[Acute promyelocytic leukemia recurrence diagnosed due to granulocytic sarcoma of the external auditory canal].

A 47-year-old man was diagnosed with acute promyelocytic leukemia (APL) accompanied by pancytopenia and left forearm swelling. Complete remission was achieved with remission induction therapy using all-trans retinoic acid (ATRA), and consolidation therapy was completed. Three months after the treatment, left ear closure was observed, and a mass lesion was found in the left external auditory canal. An initial tumor biopsy only revealed inflammatory cell infiltration. Moreover, the tumor's rebiopsy performed 3 months later revealed MPO-positive and CD68-positive granulocyte infiltration. Furthermore, the rebiopsy revealed 4.9×105 copies/µgRNA of PML/RARα, the patient was diagnosed with locally recurrent APL. A bone marrow examination 2 weeks later confirmed an increase in myeloblasts and promyelocytes for the first time since the confirmation of remission. Therefore, it was diagnosed as bone marrow recurrence. Reinduction therapy using ATRA and arsenic trioxide again led to complete remission, after which autologous peripheral blood stem cell transplantation was performed. Currently, complete remission is being maintained. In this case, the recurrence of the external auditory canal lesion preceded the bone marrow recurrence. Therefore, it is important to note the nonspecific leukemia recurrence patterns of the external auditory canal.

Perisurgical induction of eculizumab in a patient with paroxysmal nocturnal hemoglobinuria: its inhibition of surgery-triggered hemolysis and the consequence of subsequent discontinuation.

Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by complement (C')-induced lysis of PNH red blood cells (RBCs), which are deficient in the expression of CD55 and CD59. Surgery is one of the major clinical situations that trigger hemolytic attack and thrombosis in PNH. We describe here a case of 64-year-old man with classic PNH complicated by early-stage gastric cancer requiring distal gastrectomy under general anesthesia. We administered humanized monoclonal anti-C5 antibody (eculizumab; Soliris) for a limited period (600 mg, once a week × four times) perisurgically. Eculizumab effectively inhibited the C' system and the patient underwent a curative distal gastrectomy without significant surgery-triggered hemolytic attack. Although discontinuation of eculizumab induced mild hemolysis 2 weeks after the last administration, it was treated conservatively without thrombotic complication. Limited-term induction of eculizumab could be an option for PNH patients with transient and anticipated high risks, with careful preparation for the discontinuation-related risks afterwards.

Post-transplant gastric antral vascular ectasia after intra-venous busulfan regimen.

Gastric antral vascular ectasia (GAVE) is an angiodysplastic disorder that causes gastric bleeding. GAVE can develop as a complication of hematopoietic stem cell transplantation (HSCT-GAVE), and it has been suggested that it may be associated with oral administration of busulfan. We report two cases of HSCT-GAVE after a conditioning regimen containing intra-venous busulfan (ivBu), not oral busulfan. The first case, a 42-year-old woman with blastic plasmacytoid dendritic cell neoplasm, underwent second allogeneic HSCT with conditioning regimen consisting of cyclophosphamide (120 mg/kg) and ivBu (12.8 mg/kg). HSCT-GAVE developed on day 84 post-transplant, and argon plasma coagulation (APC) was performed successfully. The second case, a 60-year-old woman with acute myelogenous leukemia, underwent allogeneic HSCT with the conditioning regimen consisting of ivBu (12.8 mg/kg) and fludarabine (150 mg/kg). She developed melena and was diagnosed with GAVE by endoscopy on day 145 post-transplant. Although complete hemostasis was not achieved despite four administrations of APCs, the melena spontaneously terminated on day 235 post-transplant. To our knowledge, this is the first report describing HSCT-GAVE after ivBU-based HSCT. Although there is no established therapy for HSCT-GAVE, APC may be an option for HSCT-GAVE.

[Autoimmune hemolytic anemia in a patient with idiopathic interstitial pneumonia].

We report a rare case of autoimmune hemolytic anemia (AIHA) complicated by idiopathic interstitial pneumonia (IIP). A sixty-year-old man was diagnosed as having IIP in January 2009. In March, when he was hospitalized for the introduction of home oxygen therapy, severe anemia was detected. Based on the findings showing elevated levels of lactate dehydrogenase and indirect bilirubin, a decreased level of haptoglobin, positive Coombs test, and splenomegaly, a diagnosis of AIHA was made. Although anti-DNA antibody was found, diagnostic criteria for systemic lupus erythematosus and other collagen diseases were not fulfilled. Therefore, we concluded that AIHA coexisted with IIP. Treatment with prednisolone led to improvement of both AIHA and IIP. There has not been any exacerbation even after a gradual reduction of prednisolone to 7.5 mg/day. Coexistence of AIHA and IIP is rare, and accumulation of case reports is needed to gain a better understanding of this condition.