Liver Transplantation After Hematopoietic Stem Cell Transplant for the Treatment of Sickle Cell Disease: A Case Report.

Sickle cell anemia is the most common of the hemoglobinopathies, in which the abnormal hemoglobin formed in deoxygenation states undergoes a polymerization process with consequent erythrocyte deformation and vaso-occlusive events. The need for multiple blood transfusions, prolonged ineffective erythropoiesis, hemolysis, and increased iron absorption can cause iron overload in the liver, leading to liver fibrosis. Hematopoietic stem cell transplantation (HSCT) is currently the only treatment with a curative potential for this disease and can establish normal complete or partial donor-derived erythropoiesis and stabilize or restore function in affected organs, preventing further deterioration of function. However, it does not reverse preexisting liver fibrosis and siderosis. One of the possible complications of patients who undergo HSCT is chronic liver disease, which has a multifactorial cause, with iron overload being an important factor. In the long term, the prevalence of chronic liver disease in HSCT patients, including cirrhosis and its complications, can be significant. Solid organ transplantation after allogeneic hematopoietic cell transplantation for end-organ failure remains a very rare event. It may offer a valuable treatment strategy in selected recipients, although it is associated with significant morbidity and mortality. We report the case of a patient with sickle cell anemia who underwent HSCT and developed severe liver dysfunction requiring liver transplantation 13 years after the procedure. We found no previous report in the literature of orthotopic liver transplant after HCT for the treatment of sickle cell disease.

Bullet embolism to the heart secondary to gunshot wound of the left subclavian vein: A case report.

Missile embolism as a consequence of gunshot wounds is a rare occurrence, and can lead to severe complications such as endocarditis, pulmonary thromboembolism and arrythmias. The correct diagnosis of bullet embolism can be challenging in an emergency care setting, often requiring a combination of clinical, radiological and surgical resources. The management of a venous missile embolism depends on characteristics such as size and location of the projectile, and must be highly individualized for each patient. In this report, a case of bullet embolism to the heart in a patient who suffered a gunshot wound to the left subclavian vein provides a backdrop for the discussion of the diagnosis and treatment of this rare ballistic injury.

Combined Liver and Kidney Transplant in Acute Intermittent Porphyria: A Case Report.

BACKGROUND Acute intermittent porphyria is an inherited disease caused by a defect in heme biosynthesis, with accumulation of neurotoxic metabolites leading to acute neurovisceral symptoms. Some patients develop long-term neurological and renal damage after the acute episodes, many of them requiring hemodialysis. Since heme production in the human body occurs predominantly in the bone marrow and liver, liver transplantation has been shown to significantly reduce the production of neurotoxic metabolites, effectively controlling the disease. Patients with severe acute intermittent porphyria who have chronic kidney failure may benefit from combined kidney and liver transplant. Only 2 uses of this approach have been previously reported in the literature. CASE REPORT We report here the case of a 19-year-old male patient who received a combined liver and kidney transplant for the treatment of acute intermittent porphyria. He presented the first symptoms of the disease 4 years before the procedure, with abdominal pain and significant neurological impairment, with weakness requiring prolonged mechanical ventilation. He also had chronic kidney failure secondary to the porphyria. A combined liver and kidney transplant was performed, with no intraoperative complications. The explanted liver showed light siderosis, as well as portal and perisinusoidal fibrosis at microscopy. At 3.5 years of follow-up, he remains clinically well, with normal hepatic and renal function, had had no further acute porphyria episodes, and shows progressive neurological recovery. CONCLUSIONS This case demonstrates that combined liver and kidney transplant can be a curative treatment for patients with severe acute intermittent porphyria associated with end-stage renal failure. The patient shows satisfactory long-term function of both grafts, with no clinical or biochemical signs of porphyria recurrence.

Tacrolimus-Associated Psychotic Disorder: A Report of 2 Cases.

Psychotic disorders are a group of psychiatric disorders characterized by the presence of delusions, hallucinations, bizarre behavior, and disorganized speech. There are several possible causes for the occurrence of psychotic disorders in patients who underwent solid organ transplant, including pre-existing mental illness, electrolyte disturbances, infections of the central nervous system, and adverse reaction to drugs. Calcineurin inhibitors are a class of immunosuppressive drugs, such as tacrolimus and cyclosporine, that are currently considered the mainstay in the immunosuppressive drug regimen of patients who underwent solid organ transplant. Neurotoxicity is one of the adverse reactions associated with the use of calcineurin inhibitors, ranging from upper limb tremors to psychotic disorders and seizures. We report the cases of 2 liver transplant recipients who developed severe psychotic disorder 1 month after the procedure. After an extensive investigation for other possible triggers of psychiatric disease, the use of tacrolimus was considered to be the most likely cause for the acute psychotic disorder. In less than 24 hours after suspension of that drug, all symptoms disappeared in both patients, making a causal relationship with tacrolimus even more likely. The patients were then given cyclosporine, another drug from the same class, allowing for adequate immunosuppression and preserved graft function, with no further psychiatric symptoms. This report confirms that a 24-hour trial of tacrolimus suspension can be safe and effective in the diagnosis of drug-related psychotic disorders in patients who underwent liver transplant. This article is compliant with the Helsinki Congress and the Istanbul Declaration.

Visceral Leishmaniasis in a Liver Transplant Patient: A Case Report.

Leishmaniasis is an infection caused by protozoa of the genus Leishmania, transmitted by sandflies and endemic to more than 88 countries. Visceral leishmaniasis in immunosuppressed patients is a growing concern. We report the case of a 61-year-old male patient with a previous history of alcoholic cirrhosis and portal vein thrombosis who underwent liver transplantation for the treatment of hepatocellular carcinoma. Thirty-six days after the procedure, the patient showed an increase in liver enzymes and was diagnosed with moderate acute rejection of the graft. He was treated with high-dose intravenous corticosteroids, and while showing improvement in biochemical markers, he became febrile 12 days after corticosteroid treatment. He presented daily episodes of fever, even after the use of several antimicrobial, antiviral, and antifungal agents, and a number of negative cultures from different sites were obtained. A bone marrow biopsy was then performed, showing a large number of amastigote forms of Leishmania spp. Treatment with liposomal amphotericin B was initiated; however, the patient progressed to refractory septic shock and death. This case highlights several aspects of visceral leishmaniasis in liver transplant recipients, such as the association of malnutrition to Leishmania infection and the challenges of diagnosing leishmaniasis in cirrhotic patients in which splenomegaly and pancytopenia, the hallmarks of leishmaniasis, may also be attributed to portal hypertension and end-stage liver disease. A high index of suspicion is necessary for the correct diagnosis and treatment of leishmaniasis in this group of patients. This study is compliant with the Helsinki Congress and the Istanbul Declaration.

Extensive Inferior Vena Cava Thrombosis in a Liver Transplant Patient: A Case Report.

Thrombosis of the inferior vena cava is a clinical condition with very diverse presentations, ranging from asymptomatic patients to others with severe edema in the legs and lower torso. We report the case of a 27-year-old female patient, previously diagnosed with autoimmune hepatitis, with asymptomatic extensive thrombosis of the inferior vena cava. The thrombus extended from the renal veins up to the emergence of the hepatic veins, causing post-sinusoidal portal hypertension (Budd-Chiari syndrome). The patient underwent an orthotopic cadaveric liver transplant with removal of the retrohepatic vena cava and thrombectomy of blood clots from the infrahepatic vena cava. She initially recovered well from surgery, but on the 8 postoperative day she had a significant increase in hepatic injury markers and was diagnosed with rethrombosis of the inferior vena cava and hepatic veins. A surgical thrombectomy was performed, with an intraoperative finding of chronic thrombus in both renal veins, previously undiagnosed. The thrombectomy was successful, but the patient's hepatic function continued to worsen and a second liver transplant was performed. After the second transplant she underwent several imaging exams that showed no signs of rethrombosis. She was kept on postoperative anticoagulation indefinitely, first with intravenous heparin then with rivaroxaban. An extensive investigation failed to identify any causes of thrombophilia associated with this vast thrombosis. She is currently alive and with good graft function 1 year and 4 months after the second transplant.