Intra-abdominal, angiofollicular lymph node hyperplasia (plasma-cell variant) with an antierythropoietic factor.
An 11-year-old girl presented with a refractory hypochromic microcytic anemia, hypoferremia, normoblastic hyperplastic bone marrow, hypergammaglobulinemia, and growth retardation. Many varied treatments failed to produce any improvement. Ferrokinetic studies revealed rapid plasma clearance and increased plasma iron turnover, but impaired incorporation of 59Fe. Excretion of 57Co after an oral dose indicated an increased iron absorption. A (99M)Tc-sulfur colloid scintigram of the abdomen failed to demonstrate abnormal uptake. A nodal mass showing the plasma-cell variant of angiofollicular hyperplasia was removed from the gastrolienal ligament. Follow-up studies at 3 and 6 months revealed complete correction of the anemia, a 4.8-cm increase in height, and normal serum gamma-globulin levels. Serum obtained before operation inhibited the incorporation of 59Fe that was induced by a standard dose of erthyropoietin in the exhypoxic mouse system, and this inhibition persisted in serum obtained 3 days after surgery but disappeared by 6 days. The data suggest that the hyperplastic angiofollicular lymph node (plasma-cell variant) secreted a substance the inhibited erythropoiesis.
D000747:Anemia, Hypochromic / Q000097:blood* / Q000150:complications; D001827:Body Height; D002648:Child; D003864:Depression, Chemical; D004920:Erythropoiesis / Q000187:drug effects; D004921:Erythropoietin / Q000037:antagonists & inhibitors*; D006801:Humans; D006965:Hyperplasia / Q000097:blood / Q000276:immunology / Q000473:pathology; D007074:Immunoglobulin G; D007501:Iron / Q000378:metabolism; D008198:Lymph Nodes / Q000276:immunology / Q000473:pathology; D008297:Male; D005719:gamma-Globulins
D007074:Immunoglobulin G; D005719:gamma-Globulins; D004921:Erythropoietin; D007501:Iron