Cases reported "Hematologic Diseases"

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1/201. Importance of basophilia in haematopoietic disorders.

    To the significance of basophilia in haematopoietic disorders, six draw attention to cases have been analyzed. Associated diseases included acute myelogenous leukaemia (AML-M2, M3, M4, and M6), refractory anaemia with excess of blasts (RAEB) and RAEB in transformation (RAEB-T). Two AML cases (M2, M6) were preceeded by myelodysplastic syndromes (MDS). All patients showed greater than 3% basophilia in peripheral blood and bone marrow. basophils were identified successfully by metachromatic staining with toluidine blue in all cases. Three patients (M3, M4, RAEB) presented with lymphadenopathy, suggesting an association with extramedullary involvement. Neutrophil alkaline phosphatase (NAP) activity was significantly reduced in four patients with AML (M2, M3, M4) and RAEB-T. The clinical course was generally unfavourable characterized by short remission duration or disease progression except for the patient with RAEB. Haemorrhage was the main cause of death rather than infection. cytogenetic analysis revealed unique abnormalities involving chromosomes 3q21, 5q31, and 17q11 where the genes for some haematopoietic growth factors or their receptors are located, in addition to t(6;9) and t(15;17). ( info)

2/201. Booze and blood: the effects of acute and chronic alcohol abuse on the hematopoietic system.

    Acute and chronic alcohol abuse are common conditions in patients admitted to hospitals. Alcohol has widespread direct and indirect effects on the hematologic system which can mimic and/or obscure other disorders. Leukocyte, erythrocyte, and thrombocyte production and functions are affected directly. liver damage secondary to alcohol abuse also impacts red blood cells also impacts red blood cells and the hemostatic mechanisms. Nutritional deficiencies are caused not only by poor dietary habits practiced by alcohol abusers, but by the effect of alcohol on the absorption, storage, and utilization of several vitamins. Identifying these numerous effects results in a more comprehensive and clinically accurate understanding of the patients hematologic status. ( info)

3/201. Hematologic effects of thrombophilia.

    The hematologic effects of thrombophilia are subtle, and when recognized may provide clues for the diagnosis of hypercoagulation in patients. This article identifies aberrant, routine test results associated with the diagnosis of thrombophilia. The future expansion of laboratory testing for thrombophilia detection is presented in summation. ( info)

4/201. Immunohistochemical study on transforming growth factor-beta1 expression in liver fibrosis of Down's syndrome with transient abnormal myelopoiesis.

    A case of Down's syndrome associated with liver fibrosis is reported. The fibrosis was diffusely distributed along sinusoids, and an excess of megakaryocytes was also found in the liver. To determine the mechanism of liver fibrosis in Down's syndrome, we immunohistochemically stained the liver with markers of myofibroblast-like cells, antialpha smooth muscle actin antibodies and antidesmin antibodies. The myofibroblast-like cells were found along sinusoids, suggesting that liver fibrosis in Down's syndrome is caused by the myofibroblast-like cells derived from Ito cells/lipocytes. The expression of transforming growth factor (TGF)-betal, which is an important mediator of the activation of lipocytes, was immunohistochemically examined. The accumulation of TGF-betal was observed in cells in the sinusoidal spaces, which involve the intracellular expression of megakaryocytes. Together, these findings suggest that megakaryocyte-derived TGF-betal is one of the likely candidates in the lipocyte activation of liver fibrogenesis in Down's syndrome. ( info)

5/201. 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. ( info)

6/201. Sinus polyp-associated soft tissue lesion and unilateral blindness: complications of extraction in leukemic patient.

    A case of an inflammatory polyp-associated lesion extending through an extraction socket appearing as an intraoral nodular lesion and unilateral blindness secondary to leukemic optic nerve head infiltration is reported. The patient was a 28-year-old male whose his upper first molar had been extracted fifteen days previously. The lesion was an asymptomatic soft tissue mass, red in color and hot tender to palpation, involving the alveolar ridge in the maxillary molar area. Although this is apparently a rare occurrence, the nature of the lesion was suggested by the history, clinical appearance, and radiographic findings. Excision of the inflammatory lesion was followed by complete healing with closure of the lesion. Unfortunately, the blindness was irreversible. The patient is still under leukemia therapy. review of the literature did not yield any other such cases. The role of oral lesions as a diagnostic indicator and the importance of dental surgeons in the diagnosis of leukemic patients are discussed. It is concluded that proper precautions and meticulous early diagnosis are required in these patients and that dental practitioners should be aware of the diagnostic features and possibilities of oral complications associated with leukemia. ( info)

7/201. 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. ( info)

8/201. 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. ( info)

9/201. patients with myelodysplastic syndromes benefit from palliative therapy with amifostine, pentoxifylline, and ciprofloxacin with or without dexamethasone.

    Thirty-five patients with myelodysplastic syndrome (MDS) were registered on protocol MDS 96-02 and were receiving continuous therapy with pentoxifylline 800 mg 3 times a day and ciprofloxacin 500 mg twice a day by mouth; dexamethasone was added to the regimen for the partial responders and the nonresponders after 12 weeks at a dose of 4 mg by mouth every morning for 4 weeks. amifostine was administered intravenously 3 times a week at 3 dose levels (200 mg/M(2), 300 mg/M(2), and 400 mg/M(2)) to cohorts of 10 patients each. Therapy has been continued for 1 year in responders. Twenty-nine have completed at least 12 weeks of therapy and are available for response evaluation. Of the 21 men and 8 women (median age, 67 years), 20 had refractory anemia (RA), 3 had RA with ringed sideroblasts (RARS), 5 had RA with excess blasts (RAEB), and 1 had chronic myelomonocytic leukemia (CMMoL). Five had secondary MDS. No differences were noted in response rates among the 3 dose levels. Seven patients did not respond at all, and 22 showed an improvement in cytopenias (76%). Three had a triple lineage response, 10 had a double lineage response, and 9 had a single lineage response (8 of 9 in absolute neutrophil count [ANC] and 1 had more than a 50% reduction in packed red blood cell transfusions). Fifteen patients responded only after the addition of dexamethasone, whereas 7 responded before. When examined by lineage, 19 of 22 showed improved ANC, 11 of 22 demonstrated more than 50% reduction in blood transfusions, improved Hb levels, or both, and 7 of 22 showed improvement in platelet counts. Interestingly, the responses were frequently slow to appear, and continued improvement in counts was seen up to 12 months of therapy and beyond. This study supports the feasibility of treating patients with MDS with the unique approach of cytoprotection and anticytokine therapies as well as the principle that prolonged commitment to treatment is desirable when noncytotoxic agents are administered. (Blood. 2000;95:1580-1587) ( info)

10/201. incontinentia pigmenti in a surviving male is accompanied by hypohidrotic ectodermal dysplasia and recurrent infection.

    Familial incontinentia pigmenti (IP) is a rare X-linked dominant condition. The affected cases have characteristic skin lesions, hair, eye, teeth and nail abnormalities and may also have neurological problems. The diagnosis has traditionally been made on clinical grounds. Segregation analysis has suggested that it is lethal in males. Only one liveborn male has been reported who died at one day of age. female cases of IP survive because of the moderating effects of Lyonization. This child was the affected son of a female with IP. He had a novel phenotype consistent with hypohidrotic ectodermal dysplasia with immune deficiency (HED-ID) but with additional features: he had major problems with hematological disturbances, failure to thrive due to malabsorption, recurrent infections, generalized osteosclerosis and lymphedema of his lower limbs. He also demonstrated some typical features of IP with a generalized reticular skin hyperpigmentation, sparse hair and delayed eruption of teeth. The gene for NEMO (nf-kappa b Essential Modulator) has recently been shown to be mutated in cases of IP. Furthermore, most (80%) of patients possess a recurrent genomic rearrangement that deletes part of the gene resulting in an inactive NEMO protein. In the male case described here, a NEMO stop codon mutation has been identified that has arisen de novo in his affected mother. This mutation is likely to have a less severe effect on NEMO activity and may explain why this child survived for two years and 7 months. ( info)
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