Cases reported "Lymphoma, T-Cell"

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1/22. Necrotic cutaneous lesions induced by hypereosinophilic syndrome secondary to a T-cell lymphoma.

    Cutaneous lesions of hypereosinophilic syndrome (HES) may display various presentations. These are important to recognize to allow early diagnosis. We report an unusual case of a young man with HES secondary to a splenic T-cell lymphoma, revealed by diffuse necrotic cutaneous lesions. Later on, brain and heart infarctions developed, leading to the patient's death. Analysis of skin biopsy specimens showed occluded dermal vessels surrounded by activated eosinophils. Because a complete analysis of hemostasis was unremarkable, these findings suggest that dermal vessel thrombosis was the direct consequence of the migration of activated eosinophils. Cutaneous lesions of HES are protean and probably reflect the differences between the lesional events induced in situ by activated eosinophils.
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2/22. bone marrow necrosis with dyspnea in a patient with malignant lymphoma and plasma levels of thrombomodulin, tumor necrosis factor-alpha, and D-dimer.

    Non-Hodgkin's lymphoma (peripheral T cell, unspecified, clinical stage IIIEA) was diagnosed in a 54-year-old male. He was treated weekly with chemotherapy consisting of pirarubicin hydrochloride, cyclophosphamide, methotrexate, vincristine sulfate, etoposide, and prednisolone. After 6 weeks of treatment in a state of partial remission, he exhibited sudden dyspnea, chest pain, fever, and drowsiness. The patient had received 100 microg/day of granulocyte colony stimulating factor (G-CSF) for 6 days before the onset. Laboratory data showed an elevated lactate dehydrogenase (LDH) level, leukoerythroblastosis in the peripheral blood, and no decrease in the serum haptoglobin level. There were no findings of acute myocardial infarction or pulmonary thromboembolism. bone marrow specimen showed the characteristic features of necrosis without any signs of the involvement of lymphoma cells. No indications of infections were found. This patient was diagnosed as having bone marrow necrosis (BMN) during the recovery phase of bone marrow with G-CSF treatment after chemotherapy for malignant lymphoma. After conservative therapy, inhalation of oxygen and stopping the administration of G-CSF, all clinical symptoms subsided except that the elevation of LDH continued for 1 month. The plasma level of tumor necrosis factor-alpha (TNF-alpha) was high just after the onset of BMN. The thrombomodulin (TM) level was high just before the onset of BMN and continued to be high for 2 weeks. Cross-linked fibrin degradation products (D-dimer) were also high just after the onset of BMN and continued to be high for 3 weeks after the onset. Although dyspnea is a rare symptom of BMN, it is important to rule out in BMN during the recovery phase of bone marrow with G-CSF treatment after chemotherapy for malignant lymphoma. Activated neutrophils in the small vessels of the lung by G-CSF and microthrombi, suggested by the elevation of D-dimer, may participate in the onset of dyspnea, which is a rare symptom of the onset of BMN.
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3/22. A case of inflammatory demyelinating polyradiculoneuropathy associated with T-cell lymphoma.

    Malignant lymphoma may present prominent peripheral nervous system disorders with variable etiologies. We describe a patient who presented with chronic relapsing polyradiculoneuropathy accompanied by right facial nerve palsy. gadolinium enhancement of the right facial nerve and cervical spinal roots was noted on magnetic resonance imaging (MRI). sural nerve biopsy specimens showed mononuclear cell infiltration around the vessels in the epineurium. Histopathological and immunohistochemical investigations of sural nerve specimens revealed perivascular infiltration of lymphocytes with T-cell dominancy. No apparent direct invasion of lymphoma cells was seen. The results of nerve conduction studies, sural nerve biopsy and cerebrospinal fluid examination were suggestive of immune-mediated inflammatory demyelinating neuropathy. The chronic and relapsing fashion and unique radiological findings in our patient expand on the previously reported features of peripheral neuropathy associated with peripheral T-cell lymphoma.
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4/22. Successful treatment with erwinia L-asparaginase for recurrent natural killer/T cell lymphoma.

    We describe a patient with natural killer (NK)/T cell lymphoma who relapsed after autologous peripheral blood stem cell transplantation (auto-PBSCT) and was successfully treated with escherichia coli (E. coli) and erwinia L-asparaginase. A 38-year-old male patient with ulcerated tumor at the left thigh was diagnosed as having nasal type NK/T cell lymphoma on the basis of histopathological and flowcytometric findings of tumor, revealing diffuse infiltration of atypical lymphoid cells into blood vessels and expression of CD7 and CD56 antigens, but not CD3. He had tumor infiltration in the bone marrow and at the right lower lung field. After five cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) therapy, the patient achieved complete remission and received high-dose chemotherapy with auto-PBSCT, although the tumor recurred in the right leg 10 months later. Despite salvage chemotherapy, followed by local irradiation and surgical amputation, a tumor recurred at the left upper gingiva 10 days after. Using E. coli L-asparaginase (6000 U/m2/day), the tumor regressed, fever was alleviated and the serum lactate dehydrogenase decreased to normal range after several days. The asparagine synthetase expression in tumor cells was immunohistochemically negative on paraffin-embedded tissues. Because of the anaphylactoid reaction developing after E. coli L-asparaginase, alternative erwinia L-asparaginase (6000 U/m2/day) was administered, resulting in regression of tumor and fever lysis. L-asparaginase is a promising agent for the treatment of NK/T cell lymphoma.
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5/22. magnetic resonance imaging of lymphomatoid granulomatosis: punctate and linear enhancement preceding hemorrhage.

    lymphomatoid granulomatosis (LG) is an angiocentric and angiodestructive lymphoproliferative disease that mainly involves the lung. Thirty percent of patients with LG have neurological symptoms. We report serial changes in MRI of a patient with LG of the brain. Postcontrast MRI demonstrated multiple punctate and linear areas that preceded hemorrhage, as indicated by hypointensity with surrounding hyperintensity on subsequent T2-weighted images. The diagnostic value of these lesions observed with contrast MR images is discussed. We consider that enhancing areas correspond to walls of small vessels affected by LG.
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6/22. Epstein-Barr virus-associated intravascular large T-cell lymphoma presenting as acute renal failure in a patient with acquired immune deficiency syndrome.

    Intravascular lymphoma (IVL) is a rare neoplasm, recently included as a specific entity in the world health organization classification of lymphoid tumors. Most cases are of B-cell lineage; however, rare cases of T-cell phenotype have been reported. We report a human immunodeficiency virus (HIV)-positive patient who died of acute renal failure in whom IVL was identified at autopsy, predominantly involving the renal interstitial vessels. Immunohistochemical stains revealed a T-cell phenotype, which was confirmed by T-cell receptor gamma gene rearrangement studies. The lymphoma cells showed nuclear Epstein-Barr virus (EBV)-encoded rna transcripts by in situ hybridization, suggesting that EBV might be of etiologic importance in this tumor. The predominant involvement of kidney is unusual. With effective therapy, morbidity and mortality of hiv-1 infection has been substantially reduced, and survival times have been prolonged. However, the relative risk of secondary neoplasms, especially non-Hodgkin's lymphoma (NHL), has increased. Consequently, we conclude that unique types of NHL, such as this case of IVL, may be encountered more frequently in this patient population, and that NHL should be added to the list of differential diagnostic considerations in hiv-1-positive patients who develop acute renal failure.
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7/22. A male with angioimmunoblastic T-cell lymphoma and proliferative glomerulonephritis.

    In this article we present the case report of a 67-year-old male with a nephrotic syndrome due to a proliferative glomerulonephritis, associated with an angioimmunoblastic T-cell lymphoma. Diagnosis was made on an axillary lymph node biopsy and showed expanded T-cell areas with multiple blood vessels, small mature lymphocytes, eosinophils, and plasma cells. A kidney biopsy was suggestive for a proliferative glomerulonephritis with intra- and extracapillary proliferation. Hypercellular glomeruli were seen, as well as multiple floride crescents. Interstitial edema and fibrosis were absent. Immunohistochemical reactions were negative; there was some mesangial reaction with IgM in the glomeruli. Treatment with high-dose corticosteroids was initiated, with clinical improvement, and was immediately followed by therapy with cyclophosphamide, hydroxydaunomycin, vincristine, and prednisone (CHOP), which induced complete remission with a follow-up of 1 year. To our knowledge, the association of angioimmunoblastic T-cell lymphoma and proliferative glomerulonephritis has only been described twice. It concerned elderly men who developed acute renal failure a couple of months after the diagnosis of an angioimmunoblastic T-cell lymphoma. In both, immunoglobulin-containing dense deposits within glomeruli were observed, which was not the case in our patient, where only some mesangial colorization of the IgM in the glomeruli was seen.
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keywords = blood vessel, vessel
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8/22. Lymphoepithelioid cell lymphoma (Lennert's lymphoma). Report of a case with fine needle aspiration cytology.

    BACKGROUND: Lymphoepithelioid cell lymphoma (LCL) is a rare morphologic variant of peripheral T-cell lymphoma. Although their histopathologic and immunohistochemical findings are well known, the cytopathologic features have not been well documented. This report describes the fine needle aspiration cytology (FNAC) findings of a case of LCL. CASE: A 75-year-old woman presented with cervical, supraclavicular, axillary and mediastinal lymphadenopathy. FNAC of a cervical lymph node was performed. The smears contained a polymorphous infiltrate formed by abundant histiocytes disposed singly or in clusters, small and medium-sized to large atypical lymphoid cells and reactive cells, including eosinophils and plasma cells. Isolated capillary-sized vessels also were observed. Histopathologic and immunohistochemical examination confirmed the diagnosis of Lennert's lymphoma. CONCLUSION: Although histopathologic and immunohistochemical studies were required for a definitive diagnosis, the findings of FNAC in this case appeared distinctive and suggested the possibility of LCL.
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9/22. T-cell sinonasal lymphoma presenting as acute orbit with extraocular muscle infiltration.

    We describe a rare case of sinonasal T-cell lymphoma in an 11-year-old boy who presented with a right acute orbit characterized by proptosis, eyelid edema and erythema, limitation of eye movements, and excruciating pain on the right side of his face. Orbital computed tomography showed progressive right extraocular muscle enlargement. One biopsy specimen showed extensive tissue necrosis and an infiltrate of atypical cells with pleomorphic nuclei within the walls of blood vessels. Immunohistochemical studies demonstrated that these cells were positive for leucocyte common antigen (CD45), CD3 cytoplasmic, CD45RO, and terminal deoxynucleotidyl transferase and negative for CD20, CD57, CD56, CD99 and Epstein-Barr virus. Chemotherapy for T-cell non-Hodgkin lymphoma was initiated, but the patient's status deteriorated and the child died of respiratory insufficiency, sepsis, and central nervous system infection.
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keywords = blood vessel, vessel
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10/22. Intravascular T-cell lymphoma with bowel involvement: case report and literature review.

    Intravascular lymphoma (IVL) is a rare form of non-Hodgkin lymphoma characterized by massive proliferation of large, neoplastic cells in small- and medium-sized blood vessels. Most cases of IVL are of B-cell immunophenotype; fewer than 15 cases of T-cell IVL have been reported. A 23-year-old male presented with acute abdominal pain, fever, and tender lower abdomen. pathology at laparotomy revealed infiltration of colonic vessels with large lymphoid cells compatible with IVL. We reviewed all cases of IVL diagnosed at the Queen Elizabeth II Health Sciences Centre in Halifax, nova scotia, from August 1992 to August 2002. A literature review was also performed. Five additional cases of IVL were identified at this institution during a 10-year period. Three patients presented with neurological symptoms, and two with abdominal pain. In 4 of 5 cases, patients died of lymphoma within 3 months of presentation; one patient experienced a 10-month remission. While visceral involvement with IVL is common at autopsy, IVL presenting as an acute abdomen in an immunocompetent patient has not previously been described. Among the 15 cases of T-cell IVL reported in the literature, only two occurred in people under age 30. Given the rarity of T-cell IVL, it is remarkable that three cases of T-cell IVL have been diagnosed at our institution during a 10-year period.
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