Cases reported "Lung Diseases"

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1/9. Pulmonary leukostasis: role of perfusion lung scan in diagnosis and follow up.

    A patient with hyperleukocytic myelomonocytic leukemia who presented to the emergency room with sudden pleuritic chest pain and dyspnea is reported. Clinical manifestations included dyspnea tachypnea and hyperventilation. blood gas analysis revealed hypoxemia, hypocarbia, and respiratory alkalosis. Chest X ray was normal, and perfusion lung scan revealed a diffuse vascular occlusive pattern compatible with pulmonary leukostasis. The patient underwent immediate leukapheresis with subsequent mitigation of symptoms. A second perfusion lung scan showed evidence of significant improvement. To our knowledge this is the first published case of hyperleukocytosis presenting with pulmonary leukostasis that was successfully diagnosed and followed by serial perfusion lung scan.
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2/9. Pulmonary leukostasis secondary to all-trans retinoic acid in the treatment of acute promyelocytic leukemia in first relapse.

    All-trans retinoic acid has recently been shown to induce differentiation of acute promyelocytic leukemia cells in vitro and in vivo. Clinical trials of patients treated de novo, in first relapse, and with resistant disease have achieved a high rate of complete remission (CR). The overall toxicity is substantially less than standard induction chemotherapy, with the notable exception of deaths attributed to the development of pulmonary leukostasis coinciding with a rapid increase in the number of mature neutrophils. This report describes a patient who developed pulmonary leukostasis in the absence of significant leukocytosis. The patient survived pulmonary leukostasis, resumed therapy, and achieved a CR. The case demonstrates features of leukostasis which are peculiar to the 'retinoic acid syndrome'.
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keywords = leukostasis
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3/9. Hyperleukocytosis associated pulmonary leukostasis in acute leukaemia.

    leukostasis is a fatal complication in granulocytic leukaemia. brain and lung are most commonly involved organs in leukostasis. In the lung, the clinical presentation simulates infections and haemorrhagic complications of acute leukaemia. Being a medical emergency, early recognition of leukostasis and initiation of therapy prevents mortality.
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4/9. leukostasis: a phenomenon of prolymphocytic leukemia.

    We are reporting a case of a patient with prolymphoctic leukemia complicated by fatal leukostasis. Multiple leukemic thrombi and leukoaggregates were demonstrated on postmortem examination in both the brain and lungs. This represents the first report pathologically documenting leukostasis in a chronic lymphoid malignancy.
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keywords = leukostasis
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5/9. High-voltage irradiation and hydroxyurea for pulmonary leukostasis in acute myelomonocytic leukemia.

    A 29-year-old man with acute myelomonocytic leukemia had an initial leukocyte count of 192 X 10(9) cells/l and 84% blasts. During the initial treatment with hydroxyurea, 1 g/m2 orally tid, he developed pulmonary leukostasis which responded rapidly to whole-lung radiation with 1.5 Gy in one fraction. Pulmonary leukostasis in hyperleukocytotic AML is an oncologic emergency demanding rapid intervention. The combined treatment with chemotherapy and radiation appears useful.
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6/9. Fatal complement-induced leukostasis after diatrizoate injection. Principles of clinicopathologic diagnosis.

    A case of fatal radiographic contrast medium (RCM)-induced pulmonary granulocyte aggregation (leukostasis) showed massive elevation of the postmortem histamine level. Suspicion of this phenomenon in diverse clinical settings permits clinical and pathological documentation via increased plasma levels of C3a and other means. The existence of effective prophylaxis stimulates a search for predictive tests.
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keywords = leukostasis
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7/9. Pulmonary leukostasis in fatal human pneumococcal bacteremia without pneumonia.

    An asplenic man developed fulminant pneumococcal bacteremia without pneumonia. He died of irreversible shock within 24 h. autopsy revealed extensive pulmonary vascular leukostasis. This condition has been described in laboratory animals after intravascular challenge with endotoxin, gram-negative bacilli, and gram-positive organisms including pneumococci. This case illustrates that death in pneumococcal disease can occur in the absence of pneumonia and may be attributable to cardiovascular collapse. We present a proposed mechanism based on activation of complement and release of vasoactive mediators.
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keywords = leukostasis
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8/9. Respiratory failure due to pulmonary leukostasis following chemotherapy of acute nonlymphocytic leukemia.

    Four patients with acute nonlymphocytic leukemia and leukocyte counts of more than 200,000/mm3 developed respiratory distress due to pulmonary leukostasis within 10-48 hours after initiation of chemotherapy. Clinically, the patients manifested fever, dyspnea, tachypnea, diffuse pulmonary rales, pleural effusions, and severe hypoxemia. Chest roentgenograms displayed diffuse pulmonary infiltrates, vascular engorgement, cardiomegaly, and pleural effusions. Three patients died from progressive respiratory failure despite ventilatory support. Pulmonary histology revealed thrombi composed of leukemic blast cells which obstructed and distended the lumens of pulmonary arterioles, capillaries, and venules. Electron microscopy studies of lung tissue showed pulmonary alveolar endothelium and basement membrane damage and interstitial edema. The pathophysiologic basis of pulmonary leukostasis and potential treatment modalities are discussed.
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9/9. Activation of plasma complement by perfluorocarbon artificial blood: probable mechanism of adverse pulmonary reactions in treated patients and rationale for corticosteroids prophylaxis.

    Perfluorocarbons have shown promise as clinical blood substitutes. Although early experience in japan with one such product--Fluosol-DA--has been uncomplicated, we observed an adverse pulmonary reaction in the first American patient to receive it and know of similar reactions in two other Americans so treated. Postulating that activation of plasma complement (C) by the perfluorocarbon emulsion might have caused the reaction, we tested the product to determine if it is an activator of complement. Incubation of Fluosol with plasma led to C3 conversion, decrement in CH50, and generation of C5a-related PMN aggregating activity; EDTA prevented such activation, while EGTA did not, suggesting that it proceeded via the alternative C pathway. Infusion of Fluosol into rabbits produced hypoxemia, neutropenia, thrombocytopenia, and pulmonary leukostasis, mimicking abnormalities previously demonstrated in rabbits receiving infusions of zymosan-activated plasma C. These deleterious responses to Fluosol were diminished by premedicating rabbits with corticosteroids (which had seemed to benefit when used empirically in our patient). in vitro and in vivo, Fluosol's effects were reproduced by Pluronic F-68, the nonionic detergent used to maintain the emulsion stability of Fluosol-DA. We conclude that adverse reactions to Fluosol are probably mediated by C activation and that steroid premedication may prevent them in susceptible patients.
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keywords = leukostasis
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