Given these findings, we recommend that HCVab is measured upon diagnosis of chronic ITP, and that splenectomy is planned in patients with HCVab in the event that prednisolone treatment is ineffective.

Although the introduction of imatinib dramatically improved the outcomes for patients with Philadelphia chromosome-positive B-cell precursor acute lymphoblastic leukemia (Ph + BCP-ALL), the survival benefit of imatinib has not been assessed in the context of Ph + mixed phenotype acute leukemia (Ph + MPAL). To clarify this important issue, we studied 42 Ph+ acute leukemia (Ph + AL) patients who received intensive chemotherapy and concurrent administration of imatinib. Of the 42 Ph + AL patients, 13 (31%) patients were categorized as Ph + MPAL (positive for both myeloid and B-cell lineage), 27 (64%) were categorized as Ph + BCP-ALL, and two (5%) were categorized as Ph + acute myeloid leukemia. The complete remission rates after the initial induction therapy were not significantly different when comparing Ph + MPAL and Ph + BCP-ALL patients (100% vs. 85%, respectively, P = 0.14). Likewise, there were no significant differences in the 5-yr overall survival (OS) or disease-free survival (DFS) rates when comparing the MPAL and BCP-ALL groups (OS: 55% vs. 53%, respectively, P = 0.87, DFS: 46% vs. 42%, respectively, P = 0.94). These findings suggest that concurrent imatinib administration with chemotherapy improved the outcomes of Ph + MPAL patients to the level seen in Ph+BCP-ALL patients and should, therefore, be considered as the standard therapy for these patients.

To investigate the clinical significance of granulocytic sarcoma (GS) in adults with acute myeloid leukemia (AML), 434 consecutive patients with AML were analyzed retrospectively. Forty-five patients (10.4%) with GS at diagnosis were younger (P < 0.001), presented with higher white blood cell counts (P = 0.03) and were more likely to conform to French-American-British M4 (P = 0.001) and M5 (P = 0.045) classifications than those without GS. In contrast, no significant difference in frequency of cytogenetic abnormalities was found between the GS and non-GS groups. Treatment outcomes in 260 patients (40 with GS) who underwent intensive chemotherapy, excluding patients with acute promyelocytic leukemia, were investigated. Complete remission rates did not differ significantly between the GS and non-GS groups (75.0% vs 79.1%; P = 0.192, respectively) or the 5-year overall survival (OS) rates (39.9% vs 38.7%; P = 0.749, respectively). However, the GS group had a significantly higher relapse rate than the non-GS group (74.2% vs 55.3%; P = 0.048) and a significantly lower 5-year disease-free survival rate (8.2% vs 25.7%, respectively; P = 0.005). When considered together with the results of multivariate analysis, which identified the presence of GS as an independent predictor for disease-free survival time, these findings indicate that GS might identify a high-risk subset of patients with AML. (Cancer Sci 2012; 103: 1513-1517 G ranulocytic sarcoma (GS), a rare extramedullary tumor composed of malignant granulocytic precursor cells that affects 3-9% of patients diagnosed with acute myeloid leukemia (AML), (1)(2)(3) occurs concomitantly with or after a diagnosis of AML. Granulocytic sarcoma can occur in any organ and common sites are bones, lymph nodes, soft tissues and skin. (4) Other organs, including the brain, (5,6) orbit (7,8) and genitourinary system, (9,10) are infrequently affected by GS. Such variation in its site of occurrence complicates accurate diagnosis of GS, and, moreover, the low incidence of GS has impeded establishment of the clinical characteristics of patients with GS. Several studies have reported that specific factors, including French-American-British (FAB) M4 and M5 classification (11,12) and the expression of CD56 (13,14) or T-cell antigens (CD2, CD4 and CD7) (15,16) on leukemia cells, are associated with GS, while others have identified an association between GS and t(8;21) (17,18) or inv(16). (19,20) Establishment of the clinical characteristics of GS has been further impeded by the mixed results obtained by several studies that investigated the prognosis of GS in limited subpopulations of AML patients. Byrd et al. (21) found that GS was associated with unfavorable outcomes and a shorter survival time (median 5.4 months with GS vs 59.5 months without GS) among AML patients carrying t(8;21), whereas Felice et al. (22) argued that GS had no adverse prognostic impact on AML patients carrying t(8;21). In light of these discrepant findings, this study aimed to clarify the clinical significance of GS...

Post-transplant lymphoproliferative disorder (PTLD) is a fatal complication of allogeneic hematopoietic stem cell transplantation (HSCT) that is caused by reactivation of Epstein-Barr virus (EBV). A successful approach, monitoring EBV-DNA load in peripheral blood (PB) accompanied by preemptive rituximab therapy, has recently been reported. Here, we describe a 29-year-old woman who developed isolated central nervous system (CNS) PTLD. She received HSCT against acute myelogenous leukemia from a related human leukocyte antigen-haploidentical donor, following a conditioning regimen that included antithymocyte globulin. Tacrolimus and methylprednisolone were given as prophylaxis for graft-versus-host disease. On day +172, the patient's consciousness deteriorated. Magnetic resonance imaging showed six ring-enhanced lesions in the cerebral hemispheres. These tumors were diagnosed, via a craniotomy and tumorectomy, as PTLD. EBV-DNA load was elevated in the cerebrospinal fluid (CSF) but not detected in PB. She was treated with whole-brain irradiation and rituximab, and achieved partial remission of the tumors. This case serves as a reminder that vigilance is required regarding the development of isolated CNS PTLD; it is worth examining EBV-DNA replication in CSF for diagnosis even when the EBV-DNA load is negative in PB.

We report a case of haemophagocytic syndrome (HPS) occurring after allogeneic bone marrow transplantation (BMT) for acute promyelocytic leukaemia (APL) in a patient in fourth complete remission (CR). Anti-cytomegalovirus (CMV) antibody (Ab) was negative in this patient before BMT. BMT was performed from an HLA-identical unrelated donor who was positive for CMV Ab. After bone marrow engraftment and haematological recovery, severe acute graft-versus-host disease (GVHD) developed. This patient was treated with methylprednisolone in addition to cyclosporin A (CsA). Acute GVHD showed partial improvement, but CMV antigenaemia was observed. Despite administration of gancyclovir and immunoglobulin, CMV antigenaemia showed no improvement and HPS developed. As no other infections or malignancies were observed, we suspect that CMV infection was the trigger for development of HPS.