We herein report a rare chromosomal abnormality observed in an acute promyelocytic leukemia (APL) patient. She had several APL derivative clones including a clone with i(17)(q10) abnormality, which consists of two kinds of structural abnormalities, a cryptic translocation of t(15;17) and an isochromosome of 17q. Although an obvious microscopic t(15;17) change was not observed on either arms of the isochromosome, PML/RARα fusion signals were detected on an interphase fluorescence in situ hybridization analysis. By several cytogenetic analyses of her bone marrow cells, it was confirmed that the i(17)(q10) clone was derived from the classic t(15;17) clone via another intervening clone, cryptic t(15;17).
Acquired haemophilia (AH) is a rare but serious bleeding disease caused by the spontaneous development of autoantibodies against coagulation factors. Acquired haemophilia A (AHA) refers to the condition wherein autoantibodies developed against factor VIII (FVIII) [1]. The incidence of AHA is 0.2-1 per million per year [1]. Acquired FVIII inhibitors are found in patients with autoimmune disorders, malignancies, diabetes, chronic liver diseases, dermatological disorders and in pregnant women [1]. Almost 50% of cases with the inhibitor have no underlying disorders [1]. Some studies have reported cases of development of FVIII inhibitors after the life-threatening infections, such as sepsis [2][3][4]. Herein, we report a case of acquired FVIII inhibitor development after peritonitis caused by Gram-negative rods in a patient who underwent distal gastrectomy.An 84-year-old man was admitted to our hospital to undergo distal gastrectomy for the treatment of early gastric adenocarcinoma, which was preoperatively diagnosed as stage 1a (cT1N0M0). This type of gastric cancer was not found to be eligible for endoscopic submucosal dissection. He had a history of adenocarcinoma of the ascending colon, which was resected 11 years earlier, with no recurrence. Physical examination did not show any palpable tumours, lymphadenopathy or bleeding. The results of a blood test, including complete blood count, liver or renal function, activated partial thromboplastin time (aPTT; 27.5 s; normal, 30-40 s) and prothrombin time (PT; 12.9 s; normal, 10-13 s), performed on admission were almost normal. The clinical course is shown in Fig. 1. Distal gastrectomy was performed and the carcinoma was completely resected. The patient complained of abdominal pain and then showed a life-threatening drop in blood pressure with purulent exudate from the drainage tube 2 days after the surgery. The levels of Creactive protein and fibrinogen degradation products (FDP) (21.6 mg dL À1 and 53.1 lg mL À1 respectively) increased, and the PT time (16.4 s) was prolonged. An anastomotic leakage from the duodenal stump was confirmed on reoperation, and new drainage tubes were indwelled. A diagnosis of peritonitis and disseminated intravascular coagulopathy was made, and doripenem and thrombomodulin were administered. Gramnegative rods (Enterobacter cloacae and Acinetobacter baumannii) were detected in the culture of perioperative ascites. The purulent exudate continued for 32 days after the gastrectomy. He also experienced aspiration pneumonia by E. cloacae, which was treated with cefmetazole and tazobactam/piperacillin (TAZ/ PIPC). Because the aPTT began to be prolonged 40 days after the gastrectomy, the patient was referred to our department when he recovered from the peritonitis and pneumonia. The aPTT was markedly prolonged (121.9 s), whereas the platelet count, PT and FDP were normal (23.1 9 10 4 lL À1 , 13.3 s and 2.6 lg mL À1 respectively). An aPTT cross-mixing study failed to correct the prolongation and the curve faced upward after incubation at 37°C for 2 ...
Background Life-threatening cytomegalovirus infection (CMVI) has been reported even in patients with malignant lymphoma (ML) who have not received hematopoietic stem cell transplantation (w/o HSCT) but had been treated with chemotherapy or radiotherapy. However, the CMVI incidence and risk factors (RFs) in patients with ML w/o HSCT have not been fully elucidated. This study aimed to evaluate the clinical aspects, including incidence and RFs, of CMVI in patients with ML w/o HSCT. Methods We retrospectively reviewed all patients with ML who received chemotherapy or radiotherapy in our department from 2005 to 2013. The overall survival (OS), incidence and RFs of CMVI, and other characteristics of patients with CMVI were analyzed. Results Overall, 236 patients with ML w/o HSCT were evaluated. Of these, 5.5% (13/236) developed CMVI; 54% (7/13) received steroid pretreatment before primary therapy (PT) for ML; and 62% (8/13) received > 2 therapeutic regimens for ML. The OS curve of patients with CMVI was significantly worse than that of patients without CMVI (p < 0.0001, log-rank test). A univariate analysis identified B symptoms (p = 0.00321), serum albumin < 3.5 g/dL (p = 0.0007837), C-reactive protein level > the upper limit of normal (p = 0.0006962), steroid pretreatment before PT for ML (p = 0.0004262), > 2 therapeutic regimens for ML (p = 0.0000818), T cell lymphoma (p = 0.006406), and non-complete remission (p = 0.02311) as RFs for CMVI. A multivariate analysis identified steroid pretreatment before PT for ML [odds ratio (OR): 4.71 (95% confidence interval [CI]: 1.06–21.0); p = 0.0419] and > 2 therapeutic regimens for ML [OR: 9.25 (95% CI: 2.33–36.8); p = 0.00159] as independent RFs for CMVI in patients with ML w/o HSCT. Conclusions Attention should be paid to CMVI development in patients with ML w/o HSCT pretreated with steroids or who had multiple therapeutic regimens.
Introduction: Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against factor VIII (FVIII). Hematological malignancies, especially lymphoid malignancies, are known to be underlying causes of AHA; however, thus far, there is no report of AHA associated with Epstein–Barr-virus-associated T/natural killer-cell lymphoproliferative disease (EBV-T/NK-LPD). Here, we present a case of AHA that developed during treatment for EBV-T/NK-LPD. History: A 69-year-old man visited our hospital because of general fatigue. Blood examination showed pancytopenia, and computed tomography revealed whole-body lymphadenopathy, but there were no findings indicating hematological malignancy from bone marrow aspiration and cervical lymph node biopsy. The level of EBV DNA in peripheral blood was extremely high, and he was diagnosed with EBV-T/NK-LPD. EBV-T/NK-LPD improved with prednisolone (PSL) administration. Seventeen months after starting treatment, the patient complained of back and right leg pain. At that time, he had been treated with low-dose PSL, and EBV-T/NK-LPD was well controlled. Imaging revealed hematoma of the right iliopsoas muscle. Prolonged activated partial thromboplastin time (APTT) was the only abnormal finding in a screening coagulation test. FVIII coagulant activity was below detection limit, and FVIII inhibitor level was increased. From these results, he was diagnosed with AHA. A higher dose of PSL was administered, and, after 1 month of treatment, FVIII activity gradually increased, and FVIII inhibitor level became undetectable. APTT also normalized, and complete remission was achieved and maintained for 13 months with low-dose PSL. During treatment, EBV-T/NK-LPD was well controlled. Conclusion: It is speculated that proliferating lymphocytes interfere with normal immune functions and that abnormal autoantibodies are produced from those lymphocytes in patients with LPD. Therefore, we speculate that EBV-infected and proliferating monoclonal NK cells might have modulated the immune system and produced autoantibodies against FVIII, thus causing AHA in this patient with EBV-T/NK-LPD.
後 天 性von Willebrand症 候 群(acquired von Willebrand syndrome:AvWS)は先天性von Wil-lebrand病(von Willebrand disease:vWD)と類 似した症状を示す病態であり, von Willebrand因 子(von Willebrand factor:vWF
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