Cases reported "Lymphadenitis"

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1/9. Mediastinal histoplasmosis with abscess.

    A mediastinal mass of clinically undetermined nature was found at autopsy to be an enormous abcess due to secondary infection in granulamatous caseating mediastinal nodes caused by Hitosplasma capsulatum. Other complications of mediastinal histoplasmosis are briefly reviewed.
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2/9. Kikuchi's histiocytic necrotizing lymphadenitis. diagnosis by fine needle aspiration.

    Two cases of Kikuchi's histiocytic necrotizing lymphadenitis diagnosed by fine needle aspiration (FNA) of enlarged lymph nodes are reported. The FNA smears contained randomly activated lymphoid cells, necrotic debris, karyorrhectic cells and prominent histiocytes, suggesting the presence of reactive lymph nodes. The true nature of the lesions was evident from the examination of cell block sections prepared from tissue fragments in the aspirates, which preserved the architectural relationships of the different cell types. The same patterns were found in retrospectively and subsequently examined excised lymph nodes from these cases. The differential diagnosis of this entity, which may simulate a malignant lymphoma because of the presence of large numbers of activated lymphoid cells, is discussed and the value of preparing FNA cell blocks is emphasized. Though this rare benign disease may be suspected clinically in the more typical cases, such as young women with cervical lymphadenopathy, fever, neutropenia and otherwise excellent condition, the diagnosis cannot be made without a lymph node biopsy, which FNA may be able to provide in some instances.
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3/9. Infantile multisystem inflammatory disease: a specific syndrome?

    We report two patients with infantile onset of evanescent rash, fever, arthropathy with severe deformities, periosteal changes, chronic meningitis, hydrocephalus, convulsions, developmental delay, papilledema, unusual uveitis, and lymphadenopathy. A few patients with similar findings have been previously reported. Although some similarity exists between findings in these patients and in others with systemic juvenile rheumatoid arthritis, they appear to differ both in regard to the nature and severity of the clinical and pathologic features. We suggest that this group of patients has a separate rheumatic disorder not yet included in the standard classifications of the childhood rheumatic diseases.
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4/9. Chronic idiopathic myelofibrosis. A reversible disease?.

    A patient with chronic idiopathic myelofibrosis was subjected to splenectomy 1 year after diagnosis. As a clinically unexpected finding, lymph node biopsy suggested the presence of non-Hodgkin lymphoma. The patient was subjected to intensive combined cytostatic therapy. In the following months, signs and symptoms of myelofibrosis regressed remarkably. The patient died 31 months after splenectomy in massive gastrointestinal bleeding. At post-mortem, myelofibrosis could not be detected in three bone marrow areas and a regular, fat-containing, hypercellular marrow was present. The nature of the previous lymph noede pathology was reconsidered, and angioimmunoblastic lymphadenopathy was diagnosed.
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5/9. Woringer-Kolopp disease: pagetoid reticulosis.

    A typical clinical and histologic case of so-called 'pagetoid reticulosis' or Woringer-Kolopp disease is reported. This is a chronic cutaneous condition usually with a single focus or at most with a few patches confined to a circumscribed region. A disseminated form has been described as Dupont-Vandaele type, but its relationship to the classic Woringer-Kolopp disease is uncertain. The nosological location of pagetoid reticulosis has not been established, but there are reasons to regard it as a particular and well-defined entity. In relation to the nature of this condition and to the origin of the 'pagetoid cells', it is tempting to consider the possibility that Woringer-Kolopp disease might be a lymphoproliferative epidermotropic disorder, classifiable as a benign form of 'cutaneous T-cell lymphoma', but the early histologic changes are rather indicative of an epidermal origin.
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6/9. Necrotizing lymphadenitis of the neck (Kikuchi's disease).

    Kikuchi's histiocytic necrotizing lymphadenitis is a benign condition originally described in the Japanese literature in 1972. We here describe the clinicopathological features, including fine needle aspirate and bone marrow biopsy, of a case of Kikuchki's disease, which to our knowledge is the first reported from a Scandinavian country. The histopathological features of the enlarged lymph nodes were documented by multiple small necrotic foci showing karyorrhectic debris and haemorrhage. The necrotic foci were surrounded by a mantle of large histiocytic-like cells with vesicular nuclei and clear cytoplasm. The nodal architecture was almost completely effaced and the node infiltrated by a mixture of lymphoid cells of variable size. neutrophils, eosinophils, and plasma cells were very few. The fine needle aspirate biopsy showed a mixture of small dark lymphocytes, larger activated lymphocytes, and many histiocytes. The bone marrow biopsy showed normal haematopoiesis but some large cells with phagocytosed leukocytes, i.e. similar to haemophagocytosis. Kikuchi's disease has a predilection for lymph nodes in the neck of young women, and is usually self-limited and subsides in 1 to 4 months. The patient described in this report received no treatment. Within 5 weeks the fever subsided and the lymph nodes diminished in size. One year later the patient is well and free of disease. We emphasize the benign nature of Kikuchi's disease, and that SLE and malignant lymphoma are the majori differential diagnoses.
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7/9. The use of fine needle aspiration biopsy in patients with multiple lymphadenopathy before open biopsy.

    When a clinician is faced with a patient who presents with lymphadenopathy and in whom the clinical examination and routine investigations are normal, excision biopsy is usually the next step of management to obtain a tissue diagnosis. In a patient who has multiple lymphadenopathy, deciding on which node to biopsy can be difficult because some of the enlarged nodes may not reflect the true disease process. When such a lymph node is biopsied, the actual diagnosis can be delayed or even missed. Fine needle aspiration biopsy has been shown to be an effective tool in the investigation of multiple lymphadenopathy. Many, if not all, of the enlarged lymph nodes can be sampled at one sitting. If this procedure fails to provide a definitive diagnosis, the clinician should then proceed on to an open biopsy. Two cases of multiple lymphadenopathy in which open biopsies failed to reveal the true nature of the disease but subsequent fine needle aspiration biopsies did are presented and discussed.
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8/9. Kikuchi's disease associated with Hashimoto's thyroiditis.

    We describe a unique association of histiocytic necrotizing lymphadenitis (Kikuchi's disease) and chronic lymphocytic (Hashimoto's) thyroiditis in a patient who presented with significant cervical lymphadenopathy and a goiter. This case illustrates the value of lymph node biopsy combined with the fine needle aspiration of the thyroid in determining the nature of the relationship between the goiter and lymphadenopathy.
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9/9. Familial liability to complications after BCG vaccination.

    Adverse reactions induced by vaccination with bacille Calmette-Guerin (BCG) were observed in three infants from two blood-related families. A boy and a girl showed disseminated BCG infection with a fatal course in the former case. The third infant had regional suppurative lymphadenitis. Cases of two other children from the same families are also described: a boy who received bcg vaccine after 5 y of age without complications, and a girl who has not yet been vaccinated. The clinical course and immunological evaluation of the reported cases suggest a congenital immunodeficiency of an unusual transient nature. The role of genetic factors in familial susceptibility to mycobacterial infection also needs to be taken into consideration.
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