Severe sepsis and multiorgan dysfunction syndrome may develop in the course of severe sepsis, intoxication, poisoning, crush injury, asystole, drowning, and several other complications. When normal intensive care is insufficient despite vasoactive drugs, respiratory aid, and dialysis, the risk for death is extremely high. Only very little benefit has been noted in various trials using drug administration such as antibodies against TNF-alfa, immunoglobulin, pentoxifylline and high dose steroids. The use of apheresis (plasma exchange, plasmapheresis, adsorption) to remove toxins, cytokines, and other compounds has been tried in an unselective as well as selective manner. Data now exists that indicate increased survival by this type of therapy. It is time to focus on randomised controlled trials with these techniques to decide the efficacy of apheresis in this area.

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This study was performed to determine whether there is any distinction to be made between single and multiple-lineage cytopenias particularly with regard to natural history and prognosis. From December 1989 to May 1994, five of 50 children (median age 7 years) with chronic immune cytopenias were diagnosed with multi-lineage immune- ediated cytopenias. Two patients presented with immune thrombocytopenia (ITP) and later developed autoimmune hemolytic anemia (AIHA); one had ITP and immune eutropenia who subsequently became Coombs’ positive but never developed AIHA. One child presented with ITP and immune neutropenia and later developed AIHA. The fifth child presented simultaneously with thrombocytopenia and neutropenia with positive antineutrophil antibody but without antiplatelet antibody and Coombs’ positivity. Four patients were given primary therapy with IVIG and one with prednisone. One patient responded to prednisone but relapsed subsequently. Further treatment with IVIG roduced initial normalization of his counts with occasional fluctuation of the absolute neutrophil count. Two responded to IVIG and are in complete remission (CR). Of the two nonresponders to IVIG, one responded subsequently to prednisone and is in CR. The other one, after being refractory to multimodality treatment, was diagnosed with a lupus erythematosis variant and is currently on alternate day prednisone. Moderate thrombocytopenia and absolute neutropenia still persist. Multi-lineage immune-mediated cytopenias may represent a pathogenic phenomenon that is distinct from autoimmune single-lineage disease. Clinical response to treatment may correlate with these differences that may be genetic in origin. Clinical course and response to therapy are less predictable when autoimmune disease is present.

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The various membrane abnormalities of sickle erythrocytes may result from excessive accumulation of oxidant damage. We measured the sera levels of malondialdehyde, products of lipid peroxidation, Na+-K+/Mg++ Adenosine 5’ triphosphatase (ATPase) and Ca++/Mg++ Adenosine 5’ triphosphatase, erythrocyte membrane enzymes, in patients with sickle cell anemia and compared their levels with that of normal controls. MDA, Na+-K+/Mg++ and Ca++/Mg++ ATPase levels of control groups were 0.90 ± 0.04 nmol/mL, 168 ± 12.9 and 140.6 ± 8.2 nmol Pi/mg prot/hour, respectively. MDA, Na+-K+/Mg++ and Ca++/Mg++ATPase levels of patients were 2.02 ± 0.01 nmol/mL, 127.0 ± 8.4 and 110.0 ± 9.6 nmol Pi/mg prot/hour, respectively. Our experimental results showed that there was a significant increase in MDA levels of patients with sickle cell anemia as compared with that of the controls. However, erythrocyte membrane Na+-K+/Mg++ and Ca++/Mg++ ATPase levels of patients with sickle cell anemia were significantly lower than the, Na+-K+/Mg++ and Ca++/Mg++ ATPase levels of normal controls.

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A female with no previous history of bleeding presented with active bleeding from multiple body sites, declining hemoglolobin levels, and markedly prolonged prothrombin times (PT), and activated partial thromboplastin times (APTT) with incomplete correction on PT mix assays. This patient showed a severe deficiency of factor X. FX levels and bleeding were refractory to multiple transfusions of fresh frozen plasma. Although plasmapheresis was started with concomitant intravenous immunoglobulin and steroid therapy, the patient died.

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Chronic a-interferon use has been reported to cause a variety of neurotoxic side effects. This case summary suggests the possibility of a new neurotoxic side effect of normal pressure hydrocephalus following chronic a-interferon use.

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Inflammatory pseudotumor of the lung is a benign tumor which is rarely seen. A mass in the left lung was observed in the chest roentgenogram of an 8 year-old boy while investigating for anemia. Preoperative investigations could not render the exact diagnosis. Pneumonectomy was required in exploratris thoracotomy. Histopathologic diagnosis was fibrohistiocytic type of inflammatory pseudotumor. Four months after the operation, anemia was cured without any further treatment.

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