躯十起丘疹、水疱10余天于2007年12月26日入院.入院前20余天,无明显诱因开始出现咳嗽、发热(测体温38℃)、流涕,无咯痰,在当地医院诊断为"上呼吸道感染",给予药物口服(具体不详),咳嗽减轻,体温降至正常.