Zhi Zhuo Du, Mi Zhou, Jing Ling, Lan Cao, Lingjun Kong, Shengqin Cheng, Peifang Xiao, Shaoyan Hu
{"title":"PD-1 Checkpoint Blockade in Patients for Acute Myeloid Leukemia after HSCT Relapse Resulted in Severe GVHD and sHLH.","authors":"Zhi Zhuo Du, Mi Zhou, Jing Ling, Lan Cao, Lingjun Kong, Shengqin Cheng, Peifang Xiao, Shaoyan Hu","doi":"10.1155/2022/1705905","DOIUrl":"https://doi.org/10.1155/2022/1705905","url":null,"abstract":"<p><p>Treatment with immune checkpoint inhibitors (ICI) such as carrizumab leads to immune-mediated adverse effects including severe acute graft versus host disease (aGVHD) and secondary hemophagocytic syndrome (sHLH). Herein, we present two cases where aGVHD and sHLH developed after ICI administration, which was treated using methylprednisolone (MP). They developed high-grade fever complicated with liver dysfunction and diarrhea 1 day after ICI administration. Treatment with MP does not alleviate symptoms because of steroid resistance. Hyperbilirubinemia, rash with blisters, and watery diarrhea showed severe aGVHD. Hyperferritinemia, hypertriglyceridemia, and cytopenias suggested the diagnosis of HLH and met the criteria for sHLH diagnosis. They were thus administered intravenous high-dose MP, methotrexate (MTX), basiliximab, ruxolitinib, etc, which resolved these symptoms.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2022 ","pages":"1705905"},"PeriodicalIF":0.7,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9800091/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10466442","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}
Timothy S Oh, Madelyn Burkart, Amir Behdad, Hatice Savas, Reem Karmali
{"title":"Ibrutinib Plus R-ICE Induces Remission in Blastoid Variant Mantle Cell Lymphoma with CNS Relapse.","authors":"Timothy S Oh, Madelyn Burkart, Amir Behdad, Hatice Savas, Reem Karmali","doi":"10.1155/2022/1930546","DOIUrl":"https://doi.org/10.1155/2022/1930546","url":null,"abstract":"<p><p>Mantle cell lymphoma (MCL) is an aggressive, difficult to treat subtype of lymphoma, resulting in relapses and poor outcomes. Novel agents such as Bruton tyrosine kinase (BTK) inhibitors have been studied in the treatment of relapsed/refractory (R/R) MCL. BTK inhibitor ibrutinib, in particular, has demonstrated improvement in survival outcomes of R/R MCL. Despite these advancements, many cases of MCL, including the more aggressive blastoid and pleomorphic variants, will undergo disease progression leading to poor survival outcomes. Blastoid variant MCL is associated with an increased risk of central nervous system (CNS) involvement, causing high mortality rates. In this case report, we discuss a patient with a diagnosis of blastoid MCL with CNS relapse who achieved a complete response (CR) after receiving standard rituximab plus ifosfamide-carboplatin-etoposide (R-ICE) salvage chemotherapy with the addition of ibrutinib. The patient subsequently underwent autologous stem cell transplantation (autoSCT) and maintained CR with ibrutinib maintenance.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2022 ","pages":"1930546"},"PeriodicalIF":0.7,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9106524/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9259440","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":"Bone Marrow Infiltration in Rosai-Dorfman Disease.","authors":"Fatima Zahra Rahali, Fatima Taher, Houda Nassih, Sanae Sayagh","doi":"10.1155/2022/3420311","DOIUrl":"https://doi.org/10.1155/2022/3420311","url":null,"abstract":"<p><p>Rosai-Dorfman disease (RDD) is a rare and benign nonLangerhans cell histiocytosis. RDD commonly affects children and young adults typically presenting with massive, painless, and bilateral cervical lymphadenopathy. Extranodal involvement is present in about 40% of cases, and bone marrow infiltration is rare and unusual. RDD prognosis is usually good, however, involvement of bone marrow is associated with poor prognosis. We report a case of RDD with bone marrow involvement occurring in a 5-year-old female. She was admitted for asthenia, gingival bleeding, and diffuse bone pain without fever. Physical examination showed pallor, petechiae over the abdomen, painless and bilateral cervical lymphadenopathy, and several bony nodules, without splenomegaly or hepatomegaly. Cell blood count revealed microcytic hypochromic anemia with thrombocytopenia. Bone marrow aspiration showed infiltration by large histiocytes with hypochromatic nuclei and abundant vacuolated cytoplasm without emperipolesis. Biopsies of bone marrow, lymph node, and bone revealed immunohistochemical features of RDD: the histiocytes were positive for CD68 and S100 protein, but negative for CD1a. The patient initially received symptomatic treatment. One week after admission, she died of septic shock before the final establishment of RDD diagnosis. This case report highlights that bone marrow involvement in RDD is rare and associated with poor prognosis. We also aim to emphasize the obligation of bone marrow exploration in patients with RDD and presenting cytopenias in order to make an early diagnosis of bone marrow infiltration.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2022 ","pages":"3420311"},"PeriodicalIF":0.7,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9779991/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10495589","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":"\"A Dangerous Black Box:\" Idiopathic Hemophagocytic Lymphohistiocytosis in Adult Patients-A Case Report and Review of the Literature.","authors":"Nada Agbariah, Javier Sanz, Alicia Rovó","doi":"10.1155/2022/5867129","DOIUrl":"https://doi.org/10.1155/2022/5867129","url":null,"abstract":"<p><p>Hemophagocytic lymphohistiocytosis (HLH) is a rare potentially life-threatening condition characterized by aberrant inflammation that can be related to genetic or sporadic forms. In both forms, triggering factors may be involved. Early detection of the underlying cause is crucial for therapeutic decision, while early intervention might be associated with better outcomes. The largest descriptions in the literature on HLH refer to pediatric cases. Adolescents and adults may also be affected, but there is scarce evidence regarding their diagnosis and management. We describe here the case of a 68-year-old Swiss woman with HLH, in whom an extensive search for underlying causes was performed, but neither trigger nor pathogenic variant was found. An early intervention first with dexamethasone and later with cyclosporine was performed. The patient showed a favorable response and did not require further hospitalization; however, one year after diagnosis, it was not possible to suspend cyclosporine due to recurrence of laboratory inflammation signs by drug tapering. The occurrence of HLH idiopathic forms represents a challenge; failure to identify the underlying triggering cause generates uncertainty, endless diagnostic investigations, and consequently additional delays in the treatment. This manuscript addresses the difficulties on this issue.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2022 ","pages":"5867129"},"PeriodicalIF":0.7,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9741541/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10704928","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}
Nii Boi-Doku Pepra-Ameyaw, William Kwasi Ghunney, Eugene Baafi Ampofo, Edeghonghon Olayemi
{"title":"Chronic Eosinophilic Leukemia Presenting as Cardiac Failure.","authors":"Nii Boi-Doku Pepra-Ameyaw, William Kwasi Ghunney, Eugene Baafi Ampofo, Edeghonghon Olayemi","doi":"10.1155/2022/7841310","DOIUrl":"https://doi.org/10.1155/2022/7841310","url":null,"abstract":"<p><p>Chronic eosinophilic leukemia (CEL) is a rare chronic myeloproliferative disorder characterized by sustained eosinophilia. Although the incidence of CEL is uncertain, it can be clinically devastating as it has a propensity to affect several important organ systems. This is of particular significance in Sub-Saharan Africa where helminthic infections are a more prevalent cause of eosinophilia. To the best of our knowledge, we present the first reported case of CEL complicated by cardiac disease in a Ghanaian. He presented with a history of orthopnoea and dyspnoea on exertion, and examination revealed a pansystolic murmur over the mitral region and moderate splenomegaly. Good symptomatic control was achieved using hydroxyurea after which haematologic and cytogenetic remission was achieved after 12 weeks on a tyrosine kinase inhibitor. Physicians working in low resource environments should exclude clonality in patients presenting with eosinophilia and end-organ damage.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2022 ","pages":"7841310"},"PeriodicalIF":0.7,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9788895/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10495586","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":"Successful Treatment of Concomitant Pleural Mucosa-Associated Lymphoid Tissue Lymphoma and Monoclonal Gammopathy of Undetermined Significance with Lenalidomide, Rituximab, and Dexamethasone.","authors":"Yoshiki Uemura, Risa Maeda, Hiroyoshi Saegusa","doi":"10.1155/2022/2027027","DOIUrl":"https://doi.org/10.1155/2022/2027027","url":null,"abstract":"<p><p>Concomitant plasma cell and B cell neoplasms in a single patient have been infrequently reported. It is known that the prognosis of these patients is worse than that of patients with single-disease onset. Generally, the chemotherapy specific for each disease is provided sequentially. It has been suggested that the specific chemotherapy for lymphoma could lead to the occurrence of refractory multiple myeloma (MM). We present a case with the concomitant occurrence of mucosa-associated lymphoid tissue (MALT) lymphoma and monoclonal gammopathy of undetermined significance (MGUS). MGUS does not usually require aggressive treatment. However, the potential adverse effects of MGUS on the treatment course of the B cell lymphoma were concerning. Therefore, we explored a new therapeutic approach that is simultaneously effective against both diseases. Combination therapy of lenalidomide (LEN) and rituximab (RIT) gained indication for follicular lymphoma and MALT lymphoma recently. LEN is also a key drug in MM treatment. Both diseases in our patient were effectively treated with the combination of LEN, RIT, and dexamethasone. With this combination therapy, we expect a prognostic improvement in concomitant MM and B cell lymphoma cases.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2022 ","pages":"2027027"},"PeriodicalIF":0.7,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9170508/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10620256","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":"The Diagnosis Is in the Smear: A Case and Review of Spur Cell Anemia in Cirrhosis.","authors":"Gabriella A Raffa, Diana M Byrnes, John J Byrnes","doi":"10.1155/2021/8883335","DOIUrl":"https://doi.org/10.1155/2021/8883335","url":null,"abstract":"<p><p>The etiology of anemia in liver cirrhosis is multifactorial; one less recognized cause is hemolytic anemia due to spur cells, known as spur cell anemia. We present the case of a 57-year-old woman with alcoholic cirrhosis who presented with symptomatic macrocytic anemia with a hemoglobin level of 7.4 g/dL and signs of decompensated liver disease. Notably, she had no signs of overt bleeding. Further workup was consistent with hemolysis, with peripheral smear demonstrating spur cells. The patient was treated with both steroids and IVIG, although she eventually expired. The characteristic morphology of spur cells is due to alteration of the lipid composition of the erythrocyte membrane, changing its shape and leading to splenic sequestration and destruction. Characteristic of this disorder is an increased ratio of cholesterol to phospholipid on the membrane, as well as low levels of apolipoproteins and low- and high-density lipoproteins. The presence of spur cells is an indicator of poor prognosis and high risk of mortality. Currently, the only definitive cure is liver transplantation. There is a paucity of literature on the prevalence of this phenomenon and even less about treatment. This case highlights the importance of recognition of spur cell anemia as a cause of anemia in cirrhosis as well as the importance of the peripheral smear in the diagnostic workup. Early recognition can lead to avoidance of unnecessary procedures. Further research is needed to elucidate the true prevalence of spur cell anemia and examine further treatment options.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2021 ","pages":"8883335"},"PeriodicalIF":0.7,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8019378/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10346158","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}
Stephen E Langabeer, Rehman Faryal, Michael O'Dwyer, Sorcha Ní Loingsigh
{"title":"Patient-Initiated Discontinuation of Tyrosine Kinase Inhibitor for Chronic Myeloid Leukemia.","authors":"Stephen E Langabeer, Rehman Faryal, Michael O'Dwyer, Sorcha Ní Loingsigh","doi":"10.1155/2020/9571691","DOIUrl":"https://doi.org/10.1155/2020/9571691","url":null,"abstract":"<p><p>The introduction of tyrosine kinase inhibitors (TKI) has revolutionised the management of patients with chronic myeloid leukemia (CML) over the last twenty years, but despite significant improvements in survival, patients exhibit long-term side effects that impact on quality of life. A major advance in CML management has been the ability to discontinue TKI therapy achieving a treatment-free remission (TFR), yet this option is only available to eligible patients who present with low-risk disease and who subsequently attain deep and sustained molecular responses. A case is described of a patient with CML who self-initiated stopping of TKI therapy when in a less than optimal molecular remission. Despite this action, the patient continues to experience a TFR with prospective close molecular monitoring performed. It is emphasized that this approach may lead to ineffective treatment discontinuation, molecular relapse, and increased patient anxiety. As TFR for patients with CML moves from clinical trials into routine clinical practice, emphasis is placed on adherence to (evolving) guidelines critical to ensure optimal counselling, selection, monitoring, and continued management of patients whether TFR is successful or not.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2020 ","pages":"9571691"},"PeriodicalIF":0.7,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/9571691","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10482689","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}
Kunhwa Kim, Harpreet Kaur, Matthew Chan, Manju Balasubramanian, Sorab Gupta, Vinicius M Jorge
{"title":"An Unusual Initial Presentation of Diffuse Large B-Cell Lymphoma as Recurrent Syncope.","authors":"Kunhwa Kim, Harpreet Kaur, Matthew Chan, Manju Balasubramanian, Sorab Gupta, Vinicius M Jorge","doi":"10.1155/2019/1082543","DOIUrl":"https://doi.org/10.1155/2019/1082543","url":null,"abstract":"<p><p>We describe a rare presentation of diffuse large B-cell Lymphoma (DLBCL) with recurrent episodes of syncope. During the workup for syncope, the patient was incidentally found to have an extensive mass in the left thorax, which was later diagnosed as stage 2 bulky disease DLBCL. This is the rare case of lymphoma presenting as recurrent syncope without cardiac involvement. The patient did not have any further episodes of syncope after her successful treatment of DLBCL.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2019 ","pages":"1082543"},"PeriodicalIF":0.7,"publicationDate":"2019-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/1082543","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37554267","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}
Per Ole Iversen, Mboka Jacob, Jamila Makame, Mclean Abisay, Mbonea Yonazi, Anna Schuh, Julie Makani
{"title":"A Massive Extradural Hematoma in Sickle Cell Disease and the Importance of Rapid Neuroimaging.","authors":"Per Ole Iversen, Mboka Jacob, Jamila Makame, Mclean Abisay, Mbonea Yonazi, Anna Schuh, Julie Makani","doi":"10.1155/2019/1742472","DOIUrl":"https://doi.org/10.1155/2019/1742472","url":null,"abstract":"<p><p>Sickle cell disease (SCD) is an inherited hemoglobinopathy leading to several serious organ complications and early death. It is mostly found in equatorial countries like Tanzania. Extradural hematoma (EDH) is a rare, but serious complication to SCD and may have debilitating consequences. Hitherto, there is no report of EDH in SCD where neuroimaging has been available before, during, and after such an event. Here, we describe a young female SCD patient who developed EDH that required surgical evacuation. She had made full recovery after three months. Neuroimaging performed two years prior to this event was unremarkable except for multiple small cerebral infarcts. On admission, neuroimaging revealed a subgaleal hematoma, possibly indicating disruption of the skull cortex due to increased hematopoiesis. Three months after evacuation of the hematoma, neuroimaging showed evidence of brain atrophy and the previously reported cerebral infarcts and multifocal bone infarction, but no vasculopathy. Possibly, disruption of the skull cortex with subsequent bleeding caused the EDH. As the differential diagnoses of neurological complications in SCD are many and some complications are reversible, neuroimaging should be performed without delay.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2019 ","pages":"1742472"},"PeriodicalIF":0.7,"publicationDate":"2019-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/1742472","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37535926","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}