Cutaneous granulomas associated with adult T-cell leukemia/lymphoma

A 59-year-old Japanese woman showed recurrent violaceous, indurated erythemas and papules on the buttocks and extremities for a period of 7 years. The lesions showed histologically epithelioid cell granulomas associated with lymphocytes. After 7 years, the patient developed adult T-cell leukemia (ATL), of the acute type, the course of which was fatal. Immunohistochemical staining of the skin sections of granulomatous lesions showed prominent infiltration of CD25-positive cells. Human T-lymphotropic virus type I proviral DNA and monoclonal T-cell receptor beta gene rearrangement were detected in the skin samples by a retrospectively performed gene analysis. We believe that the patient initially had an unusual cutaneous type of ATL in which granulomas occurred as a host-protective response against ATL progression.     

Erythema multiforme-like eruptions associated with etretinate therapy

Mucocutaneous side-effects from etretinate are common and are usually dose-dependent, whereas hypersensitivity drug eruptions are rare. Two patients are described in whom erythema multiforme-like eruptions appeared several days after oral intake of etretinate. In one patient etretinate rechallenge again produced the skin eruption. A brief review of the less common skin manifestations of etretinate therapy is provided.     

成人T细胞白血病/淋巴瘤的皮肤损害及其诊断意义

皮肤型和隐匿型成人T细胞白血病／淋巴瘤常可出现皮肤损害，可表现为皮肤T细胞淋巴瘤或湿疹样、痒疹样、荨麻疹等皮肤病，亦可作为首发体征。经对症治疗后如皮损加剧或伴全身症状，需做外周血检查和皮肤组织病理学检查，其中包括印片检查；若见异形淋巴细胞，需进一步做免疫组化检测嗜人T淋巴细胞病毒Ⅰ型(HTLV-Ⅰ)抗体，最终确诊需在外周血和(或)罹患组织内检测出HTLV-Ⅰ前病毒DNA。     

Cutaneous T-cell lymphoma with HTLV-I infection: clinical overlap with adult T-cell leukemia/lymphoma

Adult T-cell leukemia/lymphoma (ATLL) is a malignant proliferation of mature helper T lymphocytes,(1) and is caused by human T-lymphotropic virus type I (HTLV-I);(2) an HTLV-I infection endemic in the Caribbean, south-western Japan, South America and Africa.(3,4) Seroepidemiological studies suggest that it is also endemic in Brazil.(5) Although carriers of HTLV-I show polyclonal integration of virus in T lymphocytes, only patients with ATLL of various subtypes show monoclonal integration of HTLV-I in tumor cells.(6,7) Cutaneous T-cell lymphomas (CTCL) are a group of primary cutaneous lymphoproliferative diseases(8) with unknown etiology.(9) The two most common presentations of CTCL are mycosis fungoides (MF) and Sézary syndrome (SS).(10-13) However, both CTCL categories can easily resemble ATLL. Therefore, in HTLV-I endemic areas, differentiation between ATLL and CTCL must be performed, as they have different prognoses and treatment approaches.(14).     

Case of adult T-cell leukemia/lymphoma manifesting marked purpura

We present a case of a 62-year-old woman with marked purpura, first appearing on both legs, then spreading over the whole body, including the face. At presentation, the patient was thought to have Henoch-Schonlein purpura. However, a skin biopsy from a purpuric lesion revealed prominent infiltrations of atypical lymphocytes into the papillary dermis and marked extravasation of erythrocytes through the epidermis and upper dermis. Antibody to human T-lymphotropic virus type 1 (HTLV-1) was present in the serum and samples from skin lesions revealed HTLV-1 proviral DNA integration, as well as a clonal T-cell receptor Cbeta1 gene rearrangement. We therefore diagnosed this case as adult T-cell leukemia/lymphoma (ATL), and the purpuric lesions as ATL-specific. Soon after the initiation of chemotherapy, these purpuric lesions began to resolve with pigmentation.     

Erythema multiforme-like eruption associated with contact dermatitis to cutting oil

A 48-year-old Japanese man developed coalesced erythema multiforme-like lesions on the forearms and the dorsa of hands that were exposed to a cutting oil product. Subsequently, multiple, round erythematous lesions with iris formation occurred on the trunk and four extremities that had no direct contact with the oil. It is suggested that erythema multiforme can be induced via some allergic processes following a contact reaction.     

Adult T-cell leukemia/lymphoma in an adolescent presenting with skin lesions

Abstract:  Adult T-cell leukemia/lymphoma is a T-cell malignancy caused by the human T-cell lymphotropic virus-I. Adult T-cell leukemia/lymphoma is primarily a disease of adults due to the long latency period between initial infection and development of leukemia. We present a case of acute adult T-cell leukemia/lymphoma in an adolescent. Skin lesions had appeared 3 years earlier and were the initial sign of human T-cell lymphotropic virus-I infection and T-cell malignancy. Her disease failed to respond to both intensive chemotherapy and antiviral therapy. Cutaneous lesions are sometimes the initial sign of adult T-cell leukemia/lymphoma and early recognition is imperative.     

Clinicopathological features of cutaneous lesions of adult T-cell leukaemia/ lymphoma

BACKGROUND: Adult T-cell leukaemia/lymphoma (ATLL) is a human malignancy associated with human T-cell leukaemia virus type I (HTLV-I). ATLL frequently involves the skin. OBJECTIVES: To correlate the clinicopathological features and prognosis in patients with ATLL and cutaneous lesions. METHODS: We examined the HTLV-I proviral state and the clinicopathological features of the cutaneous lesions in 80 patients with serum anti-ATL antibody, to clarify the correlation between macroscopic/histopathological findings and prognosis. Southern blot analysis was performed in all cases to detect monoclonal HTLV-I proviral DNA integration. RESULTS: The cutaneous lesions of 46 patients were positive for proviral DNA integration. The median survival time of patients with monoclonal proviral DNA integration in cutaneous lesions was 14 months, which was markedly shorter than that of patients negative for proviral DNA integration (72 months). Of the 46 patients with proviral DNA, 21 had solitary or multiple red nodules (including three with subcutaneous induration), eight had multiple red papules and 17 had erythema. Patients with papules and nodules had poorer prognosis than those with erythema. Histopathologically, the prognosis was poorer in patients with nodular or diffuse infiltration of medium-sized to large lymphoma cells, compared with those with perivascular infiltration of small to medium-sized lymphoma cells. CONCLUSIONS: Our results show a close correlation between clinicopathological features of HTLV-I-associated cutaneous lesions and prognosis.     

A case of adult T-cell lymphoma leukemia with hemophagocytic syndrome

A 62-year-old Japanese woman was admitted to our clinic with virus-associated hemophagocytic syndrome (VAHS) in subcutaneous adult T-cell lymphoma leukemia (ATLL). Bone marrow aspiration showed hypocellularity, histiocytic hyperplasia, and hemophagocytosis. There was serological evidence of chronic cytomegalovirus (CMV) infection. The hemophagocytic syndrome (HPS) initially improved by some treatments, and the patient later experienced remission several times, but the CMV infection persisted. Most cases of non-tumorous HPS in adults are associated with viral or bacterial infection, and underlying diseases in non-tumorous HPS are mostly blood diseases, especially T-cell lymphoma (1, 2), but ATLL is a rare underlying disease in such cases.     

Angiocentric and angiodestructive infiltration of adult T-cell leukemia/lymphoma (ATLL) in the skin. Report of two cases

This report describes the clinical and pathological features of cutaneous lesions observed in a 73-year-old woman with terminal adult T-cell leukemia/lymphoma (ATLL) and a 42-year-old man who had chronic ATLL with persistent nasal stuffiness and rhinorrhea of 2 years' duration. Histology of the skin lesions from these two cases showed large blood vessel (arterial and venous) involvement at the level of the dermosubcutaneous junction, in which angiocentric and angiodestructive infiltration of the tumor cells was prominent and reminiscent of lymphomatoid granulomatosis (LYG) and/or polymorphic reticulosis. ATLL commonly involves the skin. Moreover, it may originate in the skin. The majority of researchers have observed perivascular and periadnexal infiltration, frequent epidermal exocytosis with Pautrier's microabscesses, and the occasional presence of leukemic cells within the vascular lumen; however, vascular involvement like that seen in our cases has not been described before. Our cases suggest that a variety of T-cell malignancies may be angiocentric and angiodestructive in nature, and that so-called LYG and LYG-like malignant lymphomas are not a single disease, but may constitute a heterogeneous group of T-cell malignancies including ATLL.     

Exchange of dominant lymphoid cell clones in a patient with adult T-cell leukemia/lymphoma

We performed a genotypic study on lymphoid cells from a patient with adult T-cell leukemia (ATL). Clonal proliferation of human lymphotropic virus type-1(HTLV-1)-infected helper T-cells was detected in the primary skin tumors, while no clonality of the virus-infected lymphocytes was observed in the peripheral blood. Following intensive chemotherapy, however, we detected the presence of two genotypically different HTLV-1-infected lymphocyte clones in different samples taken from the peripheral blood. These data demonstrated the exchange of the dominant neoplastic clone in the clinical course of ATL, suggesting the possibility that a certain clone among many repertoires of HTLV-1-infected T-cells has a capability of leukemogenesis, instead of the primary tumour cells.