A 53-year-old man with shock and arthritis 1 month after consolidation chemotherapy for acute myeloid leukemia.
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Biomedical subjects
Publications and source records attributed to R Bieger.
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An 82-yr-old woman presenting with autoimmune haemolytic anaemia was found to have a malignant thymoma. A mild, largely compensated, haemolytic anaemia was observed for 5 weeks; steroid therapy was not instituted. Subsequent excision of the thymoma was followed by prompt and permanent (3-yr follow-up) disappearance of the haemolysis. To date, only two such case reports have been published. In both cases, however, the haemolysis did not abate until some months after excision of the thymoma and subsequent administration of corticosteroids. The history presented here serves to illustrate the intimate relationship between thymomas and certain autoimmune diseases.
This case report concerns a patient with a vitamin B12 deficiency and a normal Schilling test, who had macrocytosis of many years' duration and finally polyneuropathy. All known causes of a vitamin B12 deficiency were excluded. When the Schilling test was performed with egg consumption, excretion of vitamin B12 was clearly diminished. It appears highly likely that this vitamin B12 deficiency developed as a result of malabsorption of protein-bound vitamin B12.
Out of 104 patients with microcytosis (MCV less than 80 fl), 69% had an iron deficiency, 21% a chronic disease and 10% hemoglobinopathy or thalassemia trait. The absence of bone marrow iron stores or the response to iron supplementation were used to establish the diagnosis iron deficiency. On the basis of sensitivity (90%) and specificity (100%), the serum ferritin concentration is more suitable for assessment of iron deficiency than the serum iron concentration, the total iron-binding capacity or the percentual saturation of transferrin. The red cell distribution width (RDW) is the parameter with the highest sensitivity for iron deficiency (94%). An RDW value within the reference interval can be used to exclude iron deficiency in those cases in which the serum ferritin concentration does not accurately reflect the iron stores owing to severe tissue damage, as in inflammation or malignancy.
In a population-based registry of 580 patients with non-Hodgkin's lymphoma (NHL) 54 patients had a primary gastric lymphoma, 42 an intestinal, 113 a primary extranodal lymphoma localised elsewhere than in the gastrointestinal tract and 371 a primary nodal NHL. Histological specimens were reviewed by a panel of pathologists and classified according to the Kiel classification and the International Working Formulation. The 4-year survival rates for primary gastric, intestinal, other extranodal and nodal NHL ranged from 50 to 60%; the 4-year recurrence-free survival rates were 50%, 35%, 19% and 19%, respectively. Among patients with localised intermediate-grade disease survival for those with gastric NHL was better than for those with intestinal lymphoma. Because it is population-based, our study cohort was not subjected to exclusion due to age, performance scale, etc. and therefore provides a more realistic picture of the occurrence and presentation of as well as prognosis for lymphoma in the population.
A 69-yr-old previously healthy woman, presenting with jaundice and the clinical symptoms of an atypical pneumonia, was hospitalized. Haemolytic anaemia due to cold agglutinins was diagnosed, and erythromycin therapy for a suspected Mycoplasma pneumoniae infection was instituted. Serological testing revealed, however, that the infection was attributable to Chlamydia psittaci. The literature on psittacosis and haematological complications is reviewed. Cold agglutinins are sometimes found in association with psittacosis, but a concomitant haemolytic anaemia is rare.
Since the introduction of the Working Formulation for Clinical Usage, 3 different prognostic groups are recognized among the various histological classifications of non-Hodgkin's lymphomas. We looked at the lymphomas of intermediate-grade malignancy and studied retrospectively the staging and treatment of 102 patients. Almost half of the patients with clinical stage I disease, who therefore received radiotherapy only, later appeared to have had more extended disease. For patients with stage II, III and IV disease the complete remission rate was significantly higher with CHOP than with CVP (76% versus 36%). However, once complete remission was achieved there was no difference in disease-free survival between the 2 groups (at 4 yr, 50% of the patients were in complete remission). As far as the amount of cytostatic drugs given during the initial courses of chemotherapy is concerned, no difference was found between patients who achieved complete remission and those who did not. Most patients did not receive the full dosage. For those patients who did not respond well to initial therapy, or who suffered a relapse, second line therapy was disappointing.
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Two cases of pneumococcal sepsis in splenectomized patients were complicated by purpura fulminans. In addition, acute renal failure developed in both patients, and myolysis in one. Immunological findings in the patient with myolysis suggest a possible role of pneumococcal antigen-containing circulating immune complexes in the pathogenesis of these complications.
The neoplastic T cells of a series of seven patients with chronic T-cell neoplasia were tested for helper activity on pokeweed mitogen (PWM)-induced and interleukin 2 (IL-2)-induced Ig synthesis. The neoplastic T cells of all patients had a T3+4+8-11+I1- phenotype but differed in expression of the 3A1 antigen. The neoplastic T cells of three patients had helper activity on both PWM- and IL-2-driven Ig synthesis, and in addition produced IL-2 in response to PWM stimulation. Two of these patients had hypergammaglobulinemia. In contrast, the neoplastic T cells in the remaining four patients did not produce IL-2 and did not support PWM-driven Ig synthesis. The T4+ cells of these four patients, however, provided excellent helper activity on IL-2-driven Ig synthesis. These findings emphasize the role of IL-2 in T cell-dependent Ig synthesis and clearly show that IL-2 production is required for helper activity in the PWM-driven system. It is concluded that the combined use of PWM- and IL-2-driven Ig synthesis systems allows separate analysis of IL-2 production and T-helper activity in health and disease.
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