Cases reported "Splenomegaly"

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1/30. Encephalopathy associated with haemophagocytic lymphohistiocytosis following rotavirus infection.

    A 2-year-old Japanese boy with a haemophagocytic lymphohistiocytosis (HLH) associated encephalopathy which developed after rotavirus infection is described. The neurological symptoms consisted of coma, seizures and spastic quadriplegia. On therapy with steroids, etoposide and cyclosporin A, the patient recovered without any neurological deficits. The interferon-gamma levels in serum and CSF were elevated at onset of the disease but had returned to normal at the time of clinical remission. brain MRI revealed diffuse white matter abnormalities and parenchymal volume loss. Proton magnetic resonance spectroscopy revealed elevated lactate in the abnormal lesions observed on MRI, indicating that macrophages not exhibiting aerobic metabolism had infiltrated the CNS. At the time of clinical remission, the white matter abnormalities and brain lactate had disappeared. These findings suggested that the neurological symptoms resulted from the overproduction of cytokines by activated T-cells and macrophages. The pathophysiology of a HLH associated encephalopathy was considered to be a local immune response within the CNS, because interferon-gamma can induce the expression of major histocompatibility complex class I and II antigens on glial cells in the CNS. CONCLUSION: Haemophagocytic lymphohistiocytosis associated encephalopathy should be considered early in the differential diagnosis of cases with acute onset neuropathy.
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ranking = 1
keywords = histiocytosis
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2/30. Haemophagocytosis in early congenital syphilis.

    A previously healthy male infant developed hepatosplenomegaly, severe anaemia and thrombocytopenia 5 weeks after birth. Marked haemophagocytosis was present in the bone marrow. A typical maculopapular rash suggested early congenital syphilis. The diagnosis was confirmed by serology and by the presence of untreated syphilis in both parents. CONCLUSION: syphilis needs to be excluded in infants suspected of haemophagocytic lymphohistiocytosis.
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ranking = 0.16666666666667
keywords = histiocytosis
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3/30. Hepatosplenomegalic lipidosis: what unless Gaucher? adult cholesteryl ester storage disease (CESD) with anemia, mesenteric lipodystrophy, increased plasma chitotriosidase activity and a homozygous lysosomal acid lipase -1 exon 8 splice junction mutation.

    A 36-year-old woman was admitted for hepatosplenomegaly and anemia. Bone marrow cytology showed "sea-blue histiocytes", vacuolated macrophages and plasma cells. As primary liver disease, malignancy or hematologic disorders were excluded, and plasma chitotriosidase activity was increased 27-fold over control, the presence of a lysosomal storage disease was suspected. Biochemical analysis of skin fibroblasts revealed normal glucocerebrosidase and sphingomyelinase activity, but lipid analysis showed a more than 15-fold accumulation of cholesterol esters within the cells. The activity of lysosomal acid lipase (LAL) in fibroblast homogenates was decreased to 12% of control subjects. Mutational analysis of the patient's blood showed the homozygous G-->A mutation at position -1 of the exon 8 splice donor site (E8SJM-allele) known for adult cholesteryl ester storage disease (CESD); the polymorphic background was that of the complex haplotype -6Thr, 2Gly, 894 G-->A. Based on clinical, laboratory, cytological and and biochemical findings, CESD can clearly be separated from other more frequent inherited lysosomal storage diseases, e.g. atypical forms of gaucher disease.
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ranking = 223.35165514166
keywords = sea-blue histiocyte, sea-blue, histiocyte
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4/30. Hepatosplenic B-cell lymphoma associated with hemophagocytic syndrome: a case report.

    While T-cell non-Hodgkin's lymphoma (NHL) associated with hemophagocytic syndrome (HPS) has been frequently observed, B-cell NHL associated with HPS has been rarely reported. We report a case of hepatosplenic B-cell lymphoma associated with HPS in a 41-year-old woman who presented with fever of unknown origin. An abdominal CT scan revealed splenomegaly with focal splenic infarction. splenectomy and a liver wedge biopsy showed sinusoidal-pattern infiltration of medium to large tumor cells with positive reaction to a B-lymphocyte marker. Findings on bone marrow examination showed proliferation of histiocytes with avid hemophagocytosis.
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ranking = 0.10429579763786
keywords = histiocyte
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5/30. Progressive nodular histiocytosis accompanied by systemic disorders.

    Progressive nodular histiocytosis is a proliferative process of histiocytes, the main element of which is the dermal dendrocyte. It is considered to form part of a group of histiocytic disorders related to juvenile xanthogranuloma, which also includes xanthoma disseminatum, benign cephalic histiocytosis, spindle cell xanthogranuloma and generalized eruptive histiocytosis; disorders which perhaps represent the spectrum of one single entity. We present the case of a 57-year-old man who, for 26 years, had had a progressively deforming process of cutaneous lesions, with systemic involvement, including chronic myeloid leukaemia, hepatosplenomegaly, hypothyroidism, hyperuricaemia and hypocholesterolaemia. We have not been able to establish precisely the relationship between these features.
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ranking = 1.2709624643045
keywords = histiocytosis, histiocyte
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6/30. Disseminated histoplasmosis with reactive hemophagocytosis: aspiration cytology findings in two cases.

    Two cases of disseminated histoplasmosis associated with reactive hemophagocytic syndrome are described. The clinical presentation was with prolonged unexplained fever and hepatosplenomegaly. On a strong clinical possibility of tuberculosis, antitubercular treatment was initiated in both patients. Lymph node (case 1), splenic (case 2), and bone marrow aspiration, however, showed sheets of proliferating histiocytes, and intracellular and extracellular histoplasma organisms. Aspiration cytology was thus valuable in establishing the final diagnosis. The patients had a fulminant clinical course and died of hemorrhagic shock within 48 hr of hospital admission before specific therapy could be initiated. histoplasmosis can mimic tuberculosis clinically. There is a need for an increased awareness of the clinicopathological spectrum of histoplasmosis, especially its rarer manifestations as hemophagocytic syndrome. In suspected cases, aspirations from the lymph node, liver, and spleen can be performed safely and should be utilized for early diagnosis.
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ranking = 0.10429579763786
keywords = histiocyte
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7/30. Familial splenomegaly: macrophage hypercatabolism of lipoproteins associated with apolipoprotein E mutation [apolipoprotein E (delta149 Leu)].

    splenomegaly with sea-blue histiocytes is not associated with dyslipidemia, except in severe cases of hypertriglyceridemia, tangier disease, or lecithin cholesterol acyltransferase deficiency. We describe two kindreds in which the sea-blue histiocyte syndrome was associated with an apoE variant in the absence of severe dyslipidemia. Both patients presented with mild hypertriglyceridemia and splenomegaly. After splenectomy both patients developed severe hypertriglyceridemia. Pathological evaluation of the spleen revealed the presence of sea-blue histiocytes. A mutation of apoE was demonstrated, with a 3-bp deletion resulting in the loss of a leucine at position 149 in the receptor-binding region of the apoE molecule [apoE (delta149 Leu)]. Although both probands were unrelated, they were of French Canadian ancestry, suggesting the possibility of a founder effect. In summary, we describe two unrelated probands with primary sea-blue histiocytosis who had normal or mildly elevated serum triglyceride concentrations that markedly increased after splenectomy. In addition, we provide evidence linking the syndrome to an inherited dominant mutation in the apoE gene, a 3-bp deletion on the background of an apoE 3 allele that causes a derangement in lipid metabolism and leads to splenomegaly in the absence of severe hypertriglyceridemia.
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ranking = 749.24737263268
keywords = sea-blue histiocyte, sea-blue, histiocytosis, histiocyte
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8/30. Thermophilic multidrug-resistant campylobacter fetus infection with hypersplenism and histiocytic phagocytosis in a patient with acquired immunodeficiency syndrome.

    We present a case report of a patient who had acquired immunodeficiency syndrome (AIDS) and campylobacter fetus infection with a number of unusual clinical and microbiological features. The patient had prominent gastrointestinal symptoms, splenic infarction, splenomegaly with hypersplenism, and hemophagocytic histiocytosis in the spleen and lymph nodes; the organism displayed growth on Campy-selective blood agar, thermotolerance, and resistance to quinolones, piperacillin/tazobactam, ceftazidime, and erythromycin.
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ranking = 0.16666666666667
keywords = histiocytosis
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9/30. Congenital monoblastic leukemia with 9;11 translocation in monozygotic twins : a case report.

    We report an autopsy case of congenital monoblastic leukemia that developed in monozygotic twins. The twin presented with progressive hepatosplenomegaly at 4 weeks after birth. One twin died of massive bleeding and hypovolemic shock before the treatment started. At autopsy, the liver was diffusely enlarged and showed a diffuse whitish discoloration except for the subcapsular and perivenular areas. Microscopic examination disclosed infiltration of histiocyte-like atypical cells along the sinusoids and portal areas of the liver. spleen, lymph nodes and choroid plexus were also infiltrated by the tumor cells. However, bone marrow involvement of the tumor was minimal although multifocal. On immunohistochemical staining, these atypical cells were reactive for CD68 (PGM-1) and lysozyme, suggesting that the tumor cells might have been derived from mono- histiocyte. Cytogenetic study revealed 9;11 translocation, which is frequently associated with acute monoblastic leukemia. To the best of our knowledge, this is the first report of congenital monoblastic leukemia of monozygotic twins in korea.
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ranking = 0.20859159527571
keywords = histiocyte
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10/30. Bilateral subdural effusions related to disease activity in familial hemophagocytic lymphohistiocytosis in an 8-month-old infant.

    An 8-month-old girl had classic features of hemophagocytic lymphohistiocytosis (HLH). A presumptive diagnosis of familial hemophagocytic lymphohistiocytosis was made on the basis of her age and the presence of parental consanguinity. In view of abnormal neurologic findings at presentation, a magnetic resonance imaging scan was performed and showed bilateral proteinaceous subdural effusions. These resolved within 1 week of commencement of chemotherapy for the primary condition. These subdural effusions were the only objective documentations of central nervous system involvement, along with an increased cerebrospinal fluid protein level. We also report other radiologic findings of HLH, which are of use in strengthening this diagnosis in individuals in whom the diagnosis is strongly suspected.
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ranking = 1
keywords = histiocytosis
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