Cases reported "Fever of Unknown Origin"

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1/35. Angiotropic large cell lymphoma presenting as fever of unknown origin.

    BACKGROUND: Fever has never before been described as the chief complaint and major finding in angiotropic large cell lymphoma (ALCL). ALCL is a rare and usually fatal intravascular tumor characterized by a widespread proliferation of malignant mononuclear cells within vessels of small caliber, causing their blockage. The majority present as high-grade, B-cell lymphomas with a predilection for the central nervous system and the skin. CASE REPORT: We report a 61-year-old woman who presented with a fever of unknown origin (FUO) that lasted 9 weeks from onset to death. To our knowledge, this is the first case of ALCL to present solely as a FUO, and the second case of ALCL to be diagnosed by muscle biopsy. CONCLUSION: We suggest that this rare malignancy (ALCL) be considered in the differential diagnosis of FUO.
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2/35. A patient with bilateral primary adrenal lymphoma, presenting with fever of unknown origin and achieving long-term disease-free survival after resection and chemotherapy.

    Primary adrenal lymphoma is extremely rare. We describe a 64-year-old female patient who presented with fever of unknown origin. Imaging studies demonstrated bilateral bulky adrenal masses. She underwent bilateral adrenalectomy and the pathological diagnosis was large cell immunoblastic (B-cell) lymphoma. She received adjuvant combination chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone in the following 6 months. She has been relapse free for 52 months. To the best of our knowledge, this case has the longest disease-free survival among those reported. The present case indicated that primary adrenal lymphoma should be included in the differential diagnosis of fever of unknown origin and/or suprarenal mass. Chemotherapy following surgical resection may be considered the treatment of choice.
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3/35. An aggressive intrasinusoidal lymphoma presenting with marked systemic disturbance but normal imaging studies.

    A patient with an aggressive intrasinusoidal non-Hodgkins lymphoma, presenting with marked systemic disturbance but only a mildly raised alkaline phosphatase as a localising sign is described. All imaging studies of the liver were normal and the diagnosis was delayed until a percutaneous liver biopsy was performed. Once diagnosed, the patient responded extremely well to conventional anti-lymphoma chemotherapy.
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4/35. An unusual case of pyrexia of unknown origin with cervical lymphadenopathy.

    Kikuchi's disease is usually a self limiting illness characterised by pyrexia, neutropenia, and cervical lymphadenopathy particularly in young women of Asian descent. This often leads to an initial misdiagnosis of lymphoma. A case of a young Asian woman who presented with pyrexia of unknown origin is described.
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5/35. Tuberculous gumma (cutaneous metastatic tuberculous abscess) with underlying lymphoma.

    Cutaneous tuberculosis is an infrequent first sign of disseminated tuberculosis. We describe a patient with 2 cutaneous ulcerations that grew mycobacterium tuberculosis. Despite an initial response to antimycobacterial therapy, the fever relapsed. After several months, biopsy of a single cervical lymph node showed a T cell-rich B cell lymphoma. Our patient had metastatic tuberculous abscesses (tuberculous gummas), which are secondary to disseminated tuberculosis, and an underlying occult lymphoma, both believed to be sequentially presenting as a fever of unknown origin.
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6/35. Systemic antineutrophil cytoplasmic antibody vasculitis associated with lymphoid neoplasia.

    Two cases of systemic antineutrophil cytoplasmic antibody (ANCA) vasculitis in the setting of chronic lymphocytic leukaemia and angioimmunoblastic lymphadenopathy type T cell lymphoma are reported. The two patients had fever of unknown origin associated with cutaneous vasculitis and "pulmonary-renal syndrome" with alveolar haemorrhage. Despite anti-infectious treatments, steroids, and chemotherapy, the vasculitis had a fatal paraneoplastic course in several weeks. When infection is excluded in patients with malignancy, atypical features should be promptly investigated for systemic vasculitis, and an ANCA test performed.
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7/35. Pituitary lymphoma presenting as fever of unknown origin.

    An 86-yr-old woman presented with fever of unknown origin. When laboratory evaluation revealed partial hypopituitarism, a magnetic resonance imaging scan of the head was performed and revealed a sellar mass consistent with a pituitary adenoma. Only after other possible etiologies for fever were excluded did she undergo transsphenoidal resection of the sellar mass, which proved to be a B-cell lymphoma. Primary central nervous system lymphoma of the pituitary region is a rare cause of a sellar mass, and this is the first reported case of pituitary lymphoma whose presenting manifestation was fever of unknown origin. Several disease processes can manifest themselves as fever and a sellar mass, including lymphomas. In our case, only surgical biopsy could make a diagnosis and distinguish this process from the more common pituitary adenoma.
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8/35. Positively labeled white blood cell scan with eosinophilia and absence of infection.

    PURPOSE: The authors describe the variability of Tc-99m exametazime-labeled leukocyte distribution as a function of the relative frequency of white cell types in the labeled blood. MATERIALS AND methods: A 76-year-old man who was hospitalized with fever and possible postoperative osteomyelitis underwent scintigraphic imaging with Tc-99m exametazime-labeled leukocytes. RESULTS: The white cell scan excluded any discrete focus of infection and revealed diffuse involvement of the lymph nodes and skin. The pathologic diagnosis was angioimmunoblastic T-cell lymphoma. The atypical infiltrates seen on the white cell scan can be explained by the severe eosinophilic blood count on the day of leukocyte labeling (total leukocyte count: 8,100 cells/microl with 63% neutrophils, 8.9% lymphocytes, and 22.2% eosinophils). CONCLUSION: In the labeling of the leukocyte moiety, a higher presence of any leukocyte subpopulation will modify the biodistribution and thus the image interpretation.
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9/35. Long-lasting fever of unknown origin preceding the diagnosis of intravascular lymphomatosis: a further case stimulates some remarks.

    Neurological and skin involvements usually dominate the clinical presentation of intravascular lymphomatosis (IL), while fever is the most frequent general sign. However, an onset only characterized by fever of unknown origin (FUO) has been rarely reported. We would like to describe a further case of IL, which presented a long-lasting FUO before the diagnosis. At admission, physical examination detected hepatosplenomegaly without lymph nodes enlargement or dermatological or neurological abnormalities. Significant laboratory data included severe anemia, leukopenia, thrombocytopenia, and increased serum LDH. Moreover, a chest CT evidenced bilateral multiple pulmonary infiltrates and pleural effusion. After the development of proteinuria, a diagnosis of large B-cell intravascular lymphoma was made with a renal biopsy 10 months after the onset of the clinical manifestations. So far, more than 100 cases of IL have been reported and the diagnosis often turned out to be difficult, as clinical signs did not point to a lymphoproliferative disorder. This report confirms that FUO is not only frequently associated with IL but that it even marks the real onset of the disease. We are then tempted to conclude that undiagnosed fever is not so rare in IL and if we call it FUO, it is only because diagnosis is necessarily elusive and hence time-consuming.
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10/35. Hodgkin's lymphoma as a cause of fever of unknown origin in hiv infection.

    The risk of Hodgkin's lymphoma is increased in hiv-infected patients. In these patients Hodgkin's lymphoma is an aggressive disease with poor clinical outcome. Complete remission and survival rates are far below that reported in hiv-uninfected population. Since the widespread use of highly active antiretroviral therapy, malignancies are the major cause of mortality in hiv-infected patients. We report a case of a 29-year-old hiv-positive male who presented with fever of unknown origin, pancytopenia, and hemophagocytic syndrome. The diagnosis of Hodgkin's lymphoma with bone marrow involvement was made on bone marrow biopsy, 5 months after the onset of fever. The patient was treated with chemotherapy and achieved a complete remission. Relapse occurred 28 months later and he died 31 months after initial diagnosis. Prolonged fever is frequently observed in hiv-infected patients and could represent a diagnostic challenge. Infectious diseases are the most common causes, however lymphomas are the third most common cause of fever of unknown origin in hiv disease. This case illustrates the difficulties in accurately diagnosing Hodgkin's lymphoma in patients presenting with prolonged, unexplained fever, and emphasizes the use of bone marrow biopsy to confirm Hodgkin's lymphoma, particularly if cytopenia and hemophagocytic syndrome are present.
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