S. Mayer, N. Srinivasan, J. Nguyen, R. Spilman, D. Scherbak
{"title":"A Curious Case of Hemolytic Anemia with Pseudoreticulopenia","authors":"S. Mayer, N. Srinivasan, J. Nguyen, R. Spilman, D. Scherbak","doi":"10.1155/2022/6423143","DOIUrl":"https://doi.org/10.1155/2022/6423143","url":null,"abstract":"Herein, we present a unique case of a Coombs-negative, steroid-refractory autoimmune hemolytic anemia (AIHA) complicated by pseudoreticulopenia, describe its clinical presentation, histopathologic findings, and management, and review the salient literature. Coombs-negative, steroid-refractory AIHAs represent fewer than 1% of all AIHAs. Diagnosis of the disease is difficult and often delayed due to the pursuit of alternate diagnoses following a negative Coombs test. However, when suspicion remains high for an autoimmune process, the super-Coombs test may be utilized for the diagnosis of AIHA that the traditional Coombs test fails to detect. A majority of cases respond to rituximab as the indicated second-line therapy, but delays in diagnosis and subsequent treatment may increase morbidity. Reticulopenia may be associated with AIHAs secondary to bone marrow dysfunction, but this patient had a normal function marrow confirmed on biopsy. Indeed, reticulopenia in this case was a diagnostic conundrum that further obscured the diagnosis and delayed treatment. Ultimately, reticulopenia was determined to be pseudoreticulopenia secondary to an alteration in the maturation of the erythroid lineage due to an independent, newly diagnosed pernicious anemia. The interaction of these multiple coexisting disease processes is not previously described in the literature. Increased physician awareness of steroid-refractory, Coombs-negative AIHA, and the development of pseudoreticulopenia as a laboratory finding in pernicious anemia may help to improve patient outcomes.","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"27 1","pages":""},"PeriodicalIF":0.7,"publicationDate":"2022-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85391019","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Maryam Mehdipour Dalivand, Parastou Tizro, Julie Partain, A. Aggarwal, J. Lichy, Francisco Mot Ara, V. Nava
{"title":"Concurrent B Cell Acute Lymphoblastic Lymphoma/Leukemia and Monoclonal B Cell Lymphocytosis: A Case Report with Extensive Molecular Analysis","authors":"Maryam Mehdipour Dalivand, Parastou Tizro, Julie Partain, A. Aggarwal, J. Lichy, Francisco Mot Ara, V. Nava","doi":"10.1155/2022/1132544","DOIUrl":"https://doi.org/10.1155/2022/1132544","url":null,"abstract":"Although acute lymphoblastic leukemia (ALL) and monoclonal B cell lymphocytosis (MBL) are common neoplasia, a simultaneous presentation is very unusual. Here, we present two different B cell clones, MBL and B-ALL, cocirculating in a 78-year-old African American male. Detailed molecular characterization revealed an unusual MPL (T487I) point mutation and unmutated VH4-39. After nonstandard chemotherapy, the patient remains in morphologic remission. These findings may stimulate further research to clarify the pathogenesis of hematologic neoplasms.","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"22 1","pages":""},"PeriodicalIF":0.7,"publicationDate":"2022-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83352691","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alexis Lordi, N. Ansari, M. Maroules, Anusha Manjegowda
{"title":"Splenomegaly in a Patient with a History of Pernicious Anemia; the Potential Therapeutic Effects of B12 Therapy","authors":"Alexis Lordi, N. Ansari, M. Maroules, Anusha Manjegowda","doi":"10.1155/2022/2854520","DOIUrl":"https://doi.org/10.1155/2022/2854520","url":null,"abstract":"Splenomegaly is manifested by a variety of etiologies, one of which is macrocytic anemia. Macrocytic anemia has multiple causes in itself that include; folate (Vitamin B9) and Cobalamin (vitamin B12) deficiencies. In this case report, we present a patient with a history of pancytopenia, macrocytic anemia and vitamin B12 deficiency, who underwent a splenectomy. The differential diagnoses for the cause of the patient's splenomegaly included: lymphoma, infiltrative disease, and idiopathic splenomegaly. The pathology report from the splenectomy did not reveal any evidence of lymphoma or infiltrative disease, however, it did mention vascular congestion of the spleen. In theory, vascular congestion, due to extramedullary hematopoiesis in the spleen or sequestration of blood cell lineages, could lead to pancytopenia. In prior visits to the hospital this patient was diagnosed with: splenomegaly, and macrocytic anemia due to pernicious anemia. A splenectomy puts one at increased risk for infection by encapsulated organisms, and is to be avoided if possible. There are few case reports and studies that show vitamin B12 therapy can potentially cause a reversal in the splenomegaly as well as a reversal in the pancytopenia and macrocytic anemia. We hope to show that the least invasive treatment for vitamin B12, vitamin therapy, can be of use and effective.","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"32 1","pages":""},"PeriodicalIF":0.7,"publicationDate":"2022-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80061901","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Stamatios Chrysochoou, A. Kreft, Eberhard Schneider
{"title":"Siltuximab-Related Favorable Clinical Outcome for a Patient Suffering from Idiopathic Multicentric Castleman Disease","authors":"Stamatios Chrysochoou, A. Kreft, Eberhard Schneider","doi":"10.1155/2022/1840589","DOIUrl":"https://doi.org/10.1155/2022/1840589","url":null,"abstract":"RationalCastleman disease is a rare lymphoproliferative disorder that can be subdivided into unicentric and multicentric forms, the latter of which causes a spectrum of serious medical conditions. Here, we present a case of idiopathic multicentric Castleman disease in the eighth decade of life. Patient Concerns. First hospitalized due to unexplained progressive anemia, the patient was readmitted to the hospital 18 months later with suspected lymphoma. Clinical examination revealed a progressive lymphadenopathy. Diagnoses. Histopathologic lymph node features, anemia, elevated CRP and IL6 levels, splenomegaly, and hypoalbuminemia indicated multicentric Castleman (MCD) disease. Interventions. The patient was treated intravenously with a dose of 11 mg/kg siltuximab every 3 weeks. Outcomes. Timely correct diagnosis through the stringent use of consensus diagnostic criteria and sufficient siltuximab therapy has considerably promoted favorable clinical outcomes in a patient suffering from MCD.","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"47 3","pages":""},"PeriodicalIF":0.7,"publicationDate":"2022-03-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72475451","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Osama N. Dukmak, Hamzeh M. I. Abugharbieh, Mohammad Emar, Iman Khamayseh, Salem M. Tos, Rafiq Salhab
{"title":"Double-Expressor Appendiceal Burkitt's Lymphoma: A Case Report and Literature Review","authors":"Osama N. Dukmak, Hamzeh M. I. Abugharbieh, Mohammad Emar, Iman Khamayseh, Salem M. Tos, Rafiq Salhab","doi":"10.1155/2022/6795699","DOIUrl":"https://doi.org/10.1155/2022/6795699","url":null,"abstract":"Background Appendiceal lymphoma is a very rare entity accounting for 0.015% of all gastrointestinal lymphoma cases. Acute appendicitis is the most common presentation of primary appendix neoplasms. Burkitt's lymphoma presenting as an acute appendicitis is a rare entity with around 21% of the cases presenting as a lower iliac fossa mass. Case Presentation. A 23-year-old male was admitted to the surgical ward as a case of acute appendicitis with localized tenderness in the right iliac fossa, positive rebound tenderness, a positive Rovsing's sign, and ultrasound findings of suspected complicated appendicitis. Appendectomy was performed. Histopathological examination of the appendectomy specimen revealed a double-expressor non-Hodgkin diffuse large cell lymphoma with Burkitt's-like morphology. He was sent for chemotherapy treatment. Conclusion Only 34 cases of Burkitt's lymphoma have been reported to present as acute appendicitis. Histological examination following appendectomy for an apparent appendicitis is essential. Furthermore, complete blood count and a computed tomography scan aid the diagnosis of lymphoma. Double-expressor lymphoma has been shown to have poor outcomes. Therefore, prompt and aggressive treatment is vital.","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"40 1","pages":""},"PeriodicalIF":0.7,"publicationDate":"2022-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80411975","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jorge D. Guerra, Jasmine Gowarty, Jordan Buess, James Mason, K. Halka
{"title":"A Case of Acquired Hemophilia A in a Patient with Exposure to COVID-19","authors":"Jorge D. Guerra, Jasmine Gowarty, Jordan Buess, James Mason, K. Halka","doi":"10.1155/2022/9494249","DOIUrl":"https://doi.org/10.1155/2022/9494249","url":null,"abstract":"Acquired Hemophilia A (AHA) is a very rare autoimmune condition involving immune-mediated depletion of Factor VIII, resulting in spontaneous hemorrhage. Failure to recognize AHA as a possible etiology of hemorrhage can result in delayed diagnosis and treatment. The COVID-19 pandemic has given rise to several hematologic conditions and complications, with a rare manifestation being Acquired Hemophilia A (AHA). An interesting case of AHA following SARS-CoV-2 infection is described, along with the treatment approach and a brief review of several cases describing AHA in association with COVID-19.","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"27 1","pages":""},"PeriodicalIF":0.7,"publicationDate":"2022-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88196343","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Justin Komisarof, J. Lipof, Joseph DiTursi, A. Chowdhry, Hae Yoon Grace Choung, W. Burack, L. Constine, F. Passero
{"title":"An Unusual Case of Multiple Myeloma with Light-Chain Cast Nephropathy Secondary to a Very Large Plasmacytoma without Bone Marrow Involvement","authors":"Justin Komisarof, J. Lipof, Joseph DiTursi, A. Chowdhry, Hae Yoon Grace Choung, W. Burack, L. Constine, F. Passero","doi":"10.1155/2022/7531142","DOIUrl":"https://doi.org/10.1155/2022/7531142","url":null,"abstract":"Here, we report a case of a patient who presented to Strong Memorial Hospital with new-onset renal failure and anemia and was found to have multiple myeloma with lambda light-chain cast nephropathy secondary to a very large (14 cm × 14 cm × 12 cm) plasmacytoma without bone marrow involvement. This case is notable as solitary plasmacytomas are almost never seen with concomitant myeloma-defining CRAB criteria or significantly elevated serum free light-chain ratios. Although solitary plasmacytomas are typically definitively treated with radiation, this case highlights that systemic treatment may be helpful in certain clinical scenarios.","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2 1","pages":""},"PeriodicalIF":0.7,"publicationDate":"2022-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83001066","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jan Braune, Kathrin Rieger, Olga Blau, Ulrich Keller, Lars Bullinger, Jan Krönke
{"title":"Relapse of <i>NPM1</i>-Mutated AML with Extramedullary Manifestation 17 Years after Allogeneic Hematopoietic Stem Cell Transplantation.","authors":"Jan Braune, Kathrin Rieger, Olga Blau, Ulrich Keller, Lars Bullinger, Jan Krönke","doi":"10.1155/2022/3317936","DOIUrl":"https://doi.org/10.1155/2022/3317936","url":null,"abstract":"<p><p>The majority of patients with acute myeloid leukemia (AML) with the <i>NPM1</i> mutation achieve remission with intensive chemotherapy. However, many patients subsequently relapse, which occurs frequently within the first 2-3 years after therapy, while late relapse after more than 10 years is rare and can also represent secondary/therapy-associated AML without the NPM1 mutation. Here, we present a case of <i>NPM1</i>-mutated AML that developed medullary and extramedullary relapse 17 years after allogeneic stem cell transplantation, maintaining the <i>NPM1</i> mutation and all other genetic alterations detected at first diagnosis. This exceptionally long latency between diagnosis and relapse of a genetically highly related leukemic clone implies the existence of therapy-resistant, persisting dormant leukemic stem cells in <i>NPM1</i> mutant AML.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2022 ","pages":"3317936"},"PeriodicalIF":0.7,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9812588/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10508985","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}
G A C Gamakaranage, C Kulathilake, P G N Nadeeshani, K H B P Fernandopulle, C S Moonesinghe, D Gunawardena, R S Gamage, N Perera, J Indrakumar
{"title":"A Rapid and Satisfactory Outcome with Combined Immunosuppressive Therapy in Acquired Haemophilia A with Underlying Tuberculosis.","authors":"G A C Gamakaranage, C Kulathilake, P G N Nadeeshani, K H B P Fernandopulle, C S Moonesinghe, D Gunawardena, R S Gamage, N Perera, J Indrakumar","doi":"10.1155/2022/2271228","DOIUrl":"https://doi.org/10.1155/2022/2271228","url":null,"abstract":"<p><p>Acquired haemophilia A (AHA) is a rare disorder with an incidence of 1.5 cases per million per year in the United Kingdom. The incidence could be underestimated due to difficulty in diagnosis and also due to the fact that people with low titre inhibitor levels are asymptomatic. It is usually a disease affecting elderly but a disease peak in the younger population is known. The common underlying diseases are autoimmune disorders, malignancies, infections, and drugs. However, approximately 50% of the cases do not have a specific aetiology and about 10% will not have bleeding manifestations. Therefore, an isolated prolongation of APTT should be evaluated, especially prior to any haemostatic challenges. We report a case of a middle-aged man who presented with bleeding due to AHA associated with high inhibitory titres and active pulmonary tuberculosis. He was treated with both antituberculous and combined-aggressive immunosuppressive therapy which resulted in satisfactory disease remission.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2022 ","pages":"2271228"},"PeriodicalIF":0.7,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9726257/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10365048","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}
Carolina Afonso, Adriana Roque, Cátia Almeida, Maria Beatriz Pimentão, Maria José Julião, Rodolfo Silva, Catarina Geraldes, Marília Gomes
{"title":"Methotrexate-Associated Lymphoproliferative Disorder in a Patient with Psoriasis: A Case Report and Review of the Literature.","authors":"Carolina Afonso, Adriana Roque, Cátia Almeida, Maria Beatriz Pimentão, Maria José Julião, Rodolfo Silva, Catarina Geraldes, Marília Gomes","doi":"10.1155/2022/7178065","DOIUrl":"https://doi.org/10.1155/2022/7178065","url":null,"abstract":"<p><p>Iatrogenic immunodeficiency-associated lymphoproliferative disorders (LPDs) are heterogeneous clinicopathological entities developing in patients receiving immunosuppression. Outside the posttransplant setting, methotrexate (MTX), a drug commonly used for the treatment of autoimmune diseases, is an immunosuppressive agent frequently reported to be associated with LPD. MTX-associated LPD (MTX-LPD) includes a spectrum of lymphocytic proliferations, ranging from polyclonal hyperplasia to malignant lymphoma. MTX-LPD diagnosis can be challenging, as signs and symptoms are often nonspecific and may overlap with those of several other conditions, including exacerbation of the underlying autoimmune disease. Spontaneous regression of LPD after MTX discontinuation is characteristic of MTX-LPD, therefore avoiding chemotherapeutic intervention in a significant proportion of patients. Other cases, however, should receive chemotherapy.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":"2022 ","pages":"7178065"},"PeriodicalIF":0.7,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9078817/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10252334","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}