Summary:Case reportA 4-year-old boy was first noted to have severe anemia at A 4-year-old boy with Diamond-Blackfan anemia and a history of multiple transfusions underwent umbilical 1 month of age. An evaluation showed hemoglobin 50 g/l, white blood cell count 5.5 × 10 9 /l with normal distribution, cord blood transplantation from his HLA-identical female sibling born by vaginal delivery at 38 weeks. The platelet count 385 × 10 9 /l, and 0% reticulocyte. The bone marrow showed marked erythroid hypoplasia with no patient was prepared with busulfan, cyclophosphamide and antilymphocyte globulin. Methotrexate and cycloabnormal cells and a diagnosis of Diamond-Blackfan anemia was confirmed. He was 2680 g at birth, the product sporin A were given for the prophylaxis of GVHD. Regimen-related toxicity was not observed and successful of a full-term, uncomplicated gestation. The parents were healthy and there was no consanguinity or family history engraftment occurred, including the erythroid series. No evidence of acute or chronic GVHD has been of hematological disorders. Several therapeutic approaches including intravenous high-dose methylprednisolone and observed for 14 months after transplantation. This is the first case of successful umbilical cord blood transoral corticosteroids had been tried. However, they had only a transient effect on the progressive anemia, and the patient plantation to a patient with Diamond-Blackfan anemia. Keywords: cord blood transplantation; Diamondbecame steroid-resistant and transfusion-dependent. He received 60 units of filtered and irradiated packed red cells Blackfan anemia; childhood; hemosiderosis before transplantation without any iron-chelating therapy. His HLA-type was A24, 31; B51, 52; DR15(2), 4; DQ1, 4, and not identical to the parents and a sibling (father: Diamond-Blackfan anemia is a disorder of childhood A31, 33; B44, 52; DR15(2), 8; DQ1, mother: A24, 33; B44, characterized by normochromic-macrocytic anemia, reti-51; DR4, 13; DQ1, 4, brother: A31, 33; B44, 52). culocytopenia and a normocellular marrow with a selective At 3 years of age, he displayed signs of iron overload deficiency of erythroid progenitors. Most patients respond including elevations of serum transaminase, iron and ferrito corticosteroids initially. However, about 60% require tin. CT scan showed a marked increase in the density of chronic red cell transfusion and die in the second and third the liver parenchyma, which was consistent with liver decade of life as a result of hemosiderosis. 1 Allogeneic hemosiderosis. Echocardiography revealed mild left ven-BMT is already accepted as effective therapy for steroidtricular dilatation and grade II mitral regurgitation with norresistant, transfusion-dependent patients with Diamondmal wall mobility. His mother became pregnant and a girl Blackfan anemia. 2-5 However, the limited availability of was delivered vaginally at 38 weeks without any complisuitable donors remains a problem. Recently, unbilical cord cations. At the time of delivery, 70 ml of cord blood was b...
A 67-year-old womanwith pneumoniaand diabetes mellitus was admitted with the complaints of abdominal and back pain. Sputum culture was positive for Klebsiella pneumoniae. Computed tomographic scanning (CT) of the abdomen and spinal radiograph of the lumber column revealed a paraventebral spaceoccupying lesion, abdominal aortic aneurysm and destructive change of L3 and L4. Pseudoaneurysm of the abdominal aorta associated with infectious spondylitis with paravertebral abscess was suspected and confirmed by aortography. Klebsiella pneumoniae was cultured from the abscess. The patient's condition improved rapidly after drainage of the abscess and administration of LMOX and gentamicin. Infectious pseudoaneurysm of the abdominal aorta associated with infectious spondylitis has rarely been reported. These two in combination due to Klebsiella pneumoniae has not been reported to our knowledge. The pathologic changes were found easily by CTscan. Wheninfectious aneurysm or infectious spondylitis is diagnosed alone, possible combination of these diseases should be kept in mind.Key words: Infectious Aortic Pseudoaneurysm due to Klebsiella pneumoniae, Infectious spondylitis due to Klebsiella pneumoniae.A case of infectious pseudoaneurysm of the abdominal aorta associated with infectious spondylitis due to Klebsiella pneumoniae is presented. These conditions were easily diagnosed by sputum culture, abscess culture, spinal radiograph, abdominal CT scan and aortography. The patient was eventually treated surgically and her condition has been good. CASE REPORTA 67-year-old woman was admitted to this hospital on March ll, 1987, because of fever, yellowish productive cough, pain in the abdomen, back and lumbar region and uncontrolled diabetes mellitus.Fever and productive cough started about two weeks prior to admission. She was put on antibiotics by a local physician. Pain in the abdomen, back and lumbar region started about a week previously and had worsened progressively.Physical examination revealed a temperature of 37.8°C. Moist rales were heard over the right lung field. Abdomenrevealed diffuse tenderness without rebound tenderness. There was no palpable pulsatile mass in the abdomen. There was mild percussion tenderness over the lumbar spine.Laboratory results included a white blood cell count of 9,000 with a differentiation of 60%stab and 30%segments. Erythrocyte sedimentation rate was 90 mmin one hour and 120 mmin two hours.Fasting blood sugar was 477 mg/dl. A chest
Cord blood transplantation (CBT) has been increasingly used to treat patients with hematological diseases, but active immunizations for patients have not been described. Patients certainly need immunizations following CBT, since transplanted cord blood is naive. The authors previously reported successful hematopoietic reconstitution following cord blood transplantation from an HLA-matched sibling in a transfusion-dependent child with Diamond-Blackfan anemia. No graft-versus-host disease, either acute or chronic, has been observed so far. Here, the authors report that immunological recovery of the patient has been rapid shortly after CBT and immunization has been done successfully. Vaccines (diphtheria, pertussis, tetanus, rubella, measles, and BCG) were administered during 22-34 months post-transplant. Seroconversion to these vaccines was excellent without significant adverse effects. These results indicate that both toxoid and live vaccines have been safely administered in the patient who underwent related cord blood transplantation.
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