In order to investigate the pathogenesis of anemia in childhood malignancy, we studied erythroid cell proliferation responses with bone marrow erythroid cultures and serum erythropoietin (Ep) levels in 32 children with lymphomas and malignant tumors. The erythroid colony formation from 20 normal controls (mean 68.8 colony-forming-unit erythroid [CFU-E] and 32 burst-forming-unit erythroid [BFU-E] derived colonies/10(5) mononuclear cells), was higher than that seen in children with lymphomas (mean 45.9 CFU-E and 20.7 BFU-E/10(5) cells, p less than 0.01) but similar to the values obtained from children with tumors (mean 65.1 CFU-E and 28.1 BFU-E/10(5) mononuclear cells). The degree of anemia in children with lymphomas correlated negatively (r = -0.7, p less than 0.01) with serum Ep levels. In contrast, a weak positive correlation (r = 0.3, p greater than 0.1) was observed between the degree of anemia and Ep values in the group with tumors. We suggest that the decreased number of committed erythroid progenitors in lymphomas may be the main factor for anemia in these patients, while the abnormal response of Ep to the degree of the anemia suggests a defect in erythropoiesis in children with tumors.
Primary erythrocytosis diagnosed in a 10-month-old female and followed for 12 years is described. The erythrocytosis was associated with an abnormally elevated set point of erythropoietin production in which the sensitivity fluctuated independently, but corresponded to the alterations in the oxygen-carrying capacity of the blood, when the hematocrit was lowered by phlebotomies. Extensive work for secondary erythrocytoses failed to demonstrate a recognizable cause for this abnormal erythropoietin production. Erythroid cell cultures from peripheral blood mononuclear cells showed the existence of at least two populations: one consistent with dramatic expansion of the erythron in keeping with enhanced sensitivity to endogenous erythropoietin, and the other consistent with the features of typical colonies derived from burst-forming units-erythroid (BFU-Es), seen in normal peripheral blood on days 12 to 14 of culture. The expanded population was characterized by the appearance of single colonies on days 4 to 6 and enormous response to the increasing amounts of erythropoietin, which enhanced their number, size, and maturation. The combination of clinical and in vitro data as well as the absence of any abnormality in the erythropoiesis of the parents and sibling suggest that the erythrocytosis in this child represents a new form with a benign course.
We present the case of a 66-year-old man with a history of coronary artery disease and chronic lymphocytic leukemia (CLL) who was admitted to the hospital complaining of chest discomfort and shortness of breath on exertion. The echocardiogram revealed a severe pericardial effusion and a large echogenic mass that infiltrated the lateral wall of the right atrium and ventricle and created a moderate tricuspid valve stenosis. B cell intracardiac non-Hodgkin lymphoma/CLL was diagnosed, and the patient was treated with six courses of CHOP chemotherapy. After the third course, the mass disappeared and the patient's general condition was substantially improved.
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