Cases reported "Hematologic Diseases"

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1/10. A case of lung myelolipomatosis in a patient with bronchial carcinoid.

    Myelolipomas are very rare benign tumours composed of an admixture of mature adipose tissue and normal haematopoietic cells. Although they are most commonly found in the adrenal glands, extra-adrenal myelolipomas are documented. We described a case of myelolipoma arising in the lung in a 52-year-old man. The lesion was found incidentally in association with a carcinoid. To our knowledge, this is the second instance of this neoplasm presenting as a lung lesion, and the first case associated with bronchial carcinoid. Pathogenesis and aetiology of myelolipomas are referred to in this paper with special regard to the clinical and pathological findings.
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ranking = 1
keywords = neoplasm
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2/10. Haematological disease in siblings with rothmund-thomson syndrome.

    We report two siblings with rothmund-thomson syndrome (RTS); the older sister died of acute myeloblastic leukaemia and the younger sister has a slowly progressive leucopenia. Her prognosis is guarded in view of the increased incidence of neoplasms in this condition. More than 200 cases of RTS have now been reported worldwide.1 This is the first report of siblings with haematological disease and RTS.
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ranking = 1
keywords = neoplasm
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3/10. Clinically significant cardiac infiltration in acute leukemia, lymphocytic lymphoma, and plasma cell myeloma.

    Cardiac infiltration by hematologic neoplasms leading to clinically significant cardiovascular disease is rare. Three such cases are described in this report, and it is suggested that rare manifestations of hematologic neoplasms may become more common in the future since these diseases are more amenable to therapy than heretofore. Cardiac involvement with hematologic neoplasms is of more than academic interest since this complication is likely to respond to radiotherapy.
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ranking = 3
keywords = neoplasm
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4/10. Left-sided splenorenal fusion with marked extramedullary hematopoiesis and concurrent lithium toxicity. A case report and review of the literature.

    Occasionally, heterotopic splenic tissue can occur in the renal fossa secondary to splenosis following splenic trauma or splenectomy. More rarely, it can represent a developmental anomaly secondary to the fusion of splenic and renal tissues. Splenorenal fusion can present as a renal mass, mimicking primary or secondary renal neoplasms on imaging studies, and patients can also present with symptoms of hypersplenism (anemia). We report a case of splenorenal fusion in a 51-year-old woman who initially presented with lithium toxicity, anemia, thrombocytosis, and a large renal mass that mimicked a primary renal neoplasm. The possible embryologic origin of splenorenal fusion, effects of lithium toxicity, and utility of various imaging modalities are discussed. The literature on renal heterotopic splenic tissue is also briefly reviewed.
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ranking = 2
keywords = neoplasm
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5/10. serum parathyroid hormone-related protein concentrations in patients with hematologic malignancies or solid tumors.

    The clinical significance of parathyroid hormone-related protein in humoral hypercalcemia of malignancy was investigated by determining the serum parathyroid hormone-related protein concentrations in 167 normal subjects, 56 patients with hematologic malignancy and 144 patients with solid tumor. serum parathyroid hormone-related protein was measured with a radioimmunoassay kit that recognizes the C-terminal portion of the molecule. The serum parathyroid hormone-related protein concentrations were 20.2-50.8 pmol/l (mean /- 2 SD) in normal subjects, and were elevated in 80% of the patients with malignancies with hypercalcemia, including squamous cell carcinoma and adult T cell leukemia. Moreover, two cases of B cell non-Hodgkin's lymphoma with hypercalcemia had high serum parathyroid hormone-related protein concentrations, which varied in parallel with the tumor size during the clinical course. Of 136 patients with solid tumors with normocalcemia, the serum parathyroid hormone-related protein concentration was slightly elevated in only 5.1%, all of whom were at an advanced stage. These data indicate that determination of the serum parathyroid hormone-related protein concentration is useful for differential diagnosis of humoral hypercalcemia of malignancy and prediction of its development.
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ranking = 9.488098960788
keywords = malignancy
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6/10. Primary lymph node presentation of angiocentric lymphoma associated with features of a hemophagocytic syndrome.

    The spectrum of post-thymic T-cell neoplasia includes the angiocentric immunoproliferative lesions, a group of disorders histologically exhibiting vascular infiltration and destruction; included among these disorders is angiocentric lymphoma. In contrast to the typical extranodal presentation seen in the angiocentric immunoproliferative lesions, this report describes a case of angiocentric lymphoma presenting as primary lymph node disease with clinicopathologic findings mimicking a hemophagocytic syndrome. Rearrangement of the T-cell receptor beta chain documents this case to be a clonal T-cell neoplasm. The association of this distinct histologic type of T-cell malignancy with hemophagocytic syndromes is reviewed.
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ranking = 4.162699653596
keywords = malignancy, neoplasm
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7/10. Inversion of chromosome 5 long arm in region of cell growth gene cluster in hematologic disorders.

    Inversions of the long (q) arm of chromosome #5 are reported in five cases with hematologic disorders. Inversion of 5q with breakpoints in bands 5q13 and 5q33 was found in two cases with lymphoid malignancy and in two cases of myeloid hematologic malignancy. Because an inversion of 5q with breakpoints in 5q22 and 5q33 was also found in a case with myeloproliferative syndrome, the common denominator in these five cases was band 5q33. An extraordinary cluster of genes affecting cell growth and differentiation is present on 5q and may be altered by the chromosome rearrangement of 5q in hematologic disorders.
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ranking = 6.325399307192
keywords = malignancy
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8/10. magnetic resonance imaging in pediatric hematology/oncology. Part II. Illustrative cases and assessment of technique.

    Preliminary clinical experience with magnetic resonance imaging (MRI) in 28 pediatric patients with 20 different hematological diseases, benign tumors, or malignant neoplasms is presented. The clinical results are presented in the form of case presentations that are discussed in the context of alternative diagnostic imaging modalities. Also discussed are the known biological effects, or lack thereof, the need for sedation, the effect of motion, the effect of MRI on foreign metallic objects, the financial considerations, and the trends for the future of MRI. This imaging modality has many unique merits. Present difficulties should be overcome by future innovations, making MRI even more efficacious for the diagnosis of blood diseases and cancer in children.
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ranking = 1
keywords = neoplasm
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9/10. Malignancy, mortality, and medicosurgical management of clostridium septicum infection.

    BACKGROUND. Necrotizing clostridium septicum infections (CSI) have a strong association with malignancy or immunosuppression. To clarify this relationship and determine how it impacted mortality, the experience with CSI at a single institution was reviewed. methods. Records of all patients admitted to our hospital with culture proven clostridial infection from 1966 through 1993 were reviewed. RESULTS. Among patients presenting with clinical gas gangrene, 281 had culture proven clostridial infection and 32 (11.4%) had CSI. The mortality among CSI patients was 56%, whereas 26% of all patients with clostridial infections died (p = 0.001). An associated malignancy was found in 50% of patients with CSI, whereas this was seen in only 11% of patients with other clostridial infections (p = 0.0001 for CSI versus clostridial infection overall). The remaining patients with spontaneous CSI all had evidence of immunosuppression. CONCLUSIONS. The high mortality and likelihood of associated malignancy or hematologic disease underscore the importance of a high index of suspicion and the need to search for and treat associated conditions in all patients with CSI.
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ranking = 9.488098960788
keywords = malignancy
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10/10. Systemic amyloidosis AL with temporal artery involvement revealing lymphoplasmacytic malignancy in a man presenting as polymyalgia rheumatica.

    A 68 year old man presented with a clinical and biological picture that suggested polymyalgia rheumatica. Temporal artery biopsy disclosed no inflammatory change but massive light chain amyloid deposits in the media. Further exploration showed a malignant lymphoplasmacytic haemopathy with a triclonal gammopathy and a muscular, rectal, and probable cardiac amyloidosis. Cryoglobulinaemia and high concentrations of soluble interleukin 2 receptor (sIL-2R) were also found. This is the fifth case with confirmed involvement of the temporal artery. The especially high sIL-2R concentration was thought to reflect the tumour mass rather than lymphocyte activation.
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ranking = 12.650798614384
keywords = malignancy
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