Vitamin B6 (VB6) deficiency contributes to oncogenesis and tumor progression in certain cancers, and is prevalent in cancer patients in general. VB6 is also an essential element of heme synthesis, and deficiency can lead to anemia. Primary myelofibrosis (PMF) and secondary myelofibrosis (sMF) are myeloproliferative neoplasms often presenting with anemia along with other cytopenias. We performed a prospective study to determine whether PMF and sMF patients suffer from VB6 deficiency, and whether VB6-deficient patients show improvement of anemias with VB6 supplementation. Twelve PMF patients and 11 sMF patients were analyzed. A total of 16 of 23 patients (69.6%) were found to have VB6 deficiency, but VB6 supplementation with pyridoxal phosphate hydrate did not elevate hemoglobin levels in deficient patients. None of the patients presented with vitamin B12, iron, or copper deficiencies. Four patients showed serum folate levels below the lower limit of normal and eight patients showed serum zinc levels below the lower limit of normal; however, these deficiencies were marginal and unlikely to contribute to anemia. Compared to VB6-sufficient patients, VB6-deficient patients showed significantly lower serum folate levels and higher serum copper levels. Studies elucidating the relationship of VB6 deficiency and etiology of PMF/sMF are warranted.

AimsIndolent T-lymphoblastic proliferation (iT-LBP) is a non-clonal benign condition showing extrathymic proliferation of T-lymphoblasts positive for CD3, CD4, CD8, and TdT. Isolated iT-LBP has been observed, but the majority of iT-LBPs have been seen in conjunction with other disorders, including Castleman disease, hepatocellular carcinoma, follicular dendritic cell tumours, angioimmunoblastic T-cell lymphoma, myasthenia gravis, and acinic cell carcinoma (ACC). The clinical course of iT-LBP is indolent, and no therapy is usually required. A major concern is misdiagnosis as T-lymphoblastic lymphoma, and a correct diagnosis of iT-LBP often requires not only pathological analysis but also careful monitoring of the clinical course. The aim of this study was to broaden the knowledge of pathologists and physicians concerning this as yet not well-recognised entity. Methods and resultsWe report a case of iT-LBP concomitant with ACC, along with a literature review of all 14 cases of iT-LBP reported to date. ConclusionsiT-LBP should always be considered as a differential diagnosis of T-lymphoblastic lymphoma, as the two disorders show extremely similar traits.

Background: Diffuse large B-cell lymphomas (DLBCLs) with MYC -rearrangement (MYC -R) or double protein expression (DE) of the MYC and BCL2 are associated with poor prognosis. MYC -R reportedly occurs in approximately 5-14% of DLBCL cases. While the pathological and genetic prognostic factors for DLBCL with MYC -R have been reported, the clinical prognostic factors, including the extranodal sites involved, have not been fully elucidated. In the updated National Comprehensive Cancer Network guidelines for non-Hodgkin lymphomas, the presence of pleural effusion is a poor prognostic criterion for low-grade B-cell lymphomas; however, its prognostic significance in high-grade B-cell lymphomas is unclear. Therefore, we aimed to investigate the association between the presence of pleural effusion at diagnosis and outcomes in DLBCL with MYC -R or DE.

Humoral hypercalcemia due to parathyroid hormone-related peptide (PTHrP) elevation is a well-known complication of various malignancies, but the situation is rare concerning hematological malignancies except for adult T-cell leukemia/lymphoma. We report a case of Richter's syndrome with humoral hypercalcemia, and demonstrate by reverse transcription polymerase chain reaction (RT-PCR) that peripheral blood PTHrP levels were 2,500-fold higher compared to healthy controls. PTHrP production by tumor cells in chronic lymphocytic leukemia (CLL) and Richter's syndrome has been previously demonstrated by nonquantitative methods such as immunohistochemistry and northern blot analysis, but this is the first report using the RT-PCR method. The presented case did not have hypercalcemia when initially diagnosed as small lymphocytic lymphoma (SLL), and as reported earlier, the development of hypercalcemia may be an indication of the transformation to Richter's syndrome in patients with CLL/SLL.

For younger patients with relapsed or refractory lymphomas who respond to salvage therapy, autologous stem cell trans- plantation(ASCT)is the standard of care. Recently, it was demonstrated that the gemcitabine/dexamethasone/cisplatin (GDP) regimen for patients with relapsed or refractory aggressive non-Hodgkin's lymphoma (NHL) prior to ASCT was not inferior to the standard dexamethasone/cytarabine/cisplatin (DHAP) regimen for patients with relapsed and refractory aggressive lymphoma. In Japan, most patients who receive CDDP-containing regimens are hospitalized because of the substantial transfusions required for preventing renal dysfunction. We initiated GDP therapy combined with a short period of hydration and the administration of a magnesium agent and mannitol for 5 patients with relapsed and refractory aggressive lymphoma. In 4 cases, GDP was safely administered on an outpatient basis. Furthermore, peripheral blood stem cells were successfully collected in 2 patients. After stem cell harvest, ASCT was performed in a patient with diffuse large B-cell lymphoma, with the patient remaining in complete remission (CR) after ASCT.

A 36-year-old woman with a left lung tumor was referred to our hospital since a pathological diagnosis had not been obtained at a previous medical institution. We carried out CT-guided percutaneous lung biopsy with an 18-gauge needle and obtained four samples. Immunological staining revealed the specimens to be CD30- and PAX5-positive, with large dysplastic lymphocytes negative for Bob-1 and Oct-2 with a background of small lymphocytes and eosinophils. Primary pulmonary Hodgkin lymphoma (PPHL) was diagnosed. Although PPHL is very rare, it should be included in the differential diagnosis of lung tumors and immunological staining with CD15 and CD30 is recommended. Furthermore, carefully planned CT-guided lung biopsy is useful for diagnosing PPHL.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal hematopoietic stem cell disorder that presents with hemolytic anemia, thrombosis, and bone marrow failure. Stressors such as infection and pregnancy have been known to exacerbate hemolysis in PNH patients. Surgery can also trigger prominent complement activation and is an important risk factor for hemolysis. Furthermore, the postoperative thrombosis risk is high. Eculizumab, which is a humanized monoclonal antibody against C5, suppresses hemolysis and prevents thrombosis, and thus improves quality of life for PNH patients. However, few reports have focused on eculizumab-treated PNH patients undergoing surgery. We report a 79-year-old PNH patient receiving eculizumab treatment who underwent three consecutive orthopedic surgeries requiring general anesthesia. Perioperative management was carried out routinely, as in non-PNH patients, and no postoperative complications developed. Surgery was formerly considered to be a high risk event for PNH patients, but this case raises the possibility that even elderly PNH patients may undergo surgery safely when maintained on eculizumab treatment.

Geriatrics & Gerontology InternationalVolume 15, Issue 6 p. 812-813 CASE REPORT Reactivation of hepatitis B virus during treatment with hydroxyurea in an elderly patient with essential thrombocythemia Naoki Watanabe, Naoki Watanabe Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorHajime Yasuda, Hajime Yasuda Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorYasuo Aota, Yasuo Aota Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorJun Ando, Jun Ando Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorTomonori Aoyama, Tomonori Aoyama Division of Gastroenterology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorMasaru Tanaka, Masaru Tanaka Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorAkihiko Gotoh, Akihiko Gotoh Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorNorio Komatsu, Norio Komatsu Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this author Naoki Watanabe, Naoki Watanabe Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorHajime Yasuda, Hajime Yasuda Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorYasuo Aota, Yasuo Aota Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorJun Ando, Jun Ando Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorTomonori Aoyama, Tomonori Aoyama Division of Gastroenterology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorMasaru Tanaka, Masaru Tanaka Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorAkihiko Gotoh, Akihiko Gotoh Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorNorio Komatsu, Norio Komatsu Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this author First published: 01 June 2015 https://doi.org/10.1111/ggi.12461Citations: 3Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume15, Issue6June 2015Pages 812-813 RelatedInformation

We report herein on a case of invasive aspergillosis accompanied by a subcutaneous nodular lesion. A 74-years-old male with myelodysplastic syndrome was hospitalized due to high fever and a painful subcutaneous nodule on the left thigh. Chest radiography and CT scans showed multiple nodular lesions of both lungs, and bacterial pneumonia was initially suspected. He was treated with meropenem, but the symptoms did not subside. Three days after admission, we found that β-D-glucan levels were elevated at 52.6 pg/mL. He was treated with liposomal amphotericin B (L-AMB) for invasive fungal pneumonia, and the symptoms regressed thereafter. Excisional biopsy of the nodular lesion showed a cluster of septated and branching hyphae. Serum Aspergillus antigen tests and sputum fungal culture were negative, and the fungal species could not be identified. Thus, we performed in situ hybridization (ISH) and polymerase chain reaction (PCR) with the excised subcutaneous specimens, and as a result Aspergillus fumigatus infection was diagnosed. Invasive aspergillosis with a subcutaneous lesion is a rare case, and we found that treatment with L-AMB was effective. ISH, PCR and measurement of serum trough concentration of AMPH-B are useful in diagnosis and treatment.

Hairy cell leukemia typically presents with pancytopenia and often mimics aplastic anemia. Making an accurate diagnosis is crucial, as treatment with the purine analogues cladribine and pentostatin brings about durable complete remission in the majority of patients. Surface kappa and lambda flow cytometric analyses of peripheral blood or bone marrow are a powerful screening tool, although routine gating of the entire lymphocyte region may fail to show light chain restriction due to a low tumor burden. We herein demonstrate that accurate subgating of the large lymphocyte region is essential and recommend the application of this method in all cases of pancytopenia of unknown etiology.

A 78-year-old man with liver cirrhosis was admitted to our hospital because of persistent diarrhea and abdominal pain. Colonoscopy revealed erosions and submucosal tumors in portions of the sigmoid colon and rectum, which were diagnosed as mantle cell lymphoma with biopsy. After chemotherapy consisting of vincristine and prednisone, he developed Aeromonas hydrophila septicemia, resulting in septic shock and necrotizing fasciitis despite prescribing antimicrobial agents to which this organism was susceptible. Whenever we intend to treat patients with hematological malignancies and an underlying chronic liver disease, we must keep this bacterium in mind.