Journal of Clinical and Experimental Hematopathology
Online ISSN : 1880-9952
Print ISSN : 1346-4280
ISSN-L : 1346-4280
Current issue
Showing 1-9 articles out of 9 articles from the selected issue
Review Article
  • Keisuke Kataoka, Junji Koya
    2020 Volume 60 Issue 3 Pages 66-72
    Published: 2020
    Released: September 25, 2020
    [Advance publication] Released: August 08, 2020
    JOURNALS OPEN ACCESS

    Adult T-cell leukemia/lymphoma (ATL) is an aggressive peripheral T-cell malignancy with a markedly poor prognosis. The low prevalence of ATL among human T-cell leukemia virus type-1 (HTLV-1) carriers and the long latency period before ATL onset suggest that additional genetic lesions are required for ATL leukemogenesis. Recently, a large-scale genetic analysis clarified the entire picture of genetic alterations, identified a number of novel driver genes, and delineated their characteristics. Frequent alterations are observed in the molecules belonging to T-cell receptor/NF-κB signaling and other T-cell-related pathways. A notable feature of the ATL genome is the predominance of gain-of-function alterations, including activating mutations in PLCG1, PRKCB, and CARD11. As many as one-fourth of all ATL cases harbor structural variations disrupting the 3′-untranslated region of the PD-L1 gene, leading to immune evasion of tumor cells. The frequency and pattern of these somatic alterations differ among clinical subtypes. Aggressive subtypes are associated with an increased burden of genetic alterations, and higher frequencies of TP53 and IRF4 mutations, PD-L1 amplifications, and CDKN2A deletions than indolent subtypes. In contrast, STAT3 mutations are more characteristic of indolent ATL. Furthermore, these subtypes are further classified into molecularly distinct subsets with a different prognosis by genetic alterations. We present an overview of the current understanding of somatic alterations in ATL, with specific focus on their utility in clinical settings. Furthermore, we highlight their genetic features by exploring their similarities and differences among peripheral T-cell lymphomas.

    Download PDF (1972K)
Original Articles
  • Ai Mogi, Hidenori Sasaki, Yuta Nakashima, Shotaro Chinen, Masanao Ishi ...
    2020 Volume 60 Issue 3 Pages 73-77
    Published: 2020
    Released: September 25, 2020
    [Advance publication] Released: August 08, 2020
    JOURNALS OPEN ACCESS

    The safety and feasibility of oral fluoroquinolone monotherapy in patients with low-risk febrile neutropenia (FN) were demonstrated in recent studies. Levofloxacin (LVFX) is a commonly prescribed antibiotic; however, evidence for its efficacy against FN is limited. Therefore, in this study, we retrospectively investigated the efficacy of LVFX against low-risk FN in patients with malignant lymphoma at our institution. Treatment success was defined as recovery from fever and neutropenia without alteration of the initial regimen. We recruited 29 patients between January 2013 and December 2018. The median age of the cohort was 64 (range: 21–87) years; 13 (44.8%) were aged over 65 years. In total, 22 patients had diffuse large B-cell lymphoma (DLBCL). Therapy was successful in 24 (82.8%) patients, whereas 5 had treatment failure requiring a change from LVFX to intravenous broad-spectrum antibacterial agents. No deaths related to FN were observed. Two patients required FN-related chemotherapy dose reduction in subsequent cycles. Although this cohort comprised many elderly patients, our study confirmed the efficacy of LVFX in patients with low-risk FN. This may improve the treatment of low-risk FN and malignant lymphoma.

    Download PDF (1625K)
  • Akio Sakatani, Takuro Igawa, Takeshi Okatani, Megumu Fujihara, Hideki ...
    2020 Volume 60 Issue 3 Pages 78-86
    Published: 2020
    Released: September 25, 2020
    [Advance publication] Released: July 08, 2020
    JOURNALS OPEN ACCESS

    Classic Hodgkin lymphoma (CHL) is a lymphoid neoplasia characterized by the presence of large tumor cells, referred to as Hodgkin and Reed-Sternberg (HRS) cells, originating from B-cells in an inflammatory background. As the clinical significance of B-cell markers has yet to be fully elucidated, this study aimed to clarify the clinicopathological significance of CD79a in 55 patients with CHL. They were immunohistochemically divided into two groups, comprising of 20 CD79a-positive and 35 CD79a-negative patients. There was no significant correlation between CD79a and CD20 expression (rs = 0.125, P = 0.362). CD79a-positive patients were significantly older at onset (P = 0.011). There was no significant correlation between CD79a-positivity and clinical stage (P = 0.203), mediastinal involvement (P = 0.399), extranodal involvement (P = 0.749), or laboratory findings, including serum levels of lactate dehydrogenase (P = 1) and soluble interleukin-2 receptor (P = 0.251). There were significant differences in overall survival (OS) (P = 0.005) and progression-free survival (PFS) (P = 0.007) between CD79a-positive and CD79a-negative patients (5-year OS: 64.6% and 90.5%; 5-year PFS: 44.0% and 76.6%, respectively). Five patients in whom the majority (> 80%) of HRS cells expressed CD79a consisted of 4 males and 1 female aged between 52 and 81 years; 4 of them were in a limited clinical stage. We concluded that CD79a-positive CHL may have unique clinicopathological features.

    Download PDF (2120K)
  • Takahisa Yamashita, Claudia Vollbrecht, Burkhard Hirsch, Karsten Kleo, ...
    2020 Volume 60 Issue 3 Pages 87-96
    Published: 2020
    Released: September 25, 2020
    JOURNALS OPEN ACCESS
    Supplementary material

    MYC is a transcriptional factor that regulates growth and proliferation through cell cycle pathways. MYC alterations, in particular MYC rearrangements, are important in assessing the prognosis of aggressive B-cell lymphoma. In this study, we focused on the impact of nine major cell cycle genes for MYC-driven aggressive mature B-cell lymphoma and analyzed the mutational status using targeted next generation sequencing. Our 40 cases of aggressive mature B-cell lymphomas included 5 Burkitt lymphomas, 17 high-grade B-cell lymphomas and 18 diffuse large B-cell lymphomas with MYC breaks in 100%, 88% and 11%, respectively. Our data allowed a molecular classification into four categories partially independent from the histopathological diagnosis but correlating with the Ki-67 labelling index: (I) harboring TP53 and CDKN2A mutations, being highly proliferative, (II) with MYC rearrangement associated with MYC and/or ID3 mutations, being highly proliferative, (III) with MYC rearrangement combined with additional molecular changes, being highly proliferative, and (IV) with a diverse pattern of molecular alterations, being less proliferative. Taken together, we found that mutations of TP53, CDKN2A, MYC and ID3 are associated with highly proliferative B-cell lymphomas that could profit from novel therapeutic strategies.

    Download PDF (2805K)
Case reports
  • Yasunobu Sekiguchi, Mutsumi Wakabayashi, Hiroko Iizuka, Haruko Takizaw ...
    2020 Volume 60 Issue 3 Pages 97-102
    Published: 2020
    Released: September 25, 2020
    [Advance publication] Released: August 08, 2020
    JOURNALS OPEN ACCESS

    A 47-year-old male with macroglossia presented with dyspnea on effort and chest pain at rest. Cardiac MRI revealed diffuse global subendocardial late gadolinium enhancement below the left ventricular endocardium and a dark blood pool of intracardiac contrast medium. Tongue biopsy revealed amyloid deposition, which was limited in the myocardium. He was diagnosed with primary light chain amyloidosis. His condition was stage I according to the Mayo Clinic staging system. He underwent autologous peripheral blood stem cell transplantation. On Day 10, he developed chest pain and died suddenly on Day 11. Postmortem examination revealed amyloid deposition throughout the heart.

    Download PDF (2940K)
  • Noriaki Kawano, Tetsuo Maeda, Sayaka Kawano, Yuri Naghiro, Akiyoshi Ta ...
    2020 Volume 60 Issue 3 Pages 103-107
    Published: 2020
    Released: September 25, 2020
    JOURNALS OPEN ACCESS

    The relapse of acute lymphoblastic leukemia (ALL) usually involves the bone marrow, with the central nervous system being the most frequent extramedullary site. The relapse of ALL in the female genital organs, particularly the uterus, is markedly rare. We report such a patient who developed relapse in the bone marrow and uterus. The uterine lesion, which presented as abnormal uterine bleeding, consisted of a mass on MRI and proliferation of ALL cells on histology. MRI revealed a heterogeneous high-intensity mass (T2-WI/D-WI) with a diameter of 6.8 cm, a notable decrease in the apparent diffusion coefficient (ADC), and mild enhancement by contrast enhancement study. Histological findings of the uterine cervix demonstrated the infiltration of ALL. The patient achieved remission by allogeneic haplo-identical hematopoietic stem-cell transplantation, but died of complications of the transplantation. This case suggested that attention should be paid to the uterus as a site of extramedullary relapse. In addition, abnormal uterine bleeding, which is a common sign of hormonal imbalance and hormone replacement therapy after chemotherapy, may be an initial sign of extramedullary recurrence. To confirm uterine relapse as an intractable disease, the accumulation of more cases is required.

    Download PDF (2037K)
  • Haruo Ohtani, Yoshihiro Nozaki, Takashi Murakami, Lisheng Lin, Junko S ...
    2020 Volume 60 Issue 3 Pages 108-112
    Published: 2020
    Released: September 25, 2020
    JOURNALS OPEN ACCESS
    Supplementary material

    We report an autopsy case of acute myocarditis, in which the mediastinal lymph nodes exhibited unique findings. A 15-year-old Japanese boy was diagnosed with the secondary onset of acute myocarditis. No viruses were identified. Autopsy confirmed acute lymphocytic myocarditis. Lymphadenopathy was observed, especially in pulmonary hilar/mediastinal areas. Microscopically, interfollicular areas were uniformly filled with medium-sized, round cells that resembled lymphocytes. They were immunohistochemically CD3- CD5- CD19+ CD20- CD79a- Pax-5- CD138+ MUM1+ LMP1- EBNA2- cytoplasmic IgG+ IgA- and IgM-. No monotypia was observed for kappa and lambda light chains, and multiplex polymerase chain reaction analyses of immunoglobulin heavy chain variable region diversity demonstrated oligoclonal peaks, suggesting reactive change. IgG+ or VS38c+ cells frequently co-expressed Ki-67 (up to 80%). We considered these cells abundantly present in lymph nodes to be reactive plasmablasts because they were early plasma cells with proliferative activity.

    Download PDF (3018K)
Letter to the Editor
Conference Case
feedback
Top