Secondary ALK negative anaplastic large cell lymphoma in a patient with lymphomatoid papulosis of 40 years duration.

A 49 year-old man presented to our clinic. He had a history of lymphomatoid papulosis since childhood. At age 44, regional lymph node manifestation of anaplastic lymphoma kinase (ALK) anaplastic large cell lymphoma (ALCL) developed. Chemotherapy resulted in complete remission of the lymphadenopathy. Four years later, systemic relapse was detected which was refractory to therapy. Histology and immunohistochemistry showed congruent characteristics of multiple skin and lymph node biopsies: diffuse mixed infiltrate with large, anaplastic CD30 cells. Immunophenotype and microscopic morphology suggested a common origin of the different manifestations-however, this could not be proven due to lack of T-cell receptor (TCR) gamma gene rearrangement in most of the samples. The diagnosis of ALK-negative systemic ALCL with cutaneous symptoms was set up at the second flare up, however, the possibility of primary cutaneous ALCL was not excluded steadily. Lymphomatoid papulosis, primary cutaneous ALCL, and systemic ALK ALCL are 3 different entities but the separation of them cannot be solved without distinctive diagnostic tools.

Central nervous system involvement in CD4+/CD56+ hematodermic neoplasm: a report of two cases.

CD4+/CD56+ hematodermic neoplasm, formerly known as blastic NK-cell lymphoma, is an uncommon, aggressive non-Hodgkin's lymphoma with cutaneous, lymph node, and bone marrow involvement at presentation. The disease is characterized by early leukemic phase; however, central nervous system involvement is rarely reported. Herein we describe two cases of CD4+/CD56+ hematodermic neoplasm with meningeal manifestation. Microscopic analysis and flow cytometry of cerebrospinal fluid proved to be diagnostic; however, imaging studies were not informative. These observations call attention to the possibility of central nervous system involvement, which could be more common than expected previously. Authors recommend routine cerebrospinal fluid analysis and prophylactic intrathecal chemotherapy in patients with this highly aggressive disease.

Intravascular large B-cell lymphoma: remission after rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisolone chemotherapy.

Intravascular lymphoma is an uncommon, very aggressive extranodal non-Hodgkin lymphoma that most frequently involves the skin and central nervous system. Most cases are of B-cell origin; T-cell phenotype is extremely rare. Malignant cells proliferate within the lumens of capillaries, arterioles, venules, and small arteries; vascular occlusion is responsible for the clinical signs and symptoms. The prognosis of this high-grade B-cell lymphoma has improved since the introduction of the anti-CD20 monoclonal antibody, rituximab. We describe a case of B-cell intravascular lymphoma successfully treated with rituximab and cyclophosphamide, doxorubicin, vincristine, and prednisolone.

Clinical, histopathological, immunophenotypic and molecular analysis of 60 patients with cutaneous T-cell infiltrates with follow up of indeterminate cases to identify T-cell lymphoma.

Diagnosis of primary cutaneous T-cell lymphomas, especially of mycosis fungoides could be difficult in early stage due to clinical and histopathological similarity to reactive inflammatory dermatoses. To assess diagnostic value of complex histological, immunophenotypic and T-cell receptor gamma gene rearrangement analysis, skin biopsy specimen and peripheral blood samples of 60 patients with suspected cutaneous T-cell lymphoma were analyzed. Our results indicate clear distinction between reactive dermatoses (benign cases, n = 31) and cutaneous T-cell lymphomas (lymphoma cases, n = 17). As definite diagnosis was not obtained in a smaller group of patients (indeterminate cases, n = 12), these patients were followed up. Repeated skin biopsy confirmed mycosis fungoides in 6/12 cases, however in 6/12 patients the diagnosis remained indeterminate. We concluded that careful and complex clinical follow up and repeated histopathological, immunophenotypic and molecular analysis is needed for an appropriate diagnosis in the assessment of early stage mycosis fungoides and uncertain clinical cases.

[Diagnosis, care, and therapeutic possibilities in intravascular B-cell lymphoma--a case report]. / Intravascularis B-sejtes lymphoma kórismézésérol, gondozásáról és kezelési lehetoségeirol egy esetünk kapcsán.

The cutaneous angiotropic lymphoma has a poor prognosis. The diagnosis and the treatment are usually late. The mortality rate is over 80% and the majority of patients die within a year because of the tumorous infiltration of parenchymal organs. The authors report here the medical history and follow up of a still living patient suffering from cutaneous angiotropic lymphoma which has been diagnosed sixty months ago. During the successful treatment course systemic treatment with psoralen ultraviolet A-rays, chlorambucil and cyclophosphamide-doxorubicin-vincristine-methylprednisolone chemotherapy was applied, and for the management of the last relapse, rituximab-cyclophosphamide-doxorubicin-vincristine-methylprednisolone polychemotherapy was used. On the basis of findings in this case a treatment using an anti-CD20 monoclonal antibody could be a promising therapeutic alternative in the management of angiotropic B-cell lymphoma which otherwise considered to be a rare entity.

[Complex histologic, immunophenotypic and molecular genetic investigation in cutaneous T-cell lymphoproliferative diseases]. / Cutan T-sejtes lymphoproliferatív betegségek komplex szövettani, immunhisztokémiai és molekuláris biológiai vizsgálata.

Diagnosis of cutaneous T-cell lymphomas, especially of mycosis fungoides is often difficult in the early stage of the disease, as well as their differentiation from other reactive inflammatory dermatoses. The diagnostic exactness could be improved by the simultaneous histological, immunophenotypic and gene rearrangement analysis of the skin biopsy specimen, and these results together with the clinical findings could help to establish the early diagnosis. Using this complex analysis effective treatment can be started in time and repeated skin biopsies are not always necessary. Authors performed this complex investigation in skin biopsy specimens and peripheral blood of 49 patients with cutaneous T-cell lymphoma and other T-cell lymphoproliferative diseases and estimated the diagnostic value of the T-cell receptor gamma gene rearrangement analysis. Based on the results of the clinical, histological, immunophenotypic and gene rearrangement studies authors could made a clear distinction between the benign dermatoses (19 cases) and malignant cutaneous lymphomas (17 cases). However less than one third of the patients (13 cases) remained unclassified, so definite diagnosis of these cases could be based on the long term follow up and on the results of the repeated skin biopsies.

Large B-cell lymphoma of the leg in a patient with multiple malignant tumours.

A patient who had primary gastric B-cell non-Hodgkin's lymphoma, invasive ductal breast cancer and a basocellular carcinoma of the forehead in her medical history was studied. Three years after polychemotherapy and irradiation of the breast cancer, a rapidly enlarging, ulcerated violaceous tumour developed on the patient's left leg. The tumour was identified by the histopathological, immunohistochemical and immunoglobulin gene rearrangement analyses as a cutaneous large B-cell lymphoma. No signs of extracutaneous involvement were detectable. Despite surgical excision, interferon-alpha2b treatment and chlorambucil + prednisone chemotherapy, a relapse occurred in the previously affected site, whereafter the patient received radiotherapy. She was lost to follow-up, and died approximately 14 months after the surgical intervention without autopsy. We discuss the clinical and histologic features and outcome of the large B-cell lymphoma of the leg, its coincidence with other diseases, and the uncommon occurrence of primary multiple malignant tumours.

Intravascular B-cell lymphoma.

Intravascular (angiotropic) lymphoma is a unique and rare cutaneous lymphoma in which the malignant T or B lymphoid cells proliferate within the lumens of small blood vessels, primarily in the skin and central nervous system. Erythematous, tender nodules, tumors, and telangiectases are the most common skin symptoms in addition to various neurologic signs. Progression of the disease produces secondary organ involvement with variable symptoms and can be fatal. We describe a case of a 74-year-old woman with edematous, infiltrated, orange-like skin with multiple telangiectases, generalized edema, severe weakness, and extremely high values of lactate dehydrogenase. Skin biopsy specimens revealed atypical large cells filling up the lumens of dermal capillaries. Immunohistochemical investigation results identified them as B cells with CD20, CD45, CD79a, Ki-67, and HLA-DR positivity. After administration of diuretics, colchicine, and systemic PUVA therapy, the patient lost her edema, her skin became tender and free of telangiectases, and laboratory alterations normalized. Because of heavy neuralgia in her legs, oral monochemotherapy was introduced with chlorambucil, and now the patient is in remission.