{"title":"Treating Acquired Factor VIII Inhibitor and Tumor-Induced Hypoglycemia in a Case of Relapsed Diffuse Large B-Cell Lymphoma.","authors":"Preston Baker, Joseph Norton, Nasheed Hossain","doi":"10.14740/jh1118","DOIUrl":"https://doi.org/10.14740/jh1118","url":null,"abstract":"<p><p>Diffuse large B-cell lymphoma (DLCBL) is a heterogenous disease, with many phenotypic subtypes and occasional paraneoplastic syndromes being present. Herein, we describe a case of a 63-year-old woman, with relapsed/refractory DLBCL (RR-DLBCL) with artifactual hypoglycemia on laboratory testing, likely related to the mechanical effects of a new factor VIII inhibitor. We demonstrate our workup, consideration, treatment, and her clinical course. This patient did not present with a bleeding phenotype despite her aberrant laboratory results, and therefore determining her risk of bleeding to weigh against further diagnostic procedures presented a difficult decision. We utilized rotational thromboelastometry (ROTEM) to assist with clinical decision making regarding her paraneoplastic factor VIII inhibitor and the patient's bleeding risk. This led to a short course of dexamethasone. Her ROTEM improved, and an excisional biopsy was performed without any bleeding. To our knowledge, this is the only reported instance where this technology was utilized in this setting. We believe utilizing ROTEM to determine bleeding risk may be a beneficial tool for clinical practice in such additional rare cases.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 3","pages":"133-137"},"PeriodicalIF":1.2,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/fe/c7/jh-12-133.PMC10332858.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9818442","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ahmed Khattab, Sunita Patruni, Gina Patrus, Yazan Samhouri, Salman Fazal, John Lister
{"title":"Donor Cell Leukemia Following Allogeneic Hematopoietic Stem Cell Transplantation.","authors":"Ahmed Khattab, Sunita Patruni, Gina Patrus, Yazan Samhouri, Salman Fazal, John Lister","doi":"10.14740/jh1124","DOIUrl":"10.14740/jh1124","url":null,"abstract":"<p><p>Approximately 25,000 allogeneic transplants are performed annually worldwide; a figure that has steadily increased over the past three decades. The study of transplant recipient survivorship has become a cogent topic and post-transplant donor cell pathology warrants further study. Donor cell leukemia (DCL) is a rare but serious complication of allogeneic stem cell transplantation (SCT) where the recipient develops a form leukemia originating from the donor cells used for transplantation. Detection of abnormalities predicting donor cell pathology might inform donor selection, and the design of survivorship programs for early detection of these abnormalities might allow therapeutic intervention earlier in the disease course. We present four recipients of allogeneic hematopoietic stem cell transplant (HSCT) from our institution who developed donor cell abnormalities allogeneic SCT, highlighting their clinical characteristics and challenges.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 3","pages":"138-144"},"PeriodicalIF":1.2,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/dd/dc/jh-12-138.PMC10332864.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9870420","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sasmith R Menakuru, Janet Roepke, Salahuddin Siddiqui
{"title":"<i>De-Novo</i> B-Cell Prolymphocytic Leukemia.","authors":"Sasmith R Menakuru, Janet Roepke, Salahuddin Siddiqui","doi":"10.14740/jh1096","DOIUrl":"https://doi.org/10.14740/jh1096","url":null,"abstract":"<p><p>B-cell prolymphocytic leukemia (B-PLL) is a rare B-cell neoplasm that typically presents with splenomegaly, a rising white blood cell count, and may or may not have B symptoms. The diagnosis usually requires a bone marrow biopsy and aspirate with flow cytometry and cytogenetic studies. At least 55% of the lymphocytes in the peripheral blood must be prolymphocytes to be defined as B-PLL. A thorough differential diagnosis would include mantle cell lymphoma, chronic lymphocytic leukemia (CLL) with prolymphocytes, hairy cell leukemia, and splenic marginal zone lymphoma. B-PLL is managed with regimens utilized for CLL, such as ibrutinib and rituximab but is tailored for each individual. The authors report a rare case of B-PLL in a patient with no known history of CLL. The authors discuss this entity in context of the 2017 and 2022 World Health Organization (WHO) classifications, the latter of which no longer recognizes B-PLL as a distinct entity. The authors hope that this article helps practitioners with the diagnosis and treatment of B-PLL. Perhaps with better recognition, and better documentation of histopathologic features of these rare cases going forward, it may prove to be a distinct entity again in future classifications.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 2","pages":"82-86"},"PeriodicalIF":1.2,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a1/55/jh-12-082.PMC10181324.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9468647","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kimberly Boldig, Amy Kiamos, Trevanne Matthews-Hew, Reeba Omman, Walter Quan
{"title":"Acute Promyelocytic Leukemia Treatment Masking Hepatic Tuberculosis: A Management Dilemma.","authors":"Kimberly Boldig, Amy Kiamos, Trevanne Matthews-Hew, Reeba Omman, Walter Quan","doi":"10.14740/jh1109","DOIUrl":"https://doi.org/10.14740/jh1109","url":null,"abstract":"<p><p>Acute promyelocytic leukemia is a form of acute myeloid leukemia (AML) that is characterized by presence of a promyelocytic leukemia-retinoic acid receptor alpha fusion. In most patients, this fusion is detected on conventional karyotype as the t(15;17)(q24.1;q21.2) translocation, but some patients have cryptic translocations with a normal karyotype. Historically, AML is associated with a poor prognosis. Treatment with all-trans retinoic acid and arsenic trioxide assures long-term survival in the majority of patients. This treatment is generally well-tolerated but may cause hepatotoxicity. This is usually identified by transaminitis but resolves after temporary cessation of treatment. Our patient's hepatotoxicity did not resolve following all-trans retinoic acid and arsenic trioxide cessation which posed a diagnostic dilemma. This prompted exploration of other possible causes of hepatotoxicity. An eventual liver biopsy identified acid-fast bacilli, confirming a diagnosis of hepatic tuberculosis. A broad differential diagnosis is imperative when investigating abnormalities in liver function, especially in chemotherapy patients when treatment cessation may cause cancer progression.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 2","pages":"100-104"},"PeriodicalIF":1.2,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/69/5b/jh-12-100.PMC10181323.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9468644","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Primary Diffuse Large B-Cell Lymphoma of the Bone.","authors":"Binoy Yohannan, Adan Rios","doi":"10.14740/jh1087","DOIUrl":"https://doi.org/10.14740/jh1087","url":null,"abstract":"<p><p>Primary lymphoma of the bone (PLB) is a rare lymphoproliferative neoplasm that can present either as solitary or multiple bone lesions. We report four patients with PLB who were successfully treated with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) followed by consolidative radiotherapy. All patients achieved a complete remission and had excellent long-term outcomes. PLB has a favorable response to combined modality treatment with chemoimmunotherapy and radiation. Long-term outcomes of PLB tend to be better than those of non-osseous diffuse large B-cell lymphoma.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 2","pages":"75-81"},"PeriodicalIF":1.2,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/09/5b/jh-12-075.PMC10181325.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9474390","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Treatment and Response Evaluation Challenges in a Pregnant Woman With B-Cell Lymphoblastic Leukemia and Li-Fraumeni Syndrome.","authors":"Bjarte Skoe Erikstein, Aymen Bushra Ahmed, Rakel Brendsdal Forthun, Friedemann Leh, Bjørn Tore Gjertsen, Håkon Reikvam","doi":"10.14740/jh1107","DOIUrl":"https://doi.org/10.14740/jh1107","url":null,"abstract":"<p><p>Li-Fraumeni syndrome (LFS) is a cancer predisposing syndrome caused by pathogenic germline <i>TP53</i> gene mutations with important therapeutic and prognostic implications for many types of cancer. A small proportion of LFS patients develop B-cell lymphoblastic leukemia (B-ALL) in adult years. Standard treatment often proves inadequate, but immunotherapy has provided new treatment options. The current case report presents a pregnant woman with LFS and newly diagnosed B-ALL with hypodiploidy developed after treatment for early-onset breast cancer. We describe the treatment course, treatment-related complications and provide laboratory data crucial for evaluating and modifying treatment for this difficult clinical case. Our findings support the need for close collaboration between clinicians and experts on immunophenotyping. Through our report, we show that immunotherapy is feasible in patients with LFS and B-ALL, despite a poor initial response to induction therapy.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 2","pages":"92-99"},"PeriodicalIF":1.2,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/4a/5f/jh-12-092.PMC10181328.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9468643","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sushanth Sreenivasan, Risha Solanki, Pragnan Kancharla, Cyrus Khan, Yazan Samhouri
{"title":"A Meningioma Mimic and Distinct Subtype of Primary Central Nervous System Lymphoma: Primary Dural Lymphoma.","authors":"Sushanth Sreenivasan, Risha Solanki, Pragnan Kancharla, Cyrus Khan, Yazan Samhouri","doi":"10.14740/jh1113","DOIUrl":"https://doi.org/10.14740/jh1113","url":null,"abstract":"<p><p>Primary central nervous system lymphoma (PCNSL) is an aggressive form of extranodal non-Hodgkin lymphoma that arises in the brain parenchyma, eyes, meninges, or spinal cord in the absence of systemic disease. Primary dural lymphoma (PDL), in contrast, arises from the dura mater of the brain. PDL is usually a low-grade B-cell marginal zone lymphoma (MZL), whereas other types of PCNSL are usually high-grade large B-cell lymphoma. This specific pathological subtype has important therapeutic and prognostic implications, making PDL a distinct subtype of PCNSL. Herein, we report a case of PDL in an African American patient, in her late thirties, who presented to our emergency room with chronic headaches. An emergent magnetic resonance imaging (MRI) of the brain showed a dural-based homogeneously enhancing extra-axial mass along the left hemisphere, which was contained within the anterior and parietal dural mater. A surgical specimen was collected after an emergency debulking procedure. The flow cytometry, done on the surgical specimen obtained, was positive for CD19<sup>+</sup>, CD20<sup>+</sup>, and CD22<sup>+</sup>, but negative for CD5<sup>-</sup> and CD10<sup>-</sup>. These findings were consistent with a clonal B-lymphoproliferative disorder. The surgical pathology specimen immunohistochemistry was positive for CD20<sup>+</sup> and CD45<sup>+</sup>, but negative for Bcl-6Cyclin D1<sup>-</sup> and CD56<sup>-</sup>. The Ki67 was 10-20%. These findings were consistent with extranodal MZL. Given the location and pathology, the patient was diagnosed with PDL. Due to MZL's indolent nature, location outside the blood-brain barrier, and known efficacy to bendamustine-rituximab (BR), we decided to treat our patient with BR. She completed six cycles without major complications, and her post-therapy brain MRI showed complete remission (CR). Our case adds to the sparse literature about PDL and highlights the efficacy of BR systemic chemotherapy on MZLs.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 2","pages":"87-91"},"PeriodicalIF":1.2,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/3c/75/jh-12-087.PMC10181329.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9475367","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Incidence and Risk of Hematological Adverse Events Associated With Immune Checkpoint Inhibitors: A Systematic Literature Review and Meta-Analysis.","authors":"Takuma Ohashi, Kaoru Takase-Minegishi, Ayaka Maeda, Naoki Hamada, Ryusuke Yoshimi, Yohei Kirino, Hiroshi Teranaka, Hiroyoshi Kunimoto, Maki Hagihara, Kenji Matsumoto, Ho Namkoong, Nobuyuki Horita, Hideaki Nakajima","doi":"10.14740/jh1090","DOIUrl":"https://doi.org/10.14740/jh1090","url":null,"abstract":"<p><strong>Background: </strong>Immune checkpoint inhibitors (ICIs) have been a breakthrough in cancer therapy. ICI therapy is generally better tolerated than cytotoxic chemotherapy; however, hematological adverse events (AEs) have not been fully analyzed. Hence, we performed a meta-analysis to evaluate the incidence and risk of ICI-related hematological AEs.</p><p><strong>Methods: </strong>A systematic literature search was performed using PubMed, EMBASE, Cochrane Library, and the Web of Science Core Collection. Phase III randomized controlled trials (RCTs) involving ICI combination regimens were selected. The experimental group received ICIs with systemic treatment, and the control group received only the same systemic treatment. Odds ratios (ORs) for anemia, neutropenia, and thrombocytopenia were calculated using a random-model meta-analysis.</p><p><strong>Results: </strong>We identified 29 RCTs with 20,033 patients. The estimated incidence rates for anemia of all grades and grades III-V were 36.5% (95% confidence interval (CI) 30.23 - 42.75) and 4.1% (95% CI 3.85 - 4.42), respectively. The incidence of neutropenia (all grades 29.7%, grades III-V 5.3%) and thrombocytopenia (all grades 18.0%, grades III-V 1.6%) was also calculated.</p><p><strong>Conclusion: </strong>Treatment with ICIs seemed unlikely to increase the incidence of anemia, neutropenia, and thrombocytopenia in all grades. However, programmed cell death-1 receptor ligand inhibitors significantly increased the risk of grades III-V thrombocytopenia (OR 1.53; 95% CI 1.11 - 2.11). Further research is needed to examine the potential risk factors.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 2","pages":"66-74"},"PeriodicalIF":1.2,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/2a/b3/jh-12-066.PMC10181326.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9475369","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rodrick Babakhanlou, Farhad Ravandi-Kashani, Dimitrios P Kontoyiannis
{"title":"Neutropenic Enterocolitis: An Uncommon, but Fearsome Complication of Leukemia.","authors":"Rodrick Babakhanlou, Farhad Ravandi-Kashani, Dimitrios P Kontoyiannis","doi":"10.14740/jh1105","DOIUrl":"https://doi.org/10.14740/jh1105","url":null,"abstract":"<p><p>Neutropenic enterocolitis (NEC) is a life-threatening condition occurring in severely neutropenic patients, following intensive chemotherapy for leukemia. Its pathogenesis is not entirely understood and believed to be multifactorial, including mucosal injury as a result of cytotoxic drugs, profound neutropenia, impaired host defense and possibly microbiota changes. Establishing an early diagnosis is key. The management of NEC remains undefined due to lack of high-quality clinical data. With a better understanding of the disease, a more conservative approach is preferred over surgical intervention. The involvement of a multi-disciplinary team, consisting of the oncologist, infectious diseases specialists and surgeons is highly recommended. This review aims to delineate insights into the pathophysiology and clinical presentation of NEC and to emphasize the diagnostic and therapeutic approach to this condition.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 2","pages":"59-65"},"PeriodicalIF":1.2,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/1c/30/jh-12-059.PMC10181327.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9468648","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Transformed Plasmablastic Lymphoma Presenting With Marked Lymphocytosis and Spontaneous Tumor Lysis Syndrome.","authors":"Yannis Hadjiyannis, Cecelia Miller, Norris I Hollie, Jayalakshmi Balakrishna, Francesca Cottini","doi":"10.14740/jh1067","DOIUrl":"https://doi.org/10.14740/jh1067","url":null,"abstract":"<p><p>The clinicopathology entity of plasmablastic lymphoma (PBL), despite broad recognition by the World Health Organization (WHO), represents a diagnostic challenge due to its overlapping features and scarce occurrence. Often, PBL arises in immunodeficient, elderly male patients, most notably those who are human immunodeficiency virus (HIV)-positive. More infrequent, cases of transformed PBL (tPBL) evolved from another hematologic disease have been identified. Herein, we describe a case of a 65-year-old male transferred from a neighboring hospital with pronounced lymphocytosis and spontaneous tumor lysis syndrome (sTLS) presumed to be chronic lymphocytic leukemia (CLL). Utilizing a complete clinical, morphologic, immunophenotypic, and molecular evaluation, we arrived at a final diagnosis of tPBL with sTLS, suspected to have evolved from the <i>NF-κB/NOTCH/KLF2</i> (NNK) genetic cluster of splenic marginal zone lymphoma (SMZL) (NNK-SMZL), a potential transformation and presentation, to our knowledge, not previously reported. However, definitive clonality testing was not performed. In this report, we also outline the diagnostic and educational considerations we faced in discerning tPBL from other more common B-cell malignancies which can present similarly, such as CLL, mantle cell lymphoma, or plasmablastic myeloma. We summarize recently reported molecular, prognostic, and therapeutic considerations for the treatment and recognition of PBL, including the successful implementation, in our patient, of bortezomib to an EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) regimen with prophylactic intrathecal methotrexate, who has since achieved complete remission (CR) and entered clinical surveillance. Lastly, this report briefly highlights the challenge we faced in this area of hematologic typification that necessitates additional review and discussion by the WHO: tPBL with potential double-hit cytogenetic versus double-hit lymphoma with a plasmablastic phenotype.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 1","pages":"49-58"},"PeriodicalIF":1.2,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/3e/6e/jh-12-049.PMC9990712.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9689461","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}