Kassem Hammoud, Robert Fulmer, Megan Hamner, Wissam El Atrouni
{"title":"Ehrlichiosis-Associated Hemophagocytic Lymphohistiocytosis: A Case Series and Review of the Literature.","authors":"Kassem Hammoud, Robert Fulmer, Megan Hamner, Wissam El Atrouni","doi":"10.1155/2023/5521274","DOIUrl":"https://doi.org/10.1155/2023/5521274","url":null,"abstract":"Background Human monocytic ehrlichiosis (HME) is a potentially life-threatening tick-borne illness. HME-associated hemophagocytic lymphohistiocytosis (HLH) is a rare entity with a paucity of published literature regarding treatment and outcome. We present the clinical features, treatment, and outcomes of 4 patients at our institutions with HME-associated HLH. This review also summarizes the current literature regarding the presentation, treatment, and outcome of this infection-related HLH. Methods We searched the PubMed database for case reports and case series. All cases were diagnosed according to the HLH-04 criteria. Results Four cases of HME-associated HLH were included from our institutions. The literature review yielded 30 additional cases. About 41% of the cases were in the pediatric population; 59% were female; and all patients had fever, cytopenia, and elevated ferritin. Most patients were immunocompetent; all but one patient with available data were treated with doxycycline, and eight of the patients with available data received the HLH-94 treatment protocol. The mortality rate was 17.6%. Conclusions HME-associated HLH is a rare but serious syndrome with significant mortality. Early treatment with doxycycline is critical, but the role of immunosuppressive therapy is individualized.","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2023 ","pages":"5521274"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9946753/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9358041","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}
Jorge Avila, Huseyin Berk Degirmenci, Pamela Contreras Chavez, Elisabeth M Battinelli, Jorge Fleisher
{"title":"Rapid Onset Severe Immune Thrombocytopenia following mRNA COVID-19 Vaccine in a Young Patient.","authors":"Jorge Avila, Huseyin Berk Degirmenci, Pamela Contreras Chavez, Elisabeth M Battinelli, Jorge Fleisher","doi":"10.1155/2023/7877536","DOIUrl":"https://doi.org/10.1155/2023/7877536","url":null,"abstract":"<p><p>The coronavirus disease 2019 (COVID-19) pandemic has affected millions of people around the world. Vaccination against COVID-19 has been approved for the following three vaccines in the United States: Pfizer-BioNTech, Moderna, and Janssen. Hematological complications of vaccination have been reported in the literature but remain as a rare phenomenon. We present the case of a patient who developed severe thrombocytopenia within twenty-four hours following the Pfizer-BioNTech vaccination. Commonly encountered differentials including heparin-induced thrombocytopenia and common viral etiologies were ruled out, and other causes such as drug reactions deemed unlikely as the etiology of this presentation after a broad workup. Nucleocapsid antibodies against COVID-19 were found to be positive which indicated that vaccination was at least the second encounter with this virus for our patient, which has been reported previously as the cause of immune thrombocytopenia (ITP), and this might be the culprit for sudden onset. He responded to the first-line ITP treatment with corticosteroids and intravenous immunoglobulin (IVIG) as evidenced by the fast recovery of platelet count and lack of recurrence of thrombocytopenia.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2023 ","pages":"7877536"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10027455/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9167005","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":"Gilteritinib as Bridging and Posttransplant Maintenance for Relapsed Acute Myeloid Leukemia with FLT3-ITD Mutation Accompanied by Extramedullary Disease in Elderly.","authors":"Masuho Saburi, Masanori Sakata, Rika Maruyama, Yosuke Kodama, Hiroyuki Takata, Yasuhiko Miyazaki, Katsuya Kawano, Junpei Wada, Shogo Urabe, Eiichi Ohtsuka","doi":"10.1155/2023/7164742","DOIUrl":"https://doi.org/10.1155/2023/7164742","url":null,"abstract":"<p><p>A 69-year-old woman was diagnosed with acute myeloid leukemia (AML) with an FMS-like tyrosine kinase 3-internal tandem duplication (FLT3-ITD) mutation. Complete remission (CR) was achieved after induction therapy, but AML resulted in a hematological relapse two months after the consolidation chemotherapy. Relapse was accompanied by multiple skin lesions that demonstrated leukemic cell infiltration as well as a drooping right eyelid with extroversion of the eye due to right oculomotor palsy. Gilteritinib was started as salvage therapy, and bone marrow blasts decreased to 0.8% after one month. Two months later, the eye symptoms improved, and the patient underwent cord blood transplantation (CBT). The skin lesions disappeared after the conditioning regimen, and the patient achieved CR status with complete donor chimerism at day 28. Gilteritinib was restarted as posttransplant maintenance therapy on day 53 of CBT. No adverse events other than mild hepatotoxicity were observed, and the patient was alive and in CR status, while continuing gilteritinib at one year and seven months after CBT. Bridging and posttransplant maintenance therapy with gilteritinib may be a promising therapeutic option for relapsed AML with the FLT3-ITD mutation in elderly patients.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2023 ","pages":"7164742"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10468783/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10154695","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}
Juliano Cordova Vargas, Caio Cardozo, Renata Stanzione, Lucas Fiore, Felipe D'Almeida Costa, Rodrigo Fonseca Abreu, Nelson Hamerschlak, Guilherme Perini
{"title":"Incidental Diagnosis of Oligosymptomatic Bilateral Perirenal Erdheim-Chester Disease during Emergency Investigation for COVID-19 Infection.","authors":"Juliano Cordova Vargas, Caio Cardozo, Renata Stanzione, Lucas Fiore, Felipe D'Almeida Costa, Rodrigo Fonseca Abreu, Nelson Hamerschlak, Guilherme Perini","doi":"10.1155/2023/4683188","DOIUrl":"https://doi.org/10.1155/2023/4683188","url":null,"abstract":"<p><p>Erdheim-Chester disease (ECD), a rare form of non-Langerhans histiocytosis, is a multisystem disorder. The case reported here refers to a 49-year-old man presenting at the emergency room with respiratory symptoms. While undergoing diagnostic tests for COVID-19 infection, tomography revealed asymptomatic bilateral perirenal tumors, while renal function remained unaltered. ECD was suggested as an incidental diagnosis and confirmed by core needle biopsy. This report provides a brief description of the clinical, laboratory, and imaging findings in this case of ECD. This diagnosis, albeit rare, should be taken into consideration in the context of incidental findings of abdominal tumors to ensure that treatment, when required, is instituted early.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2023 ","pages":"4683188"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10257540/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9622727","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}
Ram Prakash Thirugnanasambandam, Farish Mohamed Maraikayar, Marie Liu, Khalid Elbashir, John Muthu
{"title":"Fat Embolization Syndrome Secondary to Steroid Treatment in a Case of Sickle Cell Vaso-Occlusive Crisis.","authors":"Ram Prakash Thirugnanasambandam, Farish Mohamed Maraikayar, Marie Liu, Khalid Elbashir, John Muthu","doi":"10.1155/2023/5530870","DOIUrl":"https://doi.org/10.1155/2023/5530870","url":null,"abstract":"<p><p>Fat embolization syndrome (FES) is often seen as a complication of fractures and has been known to cause respiratory failure, rashes of the skin, thrombocytopenia, and neurological damage. Nontraumatic FES is uncommon and occurs due to bone marrow necrosis. Vaso-occlusive crisis in sickle cell patients secondary to steroid therapy is a rare entity and not widely acknowledged. We report a case of FES secondary to steroid therapy administered for a patient with intractable migraine. FES is an uncommon yet serious complication that occurs due to bone marrow necrosis and is usually associated with increased mortality or damaging neurologic sequelae for the surviving patient. Our patient was initially admitted for intractable migraine and worked up to rule out any acute emergency conditions. She was then given steroids for her migraine which did not subside with the initial treatment. Her condition worsened, and she developed respiratory failure along with altered mental status requiring care in the intensive care unit (ICU). Imaging studies showed microhemorrhages throughout the cerebral hemispheres, brainstem, and cerebellum. The imaging of her lungs confirmed severe acute chest syndrome. The patient also had hepatocellular and renal injuries indicative of multiorgan failure. The patient was treated with a red cell exchange transfusion (RBCx) leading to an almost complete recovery in a few days. The patient, however, had residual neurological sequelae with the presence of numb chin syndrome (NCS). This report thus highlights the need to recognize potential multiorgan failure secondary to steroid treatment and the importance of initiating treatment with red cell exchange transfusions to decrease the risk of such complications secondary to steroids.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2023 ","pages":"5530870"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10332925/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9815282","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 Cardiac Lymphoma Presenting with Thrombocytopenia, Right Heart Failure, and Cardiogenic Shock.","authors":"Samantha Kurniawan, Gita Mathur, Yvonne Bogun, Giselle Kidson-Gerber","doi":"10.1155/2023/5501131","DOIUrl":"https://doi.org/10.1155/2023/5501131","url":null,"abstract":"<p><p>Primary cardiac lymphoma (PCL) is a rare, potentially fatal subtype of non-Hodgkin's lymphoma. Thrombocytopenia has also infrequently been reported in association with other primary cardiac tumours and can add substantial morbidity to an already life-threatening diagnosis if present. We report a rare case of a 70-year-old man who presented with thrombocytopenia (91 × 10<sup>9</sup>/L) and progressive right heart failure. Transthoracic echocardiogram revealed a large 8 × 4 cm right atrial mass with severe tricuspid obstruction, confirmed as PCL on subsequent endomyocardial biopsy and immunohistochemistry. He deteriorated into cardiogenic shock precipitated by atrial fibrillation, with worsening thrombocytopenia (18 × 10<sup>9</sup>/L) in the setting of ischaemic hepatitis. The patient stabilised with initiation of high dose steroids prior to tissue diagnosis and platelet counts normalised following chemotherapy. This case demonstrates the importance of considering PCL as a diagnosis and preemptive initiation of high dose steroids to improve outcomes in PCL associated with cardiogenic shock. This case also elucidates a potential pathophysiological association between PCL and thrombocytopenia.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2023 ","pages":"5501131"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9836803/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10534004","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, Stuart Macleod, Úna Bhreathnach, Kamal Fadalla
{"title":"Imatinib Resistance in Chronic Myeloid Leukemia Associated with a D363G <i>BCR::ABL1</i> Kinase Domain Mutation.","authors":"Stephen E Langabeer, Stuart Macleod, Úna Bhreathnach, Kamal Fadalla","doi":"10.1155/2023/6673144","DOIUrl":"https://doi.org/10.1155/2023/6673144","url":null,"abstract":"<p><p>Acquired resistance to tyrosine kinase inhibitors (TKIs) remains a therapeutic challenge in the treatment of chronic myeloid leukemia (CML). The most studied reason for TKI resistance is the acquisition of mutations within the <i>BCR::ABL1</i> tyrosine kinase domain (KDM) and of which the majority of which occur at seven codons within this region. A case of CML is described in which presence of a rare D363G <i>BCR::ABL1</i> KDM resulted in a suboptimal response to frontline imatinib. Switching to dasatinib resulted in achieving a sustained major molecular response that was maintained after a subsequent switch to bosutinib due to the side effects. Reporting of such cases is important for the future management of any CML patients with this rare mutation.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2023 ","pages":"6673144"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10139808/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9761961","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}
Jiankun Tong, Sergei Aksenov, Beth M Siegel, Lihong Wei, William H Rodgers
{"title":"A Rare Case of Blastic Plasmacytoid Dendritic Cell Neoplasm Occurred in Postchemotherapy of Breast Cancer.","authors":"Jiankun Tong, Sergei Aksenov, Beth M Siegel, Lihong Wei, William H Rodgers","doi":"10.1155/2023/7573037","DOIUrl":"https://doi.org/10.1155/2023/7573037","url":null,"abstract":"<p><p>Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematologic malignancy that arises from plasmacytoid dendritic cells. BPDCN typically presents with skin lesions and may involve peripheral blood, bone marrow, lymph nodes, or extranodal sites. It usually arises de novo, and some BPDCN cases are associated with or develop into myeloid neoplasms. Here, we report a case of a 57-year-old female presenting with cervical lymphadenopathy and skin rashes during the COVID-19 pandemic in 2021 following multiple types of postmastectomy therapy for breast cancer. The patient was ultimately diagnosed with BPCDN by lymph node biopsy. To the best of our knowledge, this is the first case report of BPDCN occurring postchemotherapy of breast cancer.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2023 ","pages":"7573037"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10344635/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9828979","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":"Solitary Plasmacytoma of the Breast: A Case of an Uncommon Breast Neoplasm.","authors":"Sean McCormack, Eyad Hamad, Amar Hamad","doi":"10.1155/2023/9622042","DOIUrl":"https://doi.org/10.1155/2023/9622042","url":null,"abstract":"<p><p>Plasmacytoma is a rare cancer that originates from a single plasma cell and is characterized by the abnormal proliferation of monoclonal plasma cells. It is typically localized in a single area of the body, most commonly in the bone or soft tissue. Solitary plasmacytoma can be further classified as either solitary plasmacytoma of bone (SPB) or solitary extramedullary plasmacytoma (SEP or EMP). Diagnosis may be delayed in symptomatically silent plasmacytomas, but early diagnosis and prompt treatment are crucial for the management of this disease. The mean age for patients with plasmacytoma varies depending on the specific type of plasmacytoma, but generally, it is more common in older adults. Soft tissue plasmacytomas are uncommon, and plasmacytomas manifesting within the breast are extremely rare, especially when they are not a manifestation of multiple myeloma (MM). This report presents a case of SEP of the breast in a 79-year-old female patient. This rare disease needs to be studied further in terms of long-term survival and disease progression to MM. By raising awareness and understanding of plasmacytoma, we aim to improve outcomes and quality of life for patients affected by this disease.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2023 ","pages":"9622042"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10257539/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9620240","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":"IgE Plasma Cell Leukemia Harboring t(11;14) and 1q Amplification.","authors":"Wataru Nakahara, Takahito Ogawa, Hitomi Matsunaga, Yuki Iwasa, Momoka Horita, Mako Ikeda, Mizuki Asako, Sadaharu Iio, Yuki Iwama, Kazumasa Oka, Shuji Ueda","doi":"10.1155/2023/4747989","DOIUrl":"https://doi.org/10.1155/2023/4747989","url":null,"abstract":"<p><p>IgE plasma cell neoplasm is the rarest subtype of plasma cell neoplasms and is known for its poor prognosis and high incidence of t(11;14). However, t(11;14) has been classified as a standard-risk rather than high-risk cytogenetic abnormality in multiple myeloma. We have been unable to explain the discrepancy that the hallmark of IgE plasma cell neoplasm with a poor prognosis is a standard-risk cytogenetic abnormality. Here, we report a case of IgE primary plasma cell leukemia with extramedullary lesions of the liver, stomach, and lymph nodes. Plasma cell infiltration was pathologically confirmed in each organ. Cytogenetic analysis of plasma cells revealed t(11;14) and amplification of 1q21. Chemotherapy, with immunomodulatory imide drugs, proteasome inhibitors, and CD38 antibodies, was unsuccessful. In IgE plasma cell neoplasm, coexistence of other cytogenetic abnormalities with t(11;14) may be important. Investigating the presence of cytogenetic abnormalities coexisting with t(11;14) is not only useful for evaluating prognosis but also important for understanding the pathogenesis of the disease. Recently, venetoclax, an oral BCL2 inhibitor, has demonstrated promising efficacy in plasma cell neoplasm patients harboring t(11;14). Development of an effective venetoclax-based regimen for treating aggressive IgE plasma cell neoplasm with t(11;14) is expected.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2023 ","pages":"4747989"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10319461/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10162120","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}