Gastrointestinal bleeding due to leukemic infiltrate in myelodysplastic syndrome.
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Biomedical subjects
Publications and source records attributed to A Manoharan.
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We describe 10 cases of primary myelodysplastic syndrome in which marrow fibrosis was striking at presentation. All the cases showed trilineage dysplasia with increased megakaryopoiesis and marked reticulin fibrosis. Significant organomegaly was notably absent. This association has hitherto not been highlighted and it is important to distinguish these cases from those of idiopathic myelofibrosis with which they may be confused. Furthermore, their comparatively long survival distinguishes these cases from those previously described as acute myelodysplasia with myelofibrosis and malignant myelosclerosis. The pathogenesis of fibrosis in these cases may be related to disordered megakaryopoiesis and the platelet-derived cytokines that may be released. The treatment of these fibrotic cases remains problematical and further investigation is required.
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A 41 year old female developed reactive haemophagocytic histiocytosis secondary to herpes simplex infection, during remission induction for acute lymphoblastic leukaemia. She recovered fully with acyclovir and supportive treatment. Previous publications on the association between acute lymphoblastic leukaemia and haemophagocytic syndrome are reviewed, and the nature of the haemophagocytic disorder is discussed.
We describe a 55-year-old female with acquired amegakaryocytic thrombocytopenia who has been successfully treated with antithymocyte globulin. In-vitro studies assessing megakaryocytopoiesis in the presence of the patient's plasma and peripheral blood adherent cells showed normal or increased stimulation. This patient brings to 30 the number of adult cases of acquired amegakaryocytic thrombocytopenia now reported in the English literature. Review of this material suggests that it may be more common than has been appreciated. Several pathogenic mechanisms, especially immune mechanisms, have been identified; good, sustained remissions have been achieved in eight patients who were treated with immunosuppressive agents.
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Fibrosis in patients with myelofibrosis (MF) is seen not only in the bone marrow but also around the metaplastic foci in the spleen and liver. Following our observation of reversal of bone marrow fibrosis in MF patients treated with chemotherapy, we undertook ultrasonic splenic tissue characterization studies using sound speed measurements to assess the effect of such therapy on the splenic fibrosis. Single studies in 19 patients showed overlapping ranges among the three sub-groups classified according to the grade of bone marrow fibrosis, but the mean values in these sub-groups showed a trend towards decreasing sound speed with increasing marrow fibrosis. Sequential studies in 9 of these patients showed increased splenic sound speed with decreasing marrow fibrosis in 8 patients following treatment, with a reversal of this pattern seen in 1 patient 1-2 years post-treatment. These results suggest simultaneous reversal of fibrosis in the bone marrow and the spleen in MF patients receiving effective chemotherapy.
Bleeding and thrombosis are well known major causes of morbidity and mortality in patients with myeloproliferative disorders (MPD) but the relationship between these clinical events and the commonly found platelet function abnormalities have not been established. In this study we performed simultaneous laboratory evaluations in 54 patients with MPD to investigate abnormalities in platelet aggregation and cyclooxygenase activity, the latter by using an in vitro aspirin inhibition test; the studies were repeated in 22 patients 1-27 months later. We have found platelet hyper- and hypofunction co-existing in some patients (9/54), and change of platelet function during the course of the disease (7/22) with platelet hypofunction being the only constant abnormality over time. These results may explain the lack of correlation between the clinical events and the limited assessment of platelet function in the hitherto published studies, and also suggest the need for repeated evaluations to properly assess the relative risk for bleeding and thrombosis.
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A leukoerythroblastic blood picture and tear drop shaped red cells are two characteristic features of myelofibrosis (MF). Following our earlier observation of an increase in the hemoglobin level with chemotherapy, we have now reviewed the pre- and post-treatment peripheral blood smears from 14 patients with MF to assess the effect of treatment on the above mentioned peripheral blood features. Our results suggest that these features are reversible with effective chemotherapy.
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Splenic blood flow and splenic volume were measured by Doppler and cross sectional ultrasound in 72 human subjects consisting of 61 patients with splenomegaly and 11 normal subjects. This non-invasive and relatively simple method yielded satisfactory results in 76% of the subjects studied; failures were due to obesity, debility, distorted vascular anatomy, and excessive gas in the stomach. These preliminary results suggest that the Doppler ultrasound technique is a practical clinical method for measuring splenic blood flow.