Specific infiltrate of Hodgkin lymphoma at site of cellulitis mimicking secondary cutaneous involvement

Hodgkin lymphoma (HL) usually involves the lymph nodes, but concomitant cutaneous manifestations can also occur. The diagnosis of cutaneous involvement by HL must be supported by specific clinical and histopathological findings. We describe the case of a 56-year-old man recently diagnosed with HL of the left axillary nodes who developed cellulitis of the left trunk. Histopathological examination of a skin biopsy specimen revealed the presence of large atypical lymphoid cells with the same immunophenotype of those located in the lymph node affected by HL. Our case adds to the many cutaneous infiltrations by neoplastic cells during the course of an inflammatory skin disease, namely cellulitis.     

免疫母细胞性淋巴结病样T细胞淋巴瘤伴皮肤损害1例

免疫母细胞性淋巴结病样Ｔ细胞淋巴瘤（ＩＢＬ－ＴＣ）是特殊类型的周围Ｔ细胞淋巴瘤。临床特征为发热;淋巴结、肝脾肿大;皮疹及高γ球蛋白血症。皮疹呈多种形态,随其发展主要为二型：丘疹结节型和红皮病型。淋巴结病理示淋巴结结构破坏,有异形淋巴样细胞、免疫母细胞和浆细胞样的所谓淡染细胞（ｐａｌｅｃｅｌｌｓ）浸润。免疫学示Ｔ细胞标记。     

Hodgkin Lymphoma in a patient with mycosis fungoides: molecular evidence for separate cellular origins

We report the case of a man with mycosis fungoides (MF), who, 11 years after diagnosis, developed Hodgkin's disease. Although MF is associated with a higher than expected prevalence of other malignancies, including Hodgkin lymphoma, analysis of cells from the skin and lymph nodes showed findings that suggest a separate cellular origin for the two diseases.     

Primary cutaneous T-cell-rich B-cell lymphoma and Hodgkin's disease in a patient with Gardner's syndrome

A 50-year-old patient, suffering from familial polyposis (Gardner's syndrome), initially presented with several nodules on his left arm. Histological examination revealed primary cutaneous T-cell-rich B-cell lymphoma (TCRBCL). Staging procedures failed to detect any systemic involvement. Three years after total excision of the tumours, the patient presented with a non-specific dermatitis, enlarged axillary lymph nodes and splenomegaly. Histological and immunohistochemical examination of lymph node and spleen biopsy specimens resulted in the diagnosis of Hodgkin's disease (HD) of the nodular sclerosis type. Sequence analysis of single cells micromanipulated from skin and from lymph node lesions indicated that both lymphoma infiltrates were derived from the same precursor germinal centre B-cell clone. This is a case showing a clonal relationship between TCRBCL and HD, providing support to the B-cell origin of Hodgkin and Reed-Sternberg cells.     

Lymphomatoid papulosis followed by large-cell lymphoma: immunophenotypical and genotypical analysis

The immunophenotype and genotype of atypical cells in skin and lymph node infiltrates were investigated in a patient with lymphomatoid papulosis (LyP) complicated by anaplastic large-cell lymphoma of the lymph nodes. The large atypical cells in both skin and lymph nodes displayed an almost identical immunophenotype, i.e. CD30+ and CD25+. Southern blot analysis for T-cell receptor beta-chain gene rearrangement revealed an identical gene configuration in DNA extracted from skin and lymph node. Our results strongly support the hypothesis that clonal populations of T cells arising in cutaneous LyP lesions may undergo malignant transformation, spread into regional lymph nodes, and give rise to secondary malignant lymphomas, such as anaplastic large-cell lymphoma.     

CD56-Positive cutaneous lymphoma with multicentric Castleman's disease-like systemic manifestations.

We report a 55-year-old Japanese male with CD56+ cutaneous lymphoma. The patient had multiple cervical lymphadenopathy, a red nodule on his neck, and parotid gland nodularity. Histologic features of the biopsied cervical lymph node showed follicular hyperplasia with numerous plasma cells. A biopsied skin specimen of the nodule on his neck demonstrated dense infiltration of atypical large lymphocytes into the dermis. Immunohistochemical study of this specimen revealed CD3+, CD4+, and CD56+ expression in the majority of neoplastic cells. Polymerase chain reaction assays for the detection of Epstein-Barr virus sequences were positive for lymph node and skin DNA. Laboratory examinations showed polyclonal gammopathy, pancytopenia, and high serum interleukin-6 levels. These clinical and histological findings resembled those of multicentric Castleman's disease.     

副肿瘤性天疱疮伴发滤泡性淋巴瘤一例

报告1例伴发滤泡性淋巴瘤的副肿瘤性天疱疮。患者女,51岁,口腔、外阴疼痛性糜烂、溃疡伴全身皮疹2个月,既往全身淋巴结肿大1年。皮肤组织病理学检查示表皮可见棘细胞松解,表皮内裂隙,散在坏死红染的角质形成细胞,基底细胞液化变性及真皮浅层较致密淋巴细胞浸润。淋巴结病理检查示：非霍奇金淋巴瘤,WHO分类：滤泡性淋巴瘤,1级。     

Brainstem involvement by mycosis fungoides in a patient with large-cell transformation: a case report and review of literature

BACKGROUND: Central nervous system (CNS) involvement by mycosis fungoides (MF) is rare. As compared to meningeal involvement, intraparenchymal spread is especially rare. It is usually seen in advanced disease in conjunction with lymph node or visceral involvement. However, CNS involvement in the absence of progressive skin lesions or other extracutaneous involvement has been reported rarely in patients with transformed MF. METHOD: Case report and review of literature. RESULTS: A 71-year-old female with long-standing MF developed lymphomatous CNS involvement 10 years after the diagnosis of tumor stage MF. At this time, the patient presented with a transient episode of garbled speech followed by generalized weakness. Computerized tomography scan (CT scan) and magnetic resonance imaging scan (MRI scan) of the head revealed a subcortical lesion in the left temporo-frontal lobe. Cerebrospinal fluid (CSF) examination showed atypical T cells, and brain biopsy confirmed parenchymal involvement by T-cell lymphoma. Meanwhile, a biopsy of a skin lesion showed large-cell transformation. No lymph node or other systemic involvement was noted at this time, and the patient was treated with chemotherapy. Twelve months later, the patient developed recurrent CNS lymphoma with multiple organ involvement and expired soon thereafter. CONCLUSIONS: This case illustrates the importance of awareness of the possibility of CNS involvement by MF, especially in transformed MF. CNS involvement may be the only site of extracutaneous involvement in patients with transformed MF, and mental status changes warrant CNS surveillance for this disease.     

Myxoid variant of anaplastic large cell lymphoma involving the skin: a case report

We report the case of a 62-year-old white male who presented with a 2.6-cm ulcerating mass on the skin of the left buttock and ipsilateral inguinal lymphadenopathy. Microscopic sections of the skin lesion showed a nodular and plaque-like growth pattern of a mixed cellular infiltrate throughout the dermis and subcutaneous tissue with prominent myxoid change. There was a dominant population of medium-sized mitotically active atypical cells that expressed CD30, CD4 and EMA. These atypical cells were mixed with eosinophils, neutrophils, mature lymphocytes and histiocytes. Tissue from the inguinal lymphadenopathy showed similar pathologic features, although no residual lymph node tissue was present. A diagnosis of secondary anaplastic large cell lymphoma, myxoid variant, with skin and lymph node/perinodal soft tissue involvement was rendered at the time of complete excision of the buttock mass. The patient received five cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy with complete resolution of lymphadenopathy and no residual cutaneous disease. He was disease-free by PET/CT scan and physical examination at 16 months after chemotherapy. We present this case to highlight the histopathologic and immunophenotypic features of this entity with a discussion of the differential diagnosis and a review of the literature.     

Use of an expanded immunohistochemical panel to distinguish cutaneous Hodgkin lymphoma from histopathologic imitators

In lymph nodes, classical Hodgkin lymphoma can typically be distinguished from non-Hodgkin lymphoma (NHL) by the presence of Hodgkin and Reed-Sternberg cells that co-express CD30 and CD15. However, anaplastic large cell lymphoma (ALCL) and diffuse large B-cell lymphoma (DLBCL) can show identical features, and some cases of classical Hodgkin lymphoma lack CD15 expression, rendering them difficult to differentiate from CD30-positive NHL. The differential diagnosis of cutaneous Hodgkin lymphoma similarly includes ALCL and DLBCL, and, additionally, tumors of mycosis fungoides. Recent studies have shown that classical Hodgkin lymphoma is of B-cell origin in virtually all cases, and shows at least focal weak expression of the B-cell marker PAX5 and often focal weak expression and no expression of the B-cell markers Oct-2 and BOB.1, respectively. All three of these markers are almost invariably absent in T-cell lymphomas and are strongly expressed in B-cell lymphomas. We report a 40-year-old man with classical Hodgkin lymphoma who developed cutaneous nodules. A biopsy from one revealed Hodgkin/Reed-Sternberg cells with a similar immunophenotype to the diagnostic lymph node biopsy, namely CD30+/CD15+, diffusely but weakly PAX5+, focally weakly Oct-2+ and lacking BOB.1 expression, thereby confirming a diagnosis of cutaneous Hodgkin lymphoma. To our knowledge, this is the first report of the expression pattern of the combination of PAX5, Oct-2 and BOB.1 in the context of cutaneous involvement by Hodgkin lymphoma.     

Cutaneous presentation of recurrence of lymphoepithelioid T-cell lymphoma (Lennert's lymphoma)

Lennert's lymphoma (LL) is a T-cell lymphoma characterized by the presence of atypical T lymphocytes, admixed with histiocytes and epithelioid granulomas. Patients present with superficial lymph node involvement, mainly in the cervical areas; thoracic adenopathies and involvement of deep abdominal lymph nodes are rare. Cutaneous involvement is infrequent, reported to occur in only 4-11% of patients, and even rarer is the onset of cutaneous lesions as first sign of a recurrence. We report a female patient who presented with papules and nodules on the trunk and upper limbs as the first manifestation of recurrent LL.     

Cutaneous immunoblastic T-cell lymphoma

Summary The case of a 69-year-old male patient with an unusual type of malignant lymphoma is presented. Clinically, it was at first characterized by follicular papules and erythematous patches, later, by the development of cutaneous tumors and enlarged lymph nodes, and by a severe, finally excruciating pruritus. Treatment with PUVA (psoralen-ultraviolet-A) combined with 40–80 mg prednisolone and then with chemotherapy [COPP regimen (cyclophosphamide, vincristine, procarbacine, prednisone), high-dosage methotrexate followed by citrovorum factor rescue] was not successful. The patient died of pneumonia 2.5 years after the onset of the first clinical symptoms. An immunoblastic infiltrate was observed histologically and electromicroscopically in the initial lesions of the skin. Therefore, the diagnosis of a cutaneous immunoblastic T-cell lymphoma was tentatively made at the beginning, which was later confirmed in numerous biopsies and laboratory investigations. Immunocytologically and enzymecytochemically, the infiltrating cells were shown to be immature T cells; in the lymph nodes, numerous immunoblasts and large Sézary cells were noted beginning in the paracortical areas and leading to the destruction of the normal lymph node structure. A maximum of 18% Sézary cells was observed in the peripheral blood, though there were no very large Sézary cells or blast cells. In the autopsy, a systemic involvement with an atypical lymphoid infiltration was found in numerous internal organs.The special nature of this case justifies its classification as high-grade malignant lymphoma and its differentiation from normal cases of mycosis fungoides. In contrast, mycosis fungoides generally fulfils criteria typical of low-grade malignant lymphomas.Supported by the Deutsche Forschungsgemeinschaft     

Human herpesvirus type 8 and Epstein–Barr virus-associated cutaneous lymphoma taking anaplastic large cell morphology in a man with HIV infection

Human herpesvirus type 8 (HHV-8, Kaposi's sarcoma-associated herpesvirus)-positive lymphoma taking anaplastic large cell morphology in the skin is described in a 46-year-old man with AIDS. Multiple erythematous nodules appeared on the trunk and extremities during the treatment of AIDS. Histological examination of cutaneous nodules showed dense infiltration of CD30 + atypical lymphoid cells in the deep dermis. Immunoglobulin JH gene rearrangement was detected in these lymphoma cells. Both Epstein-Barr virus-encoded small RNA and HHV-8 mRNA (T1.1/nut-1) were detected in these lymphoma cells by in situ hybridization. Remarkable retention of the pericardial fluid was observed at the same time that cutaneous lesions grew, and lymphoma cells in the pericardial fluid showed the same phenotype as the cutaneous lymphoma. Chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisone effectively reduced both the cutaneous nodules and pericardial fluid. However, the patient died 4 months after diagnosis because of cytomegalovirus infection. As far as we know, this is the first report of an HHV-8-positive cutaneous lymphoma taking anaplastic large cell morphology. This case suggests the association of AIDS-related anaplastic large cell lymphoma with HHV-8.     

非霍奇金淋巴瘤1例

报告1例以皮肤肿瘤为首发表现的儿童非霍奇金淋巴瘤。患儿女,8岁。右侧鼻翼出现肿块3个月余,伴进行性增大1个月就诊。体格检查示局部淋巴结不增大,系统检查无异常。皮损组织病理检查示真皮内有异形淋巴样细胞浸润,免疫组化染色结果示：CD45RO（＋）,CD20,HMB45,CK,CD30和CD68均阴性,证实为T细胞淋巴瘤。     

Hodgkin lymphoma presenting as generalized pruritus in an adolescent

Pruritus is a common manifestation of Hodgkin lymphoma (HL), and given its high frequency, inclusion of itching as a B symptom of HL has been proposed. We present a 16-year-old adolescent boy with treatment-refractory eczema of 2 years' duration. Physical examination revealed a thin adolescent boy with widespread excoriations, but no eczematous or primary cutaneous lesions were identifiable. Lymph node examination revealed palpably enlarged nodes in the cervical and supraclavicular regions. Laboratory studies revealed leukocytosis and an elevated lactate dehydrogenase level. Diffuse lymphadenopathy was detected on a chest radiograph, and excisional lymph node biopsy revealed HL (nodular sclerosing subtype). The patient was classified as HL stage IIIB (Ann Arbor staging classification) after further evaluation. Chemotherapy was initiated followed by radiation therapy. The patient's pruritus markedly improved within 2 cycles of chemotherapy; however, his HL relapsed and additional salvage combination chemotherapy followed by high-dose chemotherapy and autologous stem cell transplant were required. This case underscores the need for a complete history as well as a careful skin and systemic evaluation in patients presenting with long-term pruritus, including children and adolescents.     

Primary effusion lymphoma presenting as a cutaneous intravascular lymphoma

Primary effusion lymphoma (PEL) is a rare and aggressive lymphoma that arises in the context of immunosuppression and is characterized by co‐infection with Epstein–Barr virus (EBV) and human herpesvirus‐8/Kaposi sarcoma‐associated herpesvirus (HHV‐8/KSHV). It was originally described as arising in body cavity effusions, but presentation as a mass lesion (extracavitary PEL) is now recognized. Here, we describe a case of PEL with an initial presentation as an intravascular lymphoma with associated skin lesions. The patient was a 53‐year‐old man with human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) who presented with fevers, weight loss and skin lesions concerning for Kaposi sarcoma (KS). A skin biopsy revealed no evidence of KS; however, dermal vessels contained large atypical cells that expressed CD31 and plasma cell markers but lacked most B‐ and T‐cell antigens. The atypical cells expressed EBV and HHV‐8. The patient subsequently developed a malignant pleural effusion containing the same neoplastic cell population. The findings in this case highlight the potential for unusual intravascular presentations of PEL in the skin as well as the importance of pursuing microscopic diagnosis of skin lesions in immunosuppressed patients.     

Lymphoepithelioid cell lymphoma (Lennert's lymphoma) presenting as atypical granuloma annulare

We describe a case presenting as atypical granuloma annulare where the underlying diagnosis, confirmed by lymph node biopsy, was lymphoepithelioid cell lymphoma (Lennert's lymphoma). Lennert's lymphoma is a peripheral T-cell lymphoma which may follow a variable course and transform into an aggressive phase. Cutaneous manifestations of this condition have only rarely been reported in the literature. The presence of granulomas in the skin may have either obscured the lymphoma infiltrate or may have reflected a more generalized immune response to the underlying malignancy.     

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 graft-versus-host-like reaction in systemic T-cell lymphoma

We report a case of systemic T-cell lymphoma with cutaneous lesions showing histological features of a cutaneous graft-versus-host-like-reaction. Histology from liver, lymph node and bone marrow showed a malignant T-cell infiltrate. T-cell receptor gene rearrangement studies confirmed the diagnosis. A cutaneous graft-versus-host-like reaction has been reported with disseminated malignancy and one case has been reported with systemic lymphoma. Graft-versus-host disease normally occurs when lymphocytes from an immunocompetent donor are introduced into a histo-incompatible recipient who is incapable of rejecting them. In our patient a similar reaction may have occurred if the lymphoma was composed of cytotoxic cells or if a cell-mediated immune response against the malignant T-cells cross-reacted with epidermal keratinocytes. Alternatively the malignant T-cells could have been functionally active and induced a lichenoid reaction in the skin.     

Cutaneous involvement by colonic extranodal NK/T-cell lymphoma mimicking mycosis fungoides: a case report*

We report a 51-year-old woman with cutaneous involvement by extranodal NK/T-cell lymphoma (TCL) of the colon that microscopically mimicked mycosis fungoides (MF). She had a history of fever of unknown origin for 2 months and then developed multiple erythematous papules on her trunk and extremities. A skin biopsy revealed superficial infiltration by atypical small to medium-sized lymphocytes with epidermotropism and Pautrier collections. Immunohistochemical studies showed expression of CD3 and TIA-1 with lack of expression (double negative) of CD4 and CD8. Initially, we reported the diagnosis as MF, cytotoxic variant. Thereafter, computerized tomography scan incidentally identified a colonic mass. A colonic biopsy revealed infiltration of atypical lymphoid cells with the same morphology and immunophenotype as those found in the skin. Additionally, CD56 and Epstein-Barr virus-encoded RNA in situ hybridization in both skin and colonic biopsies were diffusely positive. Thus, extranodal NK/TCL was diagnosed. Delta T-cell receptor (TCR) gene rearrangement was documented in the skin biopsy by polyacrylamide gel electrophoresis and fluorescence capillary gel electrophoresis methods. There was no TCR gene rearrangement detected in the colonic biopsy. Unfortunately, the patient died within 2 months of diagnosis.