Anaemia - a dangerous diagnosis.
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Biomedical subjects
Publications and source records attributed to P Jacobs.
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A 32-year-old man presented with left hypochondrial pain and myalgia. On examination he was found to have widespread lymphadenopathy and splenomegaly, and lymph node biopsy revealed diffuse lymphocytic lymphoma of the small cleaved cell type. There was hypereosinophilia (20 x 10(9)/1). The patient was initially treated with chlorambucil and prednisone, which controlled the lymphoma for 9 months but did not affect the eosinophilia. He then developed thrombocytopenia with further lymph node enlargement which was managed with a combination of bleomycin, vincristine and prednisone; this again controlled both spleen and lymph node size without affecting the eosinophilia. Six months after presentation the patient developed refractory cardiorespiratory failure due to endomyocardial fibrosis and fibrosing alveolitis. The cardiac failure was thought to be related to the hypereosinophilia resulting from the lymphoma, while the respiratory insufficiency was attributed to the bleomycin. This case illustrates the rare association between lymphocytic lymphoma and eosinophilia and shows that cardiac damage can be associated with these cells.
A young woman aged 18 years underwent allogeneic bone marrow transplantation for severe acute aplastic anaemia. In the 3 weeks following this procedure the platelet count remained between 10 and 30 X 10 q/l, and from the 21st day after the transplant there was relentless progression of congestive cardiac failure resulting in the patient's death 4 days after its onset. Autopsy showed widespread subendocardial haemorrhage and extensive bleeding between the myocardial fibres resulting in refractory cardiac failure. These findings are compatible with drug-induced cardiotoxicity.
Central corneal endothelial cell density was estimated in 33 consecutive patients preoperatively and at monthly intervals for 6 months after cataract surgery. The patients were divided into 3 groups by a random procedure to have either intracapsular extraction or intracapsular extraction plus Federov 4-loop iris clip lens, or extracapsular extraction with Binkhorst 2-loop iridocapsular lens. In all groups the greatest cell loss had occurred by one month and thereafter no accelerated loss was apparent except in individual patients. The limitations of such a method of assessment are discussed.
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To determine whether the plasma ferritin assay reliably reflects body iron stores, as determined by the presence of stainable iron in the marrow, 63 iron-deficient patients were serially studied after receiving a total dose infusion of iron dextran (Imferon). In 58% (Group I) anemia did not recur. In 19% (Group II) classical iron deficiency was present with low plasma ferritin levels, reduced transferrin saturation and absence of stainable iron in the marrow. In 13% (Group III) evolving iron deficiency was seen with low plasma ferritin level but normal hemoglobin and transferrin saturation. The development of overt iron deficiency was associated with a fall in the transferrin saturation and absence of stainable iron in the marrow. In the remaining 10% (Group IV) the initially raised plasma ferritin fell in parallel with the developing anemia, reduction in transferrin saturation and absence of reticuloendothelial marrow iron. These studies demonstrated that in the development of iron deficiency anemia following total dose infusion of iron dextran (Imferon) there was a relationship (r = 0.9625; p less than 0.05) between decreasing body iron stores and falling plasma ferritin levels. Accordingly, the latter measurement should replace bone marrow studies carried out for this purpose.
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The effect of Corynebacterium parvum on survival was studied in aplastic rabbits transplanted with allogeneic bone marrow cells. Twenty four hours after receiving 1200 rad of total body irradiation, NZW males were reconstituted from unrelated R females using bone marrow combined with spleen cells or with marrow alone. The effects of treating donor, or both donor and recipient, with C parvum were contrasted. Rabbits in Group I were infused with a mixture of bone marrow and spleen cells. In control aminals (Group Ia) none of the 14 animals survived to 40 days. Similarly, there were no survivors among 17 animals in group Ib where only the donors were pretreated with C. parvum. When both donor and recipient received this agent (Group Ic) three of the 13 animals (23%) survived beyond 40 days. Animals in Group II were infused with marrow cells alone. Of the controls (Group IIa), 3 out of 24 animals (12.5%) survived to 40 days; pretreatment of the donor with C. parvum (Group IIb) resulted in survival of 8 of the 18 animals (44%); when both donor and recipient received this agent (Group IIc), 16 of 26 animals (62%) survived beyond 40 days. Both the incidence and the severity of histologically demonstrable graft-versus-host disease was markedly reduced in the surviving animals. These observations demonstrate a significant effect of parenteral C. parvum on the prolongation of survival in the irradiated rabbit following transplantation with allogeneic bone marrow cells, which is related to reduction in GVHD.
A platelet derived growth factor released following platelet adhesion to the subendothelium of damaged arteries may promote the development of atherosclerotic lesions. Consequently patients with von Willebrand's disease in whom platelet adhesion is abnormal may be protected from atherosclerosis. This possibility was investigated by performing an autopsy study on an elderly patient who had suffered from severe von Willebrand's disease. Extensive atherosclerotic lesions were found at post mortem suggesting that platelets may not play a clinically significant role in the causation of atherosclerosis.
Marked hyperkalaemia occurred on more than one occasion in 3 patients with thrombocytosis caused by the myeloproliferative syndrome. Initial failure to recognize that the elevation in the serum potassium level was an artefact resulted in unnecessary admissions to hospital and inappropriate investigations and treatment. Awareness of this entity and the use of suitable confirmatory tests should avoid such problems in management.
An immunologically competent adult with acquired toxoplasmosis presented with inguinal lymphadenopathy and associated lymphatic and venous obstruction. A diagnosis of toxoplasmosis was made by demonstration the presence of the trophozoite, and response to therapy, while satisfactory, was slow. Recrudescence of lymphadenopathy was associated with a change from reactive lymphadenitis to angioimmunoblastic lymphadenopathy. There was a dramatic improvement following local therapy and systemic corticosteroids and chlorambucil. The patient remains well.
Two patients with pure red cell aplasia of undetermined aetiology are in sustained, complete remission after immunosuppressive therapy. In the first, short-term supplementary plasmapheresis was used during initial chemotherapy, and in the second, anabolic androgens were administered. In both patients haematopoietic recovery was temporally related to the withdrawal of the cyclophosphamide used to cause immunosuppression in the patients over a 4- and 6-month period respectively.
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Ethical considerations arise frequently in managing the patient with cancer. These range from controversy as to whether the diagnosis should be divulged to the patient or only to his family to whether the disease should be treated and, if so what is best for the patient. In many instances clearcut answers are not available--the type of challenge facing the physician who has accepted responsibility for managing the patient with malignant disease is illustrated by consideration of some of the problems encountered in leukaemia.
Anaesthesiology is one of the fastest growing specialties in medicine. Conventionally, the expanded responsibilities include control of intravenous infusion of blood, blood components, and plasma fractions. The advances taking place in medicine generally are reflected in the greater sophistication and range of surgical procedures that can be undertaken safely, such as elective operations on patients known to be at risk from bleeding. Their successful outcome depends upon co-ordination between anaesthetist, surgeon and haematologist. This teamwork can be illustrated by considering an approach to the problem of haemostatically unbalanced patients requiring surgical procedures.
A group of 82 adult patients with acute myelogenous leukaemia had survived in continuous first remission for more than three years was studied. These long-surviving patients were being treated at 12 referral centres in Europe and the USA, and they were compared with other patients with acute myelogenous leukaemia from 10 of these centres. There was no clear difference in the amount of induction chemotherapy or the time taken to achieve remission. Immunotherapy was not found to improve chances of long-term survival. The 82 patients were also compared with a group of 115 patients who had no appreciable difference in the number of blood or marrow myeloblasts between these two groups at presentation, but the long survivors had significantly higher initial platelet counts and were slightly younger. The long survivors also tended to have a lower total white cell count at presentation and lower granulocyte counts; there was no obvious explanation for these differences. Eight of the 82 patients relapsed from three to four years after remission and two (of 69 patients) after four to five year. Thereafter relapse was rare, and it seems likely that some of the 40 patients who have survived for five years or more are cured.