Follicular dendritic reticulum cell tumor mimicking inflammatory pseudotumor of the spleen.

In the course of a routine clinical check up of the 54 year old male a splenic well circumscribed tumor like mass of 12 cm in diameter was discovered. Splenectomy with removal of splenic hilar lymph nodes and liver wedge biopsy were performed. Four years later the patient is symptom free. In the removed spleen the tumor like lesion showed a pattern consistent with the diagnosis of inflammatory pseudotumor. However, besides lymphocytes, plasma cells, macrophages, eosinophils and myofibroblasts a high number of slightly polymorphic, frequently binucleated cells positive for CD21 and CD23 were seen. These cells which were scattered or formed smaller or larger groups and fascicles were considered to represent follicular dendritic reticulum cells (FDRCs) and the lesion a FDRC tumor. Flow cytometric DNA ploidy analysis showed a hyperdiploid cell population inside the tumor like lesion. Besides FDRC tumors of high and of intermediate malignancy the present case may represent a low grade type of moderate proliferation activity. The FDRCs of the lesion and a few smaller spindle cells were EBER positive indicative of the presence of EBV. No EBER positive cells were seen in the uninvolved spleen.

Genotypic analysis in primary systemic anaplastic large cell lymphoma.

This report presents an experience of polymerase chain reaction (PCR) analysis of T-cell receptor g- and bgene (TCR g, TCR b), and immunoglobulin heavy chain (IgH) gene rearrangements in 9 cases of primary systemic anaplastic large cell lymphoma. We showed 2 clonal IgH, 2 TCR g, 1 TCR b rearrangements. The genotype was B/T-cell in 1, T-cell in 1, B-cell in 1 and null cell-type in 6 cases. We used reverse transcriptase PCR (RT-PCR) to detect t(2;5)(p23;q35) and t(1;2)(q25;p23) translocations. T(2;5) translocation was demonstrated in 2 cases, there was no t(1;2) translocation. In most cases the molecular genetic results were found to be in agreement with immunophenotypic data.

[Molecular genetic methods in anaplastic large cell lymphoma]. / Molekuláris genetikai vizsgálatok anapláziás nagysejtes lymphomában.

INTRODUCTION: Anaplastic large cell lymphoma belongs to a relatively good prognostic group of high grade non-Hodgkin lymphomas. Molecular genetic methods can provide great help in the differential diagnosis of the disease. AIMS: Sixteen frozen lymph node samples of CD30 positive lymphomas with anaplastic morphology and 1 bone marrow aspiration were analysed with polymerase chain reaction according to T-cell receptor gene and immunglobulin heavy chain gene rearrangement. T(2;5)(p23;q35), t(1;2)(q25;p23) chromosomal translocation was also investigated with reverse transcriptase polymerase chain reaction. RESULTS: Seven cases showed null-, 3 T-, 4 B- and 2 T/B hibrid genotype. Two cases of t(2;5), whereas no t(1;2) translocation was demonstrated. CONCLUSIONS: Molecular genetic results correlated with the results of immunhistochemistry and can give further help to divide the cases into smaller, new prognostic subgroups.

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.