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Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is an uncommon disorder of the lymph glands that is characterized by an increase in the blood levels of proteins called gammaglobulins. Symptoms of AILD include fever, anemia (decreased red blood cell production), abnormal white blood cell production, and the presence of autoantibodies (antibodies that attack the body's own tissues). AILD can affect the liver, spleen, bone marrow and skin. A diagnosis of AILD is usually made by examining samples of lymph node tissue (biopsy). Early in the disease, immune cells (B cells) in the lymph tissue increase in number and make large quantities of antibodies. In some cases this results in the development of malignant (cancerous) lymph node tumors. Most treatments are not effective in treating AILD, and roughly 70 percent of those diagnosed as having AILD die within two years. Most of the patients die as a result of infections. In most cases of AILD the joints are not affected. This article describes the case report of a 61-year-old male patient who developed arthritis several months prior to the onset of AILD. Radiographs (x-rays) of the joints showed signs of swelling, but no cartilage damage or loss. Joint fluid and blood tests for the presence of rheumatoid arthritis were negative. Joint fluid from the wrist contained elevated levels of white blood cells characteristic of AILD. The patient was treated successfully with methylprednisolone and cyclophosphamide. This finding indicates that AILD may affect the joints, and that joint biopsies provide a better method for diagnosing joint involvement than joint fluid sampling. (Consumer Summary produced by Reliance Medical Information, Inc.)

The iron transport function of transferrin from normal subjects and patients with idiopathic hemachromatosis has been studied using the radioisotopes Fe59 and Fe55. It was concluded that transferrin from hemochromatotic patients functions in a normal manner in vivo.

1. By means of phlebotomy to recover and measure accumulated iron and by determining the rate of replenishment of hemoglobin after induction of iron-deficiency anemia, it is possible to compute the rate at which iron is accumulated by absorption from the diet. Normal humans are presumed to be in iron balance and absorb iron only to replace what is lost; under these conditions they accumulate none. During recovery from induced iron deficiency a normal human accumulated iron at the rate of 5 to 6 mg. per day. 2. Patients with hemochromatosis were found to accumulate iron at the rate of 1.5 to 5 mg. per day. With iron deficiency the rate increased to 8 to 10 mg. per day. Because of uncertainty concerning the manner of onset of iron accumulation in hemochromatosis, whether gradual or abrupt, it is not possible by extrapolation to establish the time of onset in these patients. However, at the rates of accumulation established in this study there was an insufficient excess of iron to permit a conclusion that such rates had existed throughout the patient’s life. If the disease began early, the initial or interim rates were less than those found. If the disease began and continued at the rate found, its onset was 10 to 17 years prior to this study. 3. In a patient with hypochromic iron-loading anemia the rate of accumulation was about 2.5 mg. per day. It was not appreciably increased by iron deficiency except when the deficiency was severe and the plasma iron was quite low. While this patient was iron deficient the plasma iron became abnormally high even before the hemoglobin mass was completely reconstituted. 4. A patient with transfusion siderosis who had recovered from his anemia was phlebotomized to remove the accumulated iron. When iron deficiency finally developed the total amount of iron which had been recovered was less than half of the 32.5 Gm. given in the transfusions some years before. It was computed that during those years the patient was losing iron at a rate of about 4.0 mg. per day. The rate of iron accumulation during his recovery from the induced iron deficiency was the same as the normal: 5 to 6 mg. per day. 5. The ability to lose iron which is in excess of requirement is implicit in the demonstration of iron-laden deciduous cells such as the glandular epithelium of the stomach and duodenum and macrophages in the intestinal villi. 6. In hemochromatosis there is a failure of the intestinal mucosal block to prevent absorption of unneeded iron. When absorption exceeds the capacity of the iron excretory mechanisms, iron accumulation occurs. In iron deficiency the excretory mechanisms become less active so that the rate of iron accumulation is further increased.

1. Using Fe59 and whole body counting, various aspects of gastrointestinal absorption of iron salts were studied in normal, iron-loaded, and iron-deficient rats. 2. Following a single intragastric dose of iron, peak small intestinal uptake was observed by 30 to 60 minutes with subsequent gradual loss over 24-hour period. 3. Iron absorption showed two phases, an initial period of rapid absorption lasting up to 2 hours during which 60 to 80 per cent of total absorption into carcass took place. The remainder took place at a slow rate over the subsequent 12 to 20 hours. 4. Depending on the relationship between body iron stores and dose of administered iron, the intestine may take up more iron than is ultimately transferred to plasma. A variable amount of this iron is lost when the epithelial cell is sloughed into the gastrointestinal lumen. 5. Regulation of iron absorption appears to involve two steps, mucosal uptake and transfer to the blood.

Using an in vitro assay for gastric intrinsic factor, it was shown that seventeen of twenty-three patients with treated and untreated tropical sprue have adequate intrinsic factor in their gastric juice.

1. Two Negro brothers with homozygous hemoglobin C disease are reported. In one splenectomy was performed. 2. Intraerythrocytic crystals in smears of peripheral blood were demonstrated only in the patient who had splenectomy. The crystals could not be found in re-examination of bone marrow preparations of this patient that were made prior to splenectomy. 3. Absence of the spleen may be a necessary factor for the formation of these crystals. 4. The presence of homozygous C disease is compatible with a normal life span.

The level of serum ferritin is a reliable indicator of body iron stores. Exceptions include liver disease, malignant diseases, and treatment of iron-deficiency anemia. The latter was noted in iron-deficient infants who showed a rise of serum ferritin to normal levels in the first week of treatment. To evaluate this in adults, 14 patients with iron-deficiency anemia were studied prior to and after beginning treatment with oral ferrous sulfate in standard dose, 300 mg t.i.d., or double dose, 600 mg t.i.d. Serum ferritin was assayed by radioimmunoassay. No rise occurred in the first 3 wk in 5 patients treated with standard dose, although hematologic response occurred. With double dose, 7 of 9 showed a ferritin rise in 2 days with return to subnormal levels within 6 days of discontinuing iron. This study indicates that standard treatment of iron deficiency anemia in adults does not cause a rise in serum ferritin until hemoglobin levels are normal. The early rise seen with double dose is most likely due to absorption of iron in excess of utilization for erythropoiesis resulting in temporary storage. When iron is discontinued, stores are rapidly depleted as reflected by the prompt decrease in serum ferritin.