Cases reported "Histiocytosis"

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1/119. Evidence for a hybrid macrophage phenotype in erythrophagocytic histiocytosis.

    PURPOSE: The phenotype of the proliferating cells in two patients with erythrophagocytic histiocytosis is described. These 6- and 18-month-old female patients presented with fever, anemia, hepatosplenomegaly, and lymphadenopathy. MATERIALS AND methods: Clinical histories were reviewed, and pathological specimens of both patients were studied by histology, and electron microscopy/immunohistochemistry using antibodies against macrophage and Langerhans cell (LC) antigens. RESULTS: histology revealed prominent erythrophagocytosis of proliferating histiocytes. By immunohistochemistry, conventional macrophage (HAM-56, alpha 1-antitrypsin, alpha 1-antichymotrypsin, lisozyme, CD68, and alpha-subunit of S-100 protein) and LC (CD1a and S-100 protein) markers were positive, as well as double labeling for CD1a and alpha 1-antichymotrypsin, in a majority of proliferating cells. Ultrastructural examination revealed Birbeck granules and prominent phagolysosomes frequently in the same cell. CONCLUSIONS: The hybrid ultrastructural and immunohistochemical phenotype between phagocytic macrophage and LC of proliferating histiocytes supports the common origin of these different histiocyte subtypes. This unusual phenotype might be the expression of the proliferating (hybrid) precursor or be the effect of unknown stimuli. Additional cases of childhood erythrophagocytic histiocytosis should be studied with immunophenotyping and ultrastructure to determine whether the hybrid phenotype represents a specific entity or an epiphenomenon.
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keywords = histiocytosis, histiocyte
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2/119. Multicentric reticulohistiocytosis in a child.

    A case of multicentric reticulohistiocytosis in an 8-year-old girl, which is a diagnosis rarely seen in children, is presented. Multicentric reticulohistiocytosis is a disorder of unknown aetiology, predominantly affecting the joints, skin and mucosa. Joint symptoms, but not cutaneous lesions, have improved with treatment with methotrexate.
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ranking = 0.99408523702346
keywords = histiocytosis
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3/119. Reactive angioendotheliomatosis or intravascular histiocytosis? An immunohistochemical and ultrastructural study in two cases of intravascular histiocytic cell proliferation.

    Two elderly women with complex medical histories presented with erythematous patches, in one case involving the face and forearms, and in the other both elbows. Punch biopsies from both patients revealed intravascular proliferations of medium-sized and large cells with luminal occlusion typical of angioendotheliomatosis. Immunostaining did not show either lymphocytic or endothelial cell antigens but was consistent with a histiocytic differentiation of the intravascular cells in both cases, and was further substantiated by ultrastructural examination in one case. One patient received a course of cyclophosphamide therapy over 15 days. skin lesions faded but did not disappear. The patient died 10 months later from cardiac and renal failure, which was most probably unrelated to the skin lesions. In the other case, lesions diminished but did not entirely resolve with treatment with low doses of oral prednisone. Angioendotheliomatosis can be divided into a malignant variant, which is an angiotropic lymphoma mostly of B-cell phenotype, and a benign, reactive variant, which is characterized by a proliferation of cells expressing endothelial cell markers. Only one case of angioendotheliomatosis with cells of histiocytic differentiation has been published previously under the name of intravascular histiocytosis. Our cases are very similar to the latter. The question arises as to whether intravascular histiocytic cell proliferation is a neoplastic proliferation of histiocytes or an early stage of classic reactive angioendotheliomatosis representing the residual cells associated with organization of microthrombi, which will be later followed by endothelial cell proliferation.
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ranking = 0.83037595184506
keywords = histiocytosis, histiocyte
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4/119. Cytophagic histiocytic panniculitis improved by combined CHOP and cyclosporin A treatment.

    In a 31-year-old Japanese man with cytophagic histiocytic panniculitis (CHP) remission was achieved by a combination of combined chemotherapy CHOP and cyclosporin A treatment. He was admitted to our hospital in January 1994 with recurrent high fever of 40.2 degrees C and tender and violaceous subcutaneous nodules on his trunk, arms and legs. He developed pancytopenia, hemorrhagic diathesis, liver dysfunction. Histological examination of the biopsied subcutaneous nodule revealed a lobular panniculitis with fat necrosis and a massive infiltration of histiocytes phagocytosing nuclear debris. He was treated initially with 40 mg/day prednisolone. However, following a reduction in prednisolone dosage, his symptoms reappeared. CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) therapy was then initiated. Three courses of CHOP treatment alleviated his symptoms and cyclosporin A was used to maintain his condition for 15 months. His medication was then discontinued and he has been in complete remission for 10 months. Combined treatment of cyclosporin A and CHOP combined chemotherapy was shown to be effective for this patient with severe CHP.
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ranking = 0.0019715876588461
keywords = histiocyte
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5/119. erdheim-chester disease: a case report.

    A 42-year-old man with erdheim-chester disease (EC) is presented. This is the first case of this disease reported in korea. The patient complained of knee pain and plain roentgenogram of the bilateral legs revealed diffusely increased density, coarsened trabecular pattern, and cortical thickening in the diaphysis, and metaphysis as well as epiphysis. magnetic resonance imaging revealed that the lesions showed low signal intensity on T1-weighted images and heterogeneously low and high signal intensity on T2-weighted images. Histological examination of the biopsy specimen showed a xanthogranulomatous lesion consisting aggregations of foamy histiocytes and Touton-type giant cells. Immunohistochemical staining showed positive reaction to anti-S-100 and lysozyme in the cytoplasm of the giant cells.
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ranking = 0.0019715876588461
keywords = histiocyte
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6/119. Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy.

    Symptomatic involvement of the gastrointestinal (GI) tract as a prominent symptom in Langerhans' cell histiocytosis (LCH) is uncommon, occurring in less than 1 to 5% of all cases, even when the disease is in its disseminated form. Up to now, there have been reports of 18 cases of LCH with GI manifestations, including our 2 cases, with diarrhea (77.7%), protein-losing enteropathy (33.3%) and bloody stool being the most frequent findings. The authors present two patients with severe diarrhea and refractory hypoalbuminemia, and with the protein-losing enteropathy documented by Cr51-labeled albumin studies. A review of the literature indicated that the presence of GI symptoms is often associated with systemic disease as well as with poor prognosis, mainly under 2 years of age. radioisotopes are useful for documenting protein loss in several diseases with high specificity and sensitivity, and their utilization in the cases reviewed here permitted diagnoses in 6 children, as well as improved therapeutic management.
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ranking = 0.82840436418622
keywords = histiocytosis
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7/119. A patient with diabetes insipidus and periorbital swellings; erdheim-chester disease.

    erdheim-chester disease is a rare multisystem disease in which a progressive xanthogranulomatous infiltration of several tissues can be seen. We describe a woman, known to have diabetes insipidus for ten years, with periorbital, retroperitoneal, mediastinal, axillar and inguinal involvement. On histological examination a granulomatous infiltration of fatty tissue and striated muscle was seen, consisting of Touton giant cells, histiocytes with foamy cytoplasm and lymphocytes. Immunohistochemical staining with CD-1a and S-100 was negative and on electron microscopy no Langerhans granules were seen. These findings led to the diagnosis of erdheim-chester disease. She had a good response on steroids. Because of some similar clinical features of Langerhans cell histiocytosis and erdheim-chester disease, a histiocyte disorder seems the most probable cause.
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ranking = 0.16962404815494
keywords = histiocytosis, histiocyte
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8/119. listeria monocytogenes and recurrent mycobacterial infections in a child with complete interferon-gamma-receptor (IFNgammaR1) deficiency: mutational analysis and evaluation of therapeutic options.

    We describe the history of a girl with interferon-gamma-receptor (IFNgammaR1) deficiency and studies performed to identify the molecular and clinical characteristics of this recently discovered disorder. This is the first report of a child from Northern europe with IFNgammaR1 deficiency. The patient, now 7 years old, first presented with swelling and reddening at the Bacille Calmette-Guerin (BCG) vaccination site, swelling of lymph nodes, hepatomegaly, and an unusually severe varicella rash at the age of 4 months. At that time, she was diagnosed with BCG histiocytosis without typical granuloma formation and was treated with antituberculous agents. During the clinical course of her illness, several different types of atypical mycobacteria and (for the first time in an IFNgammaR1-deficient patient) listeria monocytogenes were detected. Flow cytometric analysis showed that the patient's monocytes could not bind a monoclonal antibody specific for the IFNgamma-receptor. Our analysis of mRNA derived from the alpha-chain (IFNgammaR1) gene of this receptor revealed deletions of 173 bp and 4 bp in cDNA sequences originating from individual alleles. The 173 bp deletion was located between nucleotide positions 200 and 372, exactly matching those of exon 3, and the 4 bp deletion was located between nucleotide positions 561 and 564 of the coding region of the cDNA. Analysis of genomic dna revealed the presence of a G to T transition at the 5'end of the splice consensus sequence of intron 3, which explains the absence of exon 3. The other allele carried the 4-base-pair deletion (ACTC) at nucleotide positions 15-18 of exon 5. Twelve months after an allogeneic bone marrow transplantation, the patient had clinically improved.
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ranking = 0.16568087283724
keywords = histiocytosis
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9/119. Aspecific inflammatory lesion (histiocytosis?) simulating intramedullary astrocytoma. Case report.

    We report an exceptional case of aspecific inflammatory lesion of the thoracic spinal cord simulating an intramedullary glial tumor. Patient history was characterized by progressive spastic paraparesis with urinary incontinence; MR imaging (T4-T5) showed an enhancing intrinsic mass lesion. Myelotomy enabled partial resection of grayish astrocytoma-like tissue. Only light microscope examination was possible and disclosed aspecific inflammatory tissue composed of eosinophils, lymphocytes and histiocytes. After the operation, the patient improved promptly and no further therapy was administered. Control MRI after four months, one year and two years showed complete disappearance of the intramedullary mass lesion and the patient remained clinically stable with no other signs of disease. Since electron microscopic as well as immunohistochemical studies were not available, a definitive histological diagnosis was not possible. However on the basis of some clinical similarities with cases of isolated histiocytosis X of the CNS reported in the literature, we suggest that a diagnosis of isolated intramedullary eosinophilic granuloma could be reasonable.
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ranking = 0.83037595184506
keywords = histiocytosis, histiocyte
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10/119. Histiocytic lesion mimicking intrinsic brainstem neoplasm. Case report.

    This 10-year-old girl presented with a 1-month history of progressive bulbar palsy and a solitary enhancing mass originating within the floor of the fourth ventricle. Results of initial imaging studies and presentation were suggestive of neoplasia. Subtotal resection was performed and pathological examination revealed the mass to be a histiocytic lesion, with no evidence of a glioma. The patient had no other stigmata of histiocytosis and was treated with steroid medications, resulting in prolonged resolution of the lesion. This case demonstrates that for discrete brainstem lesions the differential diagnosis includes entities other than glioma for which treatment is available. biopsy sampling should be considered when technically feasible.
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ranking = 0.16568087283724
keywords = histiocytosis
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