1/157. Lymphomatosis cerebri presenting as a rapidly progressive dementia: clinical, neuroimaging and pathologic findings.Primary central nervous system lymphoma (PCNSL) usually presents with clinical and neuroimaging findings consistent with single or multiple intracranial mass lesions. On cranial magnetic resonance imaging (MRI), such lesions are nearly always contrast enhancing, reflecting disruption of the blood-brain barrier at the site of tumor nodules. We describe 2 cases from the UCLA Medical Center who developed a rapidly progressive dementia due to extensive gray and white matter cerebral lesions involving much of the brain. In the patient who came to autopsy, widely infiltrating, focally necrotic B-cell plasmacytoid lymphoma was noted throughout the cerebral neuraxis. MRI findings in case 2 were consistent with diffuse lymphomatous brain infiltration without mass lesions, which was biopsy proven. We conclude that PCNSL may occur in a diffusely infiltrating form which may occur without MRI evidence of mass lesions or blood-brain barrier compromise. We refer to this entity as 'lymphomatosis cerebri' and add it to the differential diagnosis of a rapidly progressive dementia.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
2/157. Digital clubbing in a patient with progressive mantle cell lymphoma.Digital clubbing with or without hypertrophic osteoarthropathy may occur in a variety of neoplastic diseases. We present the first description of digital clubbing in a patient with progressive mantle cell lymphoma, in the absence of any other apparent etiology. We suggest that clubbing might be a paraneoplastic manifestation in mantle cell lymphoma. The literature on digital clubbing in hematological neoplasms is reviewed.- - - - - - - - - - ranking = 1.5keywords = lymphoma (Clic here for more details about this article) |
3/157. Follicular mycosis fungoides.We describe a patient with follicular mycosis fungoides (MF), a rare folliculotropic variant of cutaneous T-cell lymphoma (CTCL). Follicular involvement in CTCL usually presents clinically as alopecia mucinosa associated histologically with follicular mucinosis. Follicular MF differs from alopecia mucinosa/follicular mucinosis associated with MF with regard to its clinical presentation, histology and, presumably, prognosis. Our patient presented with the characteristic findings of follicular MF, i.e. infiltrated plaques showing numerous enlarged, comedo-like follicular infundibula; histology was dominated by exclusive folliculotropism of atypical lymphocytes sometimes forming follicular Pautrier's microabscesses, and by lack of epidermotropism and follicular mucinosis. Despite photochemotherapy and treatment with oral retinoids and interferon alpha, the patient's follicular MF rapidly developed into a progressive CTCL with large tumorous lesions, but responded to electron beam therapy. The course of our patient's disease confirms the notion that follicular MF may be associated with a worse prognosis than classical MF. However, electron beam irradiation induced remission of follicular MF that was maintained by a combination therapy consisting of extracorporeal photopheresis and interferon alfa.- - - - - - - - - - ranking = 0.25keywords = lymphoma (Clic here for more details about this article) |
4/157. Long-term survival following non-Hodgkin's lymphoma arising in wiskott-aldrich syndrome.wiskott-aldrich syndrome (WAS) is a rare X-linked recessive trait, characterized by thrombocytopenia, eczema, immunodeficiency and a high risk of malignancy, usually leukaemia or lymphoma. Until recently, most patients died before the age of 10 years. A patient with WAS who developed extranodal non-Hodgkin's lymphoma at the age of 16 years is reported. Despite thrombocytopenia at presentation, chemotherapy was well tolerated. There was disease progression after first line chemotherapy and radiotherapy, but the patient responded to second line chemotherapy with cisplatin, vincristine and etoposide. He remains disease free 9 years after completing treatment.- - - - - - - - - - ranking = 1.5keywords = lymphoma (Clic here for more details about this article) |
5/157. Development of IgG lambda multiple myeloma in a patient with cutaneous CD30 anaplastic T-cell lymphoma.We report a patient with an epidermotropic cutaneous T-cell lymphoma which transformed into an anaplastic cutaneous CD30 T-cell lymphoma. Repeated relapses required prolonged systemic puva therapy. Two years after diagnosis, the patient had several episodes of infections of the respiratory tract. serum electrophoresis now revealed significantly reduced polyclonal immunglobulin production and an additional band in the gamma fraction corresponding to IgG lambda monoclonal gammopathy. Thereafter, the patient suffered a pathologic fracture of the dorsolateral 5th rib on the right side and an accumulation of monoclonal plasma cells in the bone marrow confirmed the diagnosis of multiple myeloma (IgG lambda). Accordingly, 6 cycles of cytoreductive chemotherapy (alkeran, decortin) were given. After one year of steady state disease the patient lost weight and bone pain increased while only a few papular eruptions were detectable. radiography showed multiple small osteolytic areas. A few months later he died with signs of bone marrow insufficiency.- - - - - - - - - - ranking = 1.5keywords = lymphoma (Clic here for more details about this article) |
6/157. Invasive squamous cell carcinoma with sporotrichoid metastasis in a patient with cutaneous T cell lymphoma treated with chronic extracorporeal photopheresis.An 83-year-old Caucasian man with cutaneous T-cell lymphoma developed an aggressive squamous cell carcinoma of the left forearm, which recurred and metastasized after Mohs micrographic surgery and systemic chemotherapy with cis-platin and 5-fluorouracil. He was treated with extracorporeal photopheresis, radiation therapy, PUVA photochemotherapy, and interferon therapy for cutaneous T-cell lymphoma. Aggressive squamous cell carcinoma can occur in the setting of extracorporeal photopheresis.- - - - - - - - - - ranking = 1.5keywords = lymphoma (Clic here for more details about this article) |
7/157. High grade, synchronous colon cancers after renal transplantation: were immunosuppressive drugs to blame?Recipients of renal transplants are known to have an increased incidence of cancer, which is believed to be related to the use of immunosuppressive drugs used to prevent rejection. Although the risks of lymphoma and Kaposi's sarcoma are clearly increased in this setting, the association with colon cancer is controversial. We report a 44-yr-old woman, 20 yr post-renal transplant, and with no family history of colorectal cancer or polyps, who was found to have synchronous, poorly differentiated colon cancers associated with extensive abdominal lymph node, bone marrow, and bone (skull) metastasis. The long term immunosuppressive drugs that she had received may have been an important factor in her tumor development and/or progression. Our case and literature review suggest a possible mild, increased risk of colon cancer development in patients after renal transplantation.- - - - - - - - - - ranking = 0.25keywords = lymphoma (Clic here for more details about this article) |
8/157. Reversible methotrexate associated lymphoproliferative disease evolving into Hodgkin's disease.We describe a case of nodular sclerosing Hodgkin's disease (NSHD) developing in a 61-year-old woman with seropositive rheumatoid arthritis treated with oral methotrexate (MTX) 5 to 15 mg/week for 5 years. Computed tomography (CT) of the abdomen revealed splenomegaly and marked abdominal and retroperitoneal lymphadenopathy. MTX was discontinued; several weeks later prednisone 10 mg/day was added to control worsening polyarthralgia. Repeat CT at 3 months showed almost complete regression of the splenomegaly and lymphadenopathy. However, CT studies at 10 months showed asymptomatic progression of lymphadenopathy, which on biopsy revealed NSHD. patients with apparently reversible MTX associated lymphoproliferative disorder require periodic monitoring for asymptomatic development of malignant lymphoma.- - - - - - - - - - ranking = 0.25keywords = lymphoma (Clic here for more details about this article) |
9/157. Blastic/blastoid transformation of follicular lymphoma: immunohistologic and molecular analyses of five cases.Progression of follicular lymphoma to a higher-grade malignancy frequently heralds a poor prognosis. Clinical transformation is variably accompanied by a spectrum of histologic changes characterized by alteration in growth and cytology. Although several cytogenetic events and potential oncogenes have been documented in this progression, the underlying molecular mechanisms are largely unknown. We present five patients with an unusual histologic transformation of follicular lymphoma manifested by blastic/blastoid morphology. This transformation is histologically distinct from other types of transformation of follicular lymphoma. All five cases exhibited the t(14;18) translocation and expressed the BCL-2 protein. In addition, two of the five patients showed increased levels of the p53 protein within neoplastic cells implicating a possible role for this oncogene in blastic/blastoid transformation. The lack of BCL-1 and myeloid antigens by immunohistochemistry and flow cytometry studies served to distinguish blastic/blastoid transformation of follicular lymphoma from its morphologic mimics. This distinction is clinically important because lymphoblastic and myeloid leukemias require significantly different therapeutic modalities and show better prognosis. Moreover, the lack of Epstein-Barr virus-specific mRNA suggests that this virus is unlikely to participate in blastic/blastoid transformation of follicular lymphoma.- - - - - - - - - - ranking = 2.25keywords = lymphoma (Clic here for more details about this article) |
10/157. Coexistence of two distinct cell populations (CD56( )TcRgammadelta( ) and CD56( )TcRgammadelta(-)) in a case of aggressive CD56( ) lymphoma/leukemia.BACKGROUND AND OBJECTIVE: Large granular lymphocytes derive from two major lineages: one expressing the CD3 surface antigen (t-lymphocytes), and the other lacking this marker (NK-cells). Although developmental overlaps between natural killer cells and T-cells have been described, malignancies derived from these two cell types are considered as distinct lymphoid disorders. DESIGN AND methods: We report the case of a 30-year old man affected by a lymphoma/leukemia syndrome presenting with hepatosplenic lymphoma which rapidly transformed into aggressive NK-leukemia. Extensive flow cytometry studies and molecular analysis were repeated during the course of the disease, and showed an unexpected changing pattern. RESULTS: At diagnosis, flow cytometry analysis showed the co-existence of two cell populations, one CD56( ), CD3( ), TcRgd( ), and the other CD56( ), CD3(-) and TcRgd(-). Molecular analysis showed that the TcR genes had the same clonally rearranged pattern involving b, g and d genes in both populations. At disease relapse and during the terminal refractory phase, only CD3(-) cells were present. INTERPRETATION AND CONCLUSIONS: This is an unusual case of CD56( ) aggressive lymphoma/leukemia characterized by the clonal expansion of two phenotypically different cell populations, variably balanced during the course of the disease. The presence of the same TcR genomic rearrangement suggests the origin from a common progenitor able to differentiate along both T- and NK-pathways.- - - - - - - - - - ranking = 1.75keywords = lymphoma (Clic here for more details about this article) |
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