No abstract

Three patients diagnosed as having remitting seronegative symmetrical synovitis with pitting edema syndrome, pemphigus erythematosus and idiopathic interstitial pneumonia were treated with oral prednisolone. Several weeks after starting the treatment, they experienced repeated chest pain attacks between midnight and early morning, although none of the patients had a past history of ischemic heart disease. One of the patients exhibited aggravation of symptoms soon after increasing the dose of prednisolone. A definitive diagnosis of vasospastic angina was made using electrocardiograms, coronary angiography and vasospasm provocation tests. These cases emphasize that clinicians should be aware of the possible occurrence of vasospastic angina following the initiation of corticosteroid therapy.

We describe the novel karyotype of a 33-year-old woman with severe mental retardation and multiple cardiac anomalies, including patent ductus arteriosus, a ventricular septal defect, pulmonary atresia, and an overriding aorta. Her karyotype was 46, XX, add(17)(p13). The short arm of chromosome 17 was slightly elongated owing to the deletion of the distal portion of that chromosome and the addition of extra material from another chromosome. Miller-Dieker syndrome is characterized by a patent ductus arteriosus, lissencephaly, and the deletion of chromosome 17p13.3; however, as the patient's brain surface appeared normal on computed tomography, Miller-Dieker syndrome was excluded. The breakpoint in her chromosome 17 was probably located distal to band 17p13.3. In fact, fluorescence in situ hybridization analysis demonstrated that band 17p13.3 was intact. To date, genes distal to 17p13.3 have not been implicated in cardiac anomalies. This patient probably carries a novel deletion on the short arm of chromosome 17.

We report a case of a 45-year-old woman with Ehlers-Danlos syndrome (EDS) type IV, the vascular type, who presented with multiple coronary artery ruptures causing cardiac tamponade. She had sudden onset of chest pain soon after transarterial embolization for right carotid-cavernous fistula. Transthoracic echocardiography confirmed cardiac tamponade and hypokinetic inferolateral wall. Enhanced CT and transesophageal echocardiography ruled out aortic dissection. Coronary angiography showed contrast extravasation from multiple sites of the right coronary artery and left circumflex coronary artery. We suspected EDS type IV, and a skin biopsy for DNA and RNA analysis was done after taking written informed consent. Polymerase chain reaction (PCR) and sequencing of the PCR product showed a heterozygous missense mutation of codon 85 in the COL3A1 gene, which converted glycine to aspartic acid, and thus a diagnosis of EDS type IV was established. To our best knowledge, this is the first case of EDS type IV causing multiple coronary artery ruptures.

BACKGROUND Isohemagglutinins against ABO antigens absent on both recipient and donor red blood cells (RBCs) increase or decrease after ABO‐incompatible hematopoietic stem cell transplantation (HSCT). However, few reports have described the changes in the isohemagglutinin titers and the characteristics in patients with recurrent hematologic conditions after ABO‐incompatible HSCT. CASE REPORT A 59‐year‐old female with acute erythroid leukemia received a peripheral blood stem cell transplant from her HLA‐haploidentical daughter. The patient was typed as group O with anti‐ A (4+) and B (4+) isohemagglutinins, while the donor was typed as group B. The bone marrow cells achieved complete donor cell chimerism on Day 13 after HSCT. On Day 120, the patient showed 97% B RBC type with persistent anti‐A (3+) and without anti‐B antibodies. On Day 375, her leukemia relapsed, and recipient type O RBCs and anti‐B antibodies sequentially reemerged. However, clinicolaboratory hemolysis and erythroid aplasia were not detected in the patient. RESULTS The post‐HSCT sera agglutinated the allo B RBCs, but not the donor B RBCs, while the pre‐HSCT sera agglutinated both RBCs. The burst‐forming/colony‐forming units of erythroid formation from the donor peripheral blood stem cells were impaired by only the pre‐HSCT sera and not by the post‐HSCT sera. CONCLUSION To our knowledge, this is the first report investigating the characteristic changes of isohemagglutinins between the pre‐ and post‐HSCT sera in a patient with recurrent acute myeloid leukemia. The present study suggests that the plasma cells producing anti‐donor B RBCs in the patient have been selectively eliminated or induced into an anergic state by the post‐HSCT immunologic reconstruction.