Letter: Detection of scanty blood parasites.
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Biomedical subjects
Publications and source records attributed to P Jacobs.
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Iron overload with prominent skin pigmentation and hepatic parenchymal siderosis is reported in a patient with refractory sideroblastic anaemia. Failure to reverse the sideroblastic changes or the anaemia with conventional therapy prompted us to use a graded phlebotomy programme. The rising haemoglobin level in spite of repeated venesections is support for this approach in selected patients with this diagnosis.
Experiments in rabbits with radiation-induced bone marrow aplasia have demonstrated that intramedullary block grafting is a feasible procedure. This technique requires surgical interference and offers no advantage over intravenous reconstitution. We therefore suggest that it has no value in therapeutic bone marrow transplantation.
Two patients are reported in whom splenectomy during the course of myelofibrosis was followed by rapid deterioration in the function of a previously involved liver, and was characterised by refractory ascites, increasing jaundice, and death. It is important to recognise this complication, since ectopic haematopoiesis can be demonstrated on liver biopsy and, perhaps, controlled with radiotherapy or cytotoxic chemotherapy.
Infusion of functional allogeneic granulocytes is rational therapy in pyrexial patients with severe neutropenia when combined with appropriate antibiotic administration. On the basis of our first year's experience we endorse this approach, but emphasise the need for further critical evaluation of these cells, and present an approach to placing this procedure on a more scientific basis.
Two cases of acute leukaemia, one monoblastic and one myelomonocytic, in which hypokalaemia, hypomagnesaemia and hypocalcaemia were documented, are reported. Urinalysis demonstrated renal loss of potassium and magnesium without other evidence of renal tubular dysfunction. The mechanism and clinical significance of these abnormalities are briefly discussed.
Skeletal age was estimated by examination of radiographs of the carpus in 182 children suffering from Perthes' disease after the reliability of the Greulich and Pyle Atlas had been checked for a control group of British children. A striking tendency to delayed skeletal maturation was shown in the children with Perthes' disease. This trait was also found in ninety-three unaffected siblings of the patients. The velocity of skeletal ageing as the disease progressed was estimated. In some patients the carpal skeleton failed to mature at all for periods of up to three years and the term "skeletal standstill" is applied to this phenomenon. The significance of these findings is discussed and it is suggested that the maturation defect may have aetiological significance.
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Bone marrow iron stores rise in proportion to the total body iron store in dietary iron overload. The situation in the genetic disorder of idiopathic hemochromatosis is not as clear. A method for measuring the storage iron concentration chemically on samples of bone marrow obtained by trephine needle biopsy was therefore developed. Its value as a measure of tissue iron stores was established in a preliminary investigation in which specimens of liver, spleen, and bone marrow were obtained at necropsies on 66 South African Negroes among whom dietary iron overload is common. A wide range of nonheme iron concentrations was found, but in each individual there was a highly significant correlation between the concentrations in the three tissues. Nonheme iron concentrations were then determined on trephine bone marrow biopsy specimens from eight Caucasian patients with untreated idiopathic hemochromatosis, and on percutaneous liver biopsy specimens from four of them. The concentrations in the livers were in the anticipated range of 5,000 mug per gram wet weight (2 per cent dry weight). In contrast the geometric mean value for bone marrow iron concentration was 186 mug per gram wet weight, a figure that fell below the fiftieth percentile for marrow iron concentrations in the South African Negroes, whereas the geometric mean liver iron concentration was above the ninetieth percentile. These findings indicate that subjects with idiopathic hemochromatosis whose liver iron stores are grossly increased do not show a comparable rise in bone marrow iron stores.
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A patient with the Bernard-Soulier or hereditary giant platelet syndrome, in whom the characteristic morphological features were present and the in vitro abnormalities of platelet aggregation were demonstrated, is reported. Although rare, this syndrome is important, since surgical procedures may be carried out under cover of infused allogeneic platelets.
Four cases of leukaemoid reactions to tuberculosis, notwithstanding the presence of Auer's rods in the myeloblasts, have previously been reported. A fifth case of disseminated tuberculosis in which Auer's rods were similarly observed, is described. However, we believe that this association does not mean that Auer's rods occur as part of a leukaemoid response, but rather that it indicates the simultaneous presence of acute leukaemia and tuberculosis.
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Of 36 patients with systemic lupus erythematosus, 3 were demonstrated to have an abnormality in their haemostatic mechanism. In 2 this was believed to be on the basis of an auto-antibody to the phospholipid of platelets in the prothrombin activator complex. In the third an inhibitor was directed against factor VIII.
A four-day residential workshop was conducted for students beginning their clinical studies at the St George Hospital in 1974. The aim of the workshop was that the students should understand the functioning of the hospital and feel comfortable as students in the hospital, so that they would learn their clinical skills more effectively in their subsequent years at the hospital. Each student lived in a hospital bed with an "illness" for two days, and spent another two days helping care for these "student patients" and exploring the hospital. Evaluation at the end of the workshop suggested that the objectives had been achieved.