Cases reported "Anemia, Aplastic"

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1/32. hepatopulmonary syndrome after allogeneic bone marrow transplantation.

    A 10-year-old girl with aplastic anemia received an allogeneic bone marrow transplantation (BMT). Three years after an uneventful course apart from chronic graft-versus-host disease (GVHD) she presented with chronic hypoxemia, reduced diffusion capacity of the lungs, normal spirometric lung function and increased bilirubin and liver enzymes. Intrapulmonary vascular dilatations were demonstrated. Pulmonary complications after BMT may include a hepatopulmonary syndrome (liver disease, hypoxemia, intrapulmonary vascular dilatations).
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keywords = lung
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2/32. Fatal acute hepatic failure induced by danazol in a patient with endometriosis and aplastic anemia.

    We describe a 47-year-old woman with severe aplastic anemia with genital bleeding who developed acute severe hepatitis after the administration of danazol while she was receiving cyclosporin. She had been diagnosed with severe aplastic anemia 1 year previously and, while hospitalized, had received methyl prednisolone pulse therapy, which was not successful. She was then referred to our hospital. She was treated with antithymocyte globulin, cyclosporin, granulocyte colony-stimulating factor, and methyl prednisolone; a good response was achieved after 3 months of this therapy. Subsequently, oral administration of cyclosporin was continued, but she was readmitted to our hospital when pancytopenia gradually developed and the genital bleeding recurred. danazol was administered for pancytopenia and endometriosis. Four days after the first administration of danazol, epigastric pain occurred, and the danazol was stopped. Eighteen days after the first danazol administration, very severe hepatic injury occurred abruptly. The patient died of hepatic failure. Postmortem examination revealed centrilobular massive necrosis of the liver. danazol was implicated as the agent responsible for causing the hepatic failure. drug interactions between danazol and cyclosporin may cause adverse effects.
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ranking = 0.60862383137119
keywords = injury
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3/32. Severe aplastic anemia associated with hepatitis and complicated by pulmonary aspergillosis: response to immune suppression and antifungal therapy.

    The true etiology of severe aplastic anemia is unknown; however, autoimmune activation of t-lymphocytes is one of the potential causes. stem cell transplantation is regarded as a first line therapy if a fully matched sibling is available. Immunosuppressive therapy is reserved for those who have no matched sibling available for transplant, or for those individuals who fall outside the age range eligible for stem cell transplantation. This case describes a child with a hepatitis-associated severe aplastic anemia for whom a fully matched sibling was available but a transplant was postponed due to active hepatitis. While awaiting bone marrow transplantation, the child acquired a life-threatening aspergillosis infection treated with amphotericin b, granulocyte infusions, and surgical resection of the involved lung. A decision was made to proceed with immunosuppressive therapy while waiting for successful treatment of the fungal infection. Following administration of equine anti-thymocyte globulin (ATG), high dose granulocyte colony stimulating factor (G-CSF), cyclosporine, and steroids, the child had partial hematopoietic reconstitution and is now followed in our clinic. This case demonstrates the extraordinary multidisciplinary care required during the early phases of treating severe aplastic anemia. With such care, recovery is a possibility.
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keywords = lung
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4/32. aspergillus niger infection in patients with haematological diseases: a report of eight cases.

    In this paper we analysed clinical, laboratory characteristics and outcome of patients with haematological diseases who developed an aspergillus niger infection, in a multicentre study involving 14 Italian Haematological Divisions during a 10-year period. The study recorded 194 consecutive microbiologically documented aspergilloses, eight of which (4%) were due to A. niger, and were observed only in five of the participating centres. The primary localization of infection was lung in seven cases and paranasal sinus in one case. Seven patients died at the end of follow-up. The death was mainly attributable to A. niger progression in six of them. Our study that collected the largest number of cases of A. niger infection in haematological malignancies confirms that this infrequent complication is characterized by a high mortality rate.
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keywords = lung
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5/32. A case of focal segmental glomerulosclerosis associated with aplastic anemia.

    The pathogenic mechanism of focal segmental glomerulosclerosis (FSGS) and aplastic anemia are associated with immunologic events which lead to glomerular cell injury or hematopoietic cell destruction. We present an extremely rare case of FSGS with aplastic anemia in a 30-yr-old woman. The laboratory examination showed hemoglobin 7.2 g/dL, white blood count of 4,200/ microL, platelet count 70,900/microL. proteinuria (2 , 3.6 g/day) and microscopic hematuria were detected in urinalysis. The diagnosis of FSGS and aplastic anemia were confirmed by renal and bone marrow biopsy. She was treated with immunosuppressive therapy of prednisone 60 mg/day orally for 8 weeks and cyclosporine A 15 mg/kg/day orally. She responded with gradually improving her clinical manifestation and increasing peripheral blood cell counts. prednisone was maintained at the adequate doses with tapering after 8 weeks and cyclosporine was given to achieve trough serum levels of 100-200 ng/mL. At review ten month after diagnosis and initial therapy, the patient was feeling well and her blood cell counts increased to near normal (Hb 9.5 g/dL, Hct 32%, WBC 8,300/microL, platelet 123,000/microL) and renal function maintains stable with normal range proteinuria (0.25 g/day).
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keywords = injury
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6/32. Flucloxacillin-induced aplastic anaemia and liver failure.

    Flucloxacillin is a commonly prescribed semisynthetic penicillinase-resistant penicillin primarily used for the treatment of cutaneous staphylococcal infections. It is well-recognized that flucloxacillin may occasionally result in fatal hepatic injury. We report the case of a 40-year-old woman who developed fulminant hepatic failure and aplastic anaemia following a course of oral flucloxacillin. At the time of transplantation the patient was severely neutropenic. Post-transplant, the patient received single donor leucocyte transfusions, which resulted in a dramatic increase in neutrophil count. The patient was discharged from hospital after 120 days with normal liver function and recovered bone marrow. In this report, we discuss the care of patients with aplastic anaemia in the peritransplant setting.
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ranking = 0.60862383137119
keywords = injury
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7/32. diagnosis of transfusion-related acute lung injury: TRALI or not TRALI?

    TRALI is a challenging diagnosis for both the transfusion specialist and the clinician. A Canadian consensus panel has recently proposed guidelines to better define TRALI and its implications. The guidelines recommend classifying each suspected case in one of the following 3 categories: (1) "TRALI," (2) "Possible TRALI," or (3) "Not TRALI." We report the clinical presentation, laboratory evaluation, and management of 3 patients with respiratory failure (RF) following allogeneic blood transfusions. These patients all experienced RF within 6 hr post-transfusion. Based on a review of the clinical and laboratory data and applying the Canadian guidelines, the first patient, a 67-yr-old man with chronic myelomonocytic leukemia, was diagnosed as "TRALI" due to the sudden onset of RF requiring intensive resuscitation. The second patient, a 55-yr-old man with aplastic anemia, was diagnosed as "Possible TRALI" due to pre-existing RF that worsened after blood transfusion. The third patient, a 1-yr-old male, was diagnosed as transfusion associated circulatory overload (TACO) and "Possible TRALI," although his RF improved after treatment with diuretics. In all 3 cases, the blood donor center was informed of the suspected TRALI reactions. The remaining blood products from the donors associated with these reactions were quarantined. After review of the clinical data, the donors associated with cases #1 and #3 were screened by the blood center for granulocyte and HLA antibodies. Using a Luminex flow bead array, the following class I and class II antibodies specific for patient #1 were identified in the respective donor: anti-A25, B8, B18, and anti-DR15, DR 17. Subsequently, donor #1 was permanently deferred. A non-specific IgM anti-granulocyte antibody was identified in the donor associated with case #3, and this donor was subsequently disqualified from plasma and platelet donations. In conclusion, the Canadian guidelines to categorize patients suspected of TRALI provide a useful framework for evaluation of these patients and their respective blood donors.
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ranking = 14067.577570983
keywords = acute lung injury, lung injury, acute lung, injury, lung
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8/32. pulmonary aspergillosis complicated by osteomyelitis.

    We report on a patient with aplastic anaemia and an invasive aspergillosis of the lung with subsequent osteomyelitis of the ribs. diagnosis was made by puncture of the soft tissues and isolation of aspergillus fumigatus. Treatment with amphotericin b induced renal function disturbances. It was successfully replaced by AmBisome. Healing occurred with recovery of the immunity of the patient. The literature on Aspergillus osteomyelitis is reviewed.
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ranking = 0.5
keywords = lung
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9/32. Successful combined antifungal salvage therapy with liposomal amphothericin B and caspofungin for invasive aspergillus flavus infection in a child following allogeneic bone marrow transplantation.

    The emergence of new antifungal compounds with alternative mechanisms of action and improved tolerability has opened up new therapeutic possibilities for the use of combined antifungal treatment in life-threatening systemic fungal infections. A case report of an 8-year-old allogeneic stem cell transplant recipient who developed a central venous catheter tunnel infection caused by aspergillus flavus is presented here. In spite of conventional and subsequent liposomal amphotericin b therapy the infection progressed rapidly and the necrosis extended further to the thoracic wall, pleura and the right lung. Combined treatment consisting of liposomal amphotericin b and caspofungin was instituted. After 30 days of dual therapy the deep fungal infection resolved and the extensive soft tissue defect showed scarring. One year post-transplant, the patient is well, with normal bone marrow function and full donor chimerism. Although there is limited clinical data on the effectiveness of echinocandins in pediatric patients with documented invasive fungal infections, this case report shows that combining liposomal amphotericin b with caspofungin could be advantageous.
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keywords = lung
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10/32. Anabolic steroid therapy and intrahepatic cholangiocarcinoma.

    A 47-year-old man who had been treated with an anabolic steroid for refractory anemia developed an intrahepatic cholangiocarcinoma with metastases to abdominal lymph nodes and lungs. Microscopically, the tumor showed mucin production and was devoid of hepatocellular elements. Previous reports have suggested a possible relationship between anabolic steroid therapy and hepatocellular carcinoma. In many such cases, there are doubts about the histological diagnosis, malignant potential, and the nature of the association between the steroids and the tumors. The presence of distant metastases attests to the malignant nature of the tumor in the present case. Despite the apparent temporal eligibility of the steroid as an etiologic agent, a causative relationship between therapy and tumor is not established.
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keywords = lung
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