{"title":"Systemic Mastocytosis in a Patient with BCR-ABL1-Positive Chronic Myeloid Leukemia in the Remission Phase.","authors":"Christos Fokoloros, Periklis Foukas, Nikolaos Georgakopoulos, Zoi Tsakiraki, Anthi Bouchla, Vasilliki Pappa, Alexandros Katoulis, Michael Makris, Sotirios Papageorgiou","doi":"10.1155/2022/7251658","DOIUrl":"https://doi.org/10.1155/2022/7251658","url":null,"abstract":"<p><p>Systemic mastocytosis (SM) comprises a group of rare disorders resulting from tissue infiltration by pathological mast cells. In a percentage ranging from 5 to 40% in various patient series, SM appears to be associated with an accompanying hematologic neoplasm (SM-AHN). The coexistence of SM with chronic myelogenous leukemia (CML) is extremely rare with only 3 cases in the literature. The natural course of CML has changed dramatically over the past 2 decades with the use of tyrosine kinase inhibitors (TKIs). We report a case of diagnosing SM in a patient in complete molecular remission of CML after stopping TKI treatment.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":" ","pages":"7251658"},"PeriodicalIF":0.7,"publicationDate":"2022-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9649302/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40692821","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}
Blake O Adnani, Kathleen O' Brien, Zaw W Myint, Brandon L Adler
{"title":"Paraneoplastic Pemphigus: A Striking Complication of Undiagnosed Lymphoma.","authors":"Blake O Adnani, Kathleen O' Brien, Zaw W Myint, Brandon L Adler","doi":"10.1155/2022/3641474","DOIUrl":"https://doi.org/10.1155/2022/3641474","url":null,"abstract":"<p><p>A 49-year-old male with no past medical history presented with acute-onset painful mucosal erosions along with flaccid bullae on his trunk, scalp, and intertriginous areas. The patient initially underwent a skin biopsy which demonstrated suprabasilar acantholysis and lichenoid interface dermatitis. This was followed by a computed tomography scan which identified a large abdominal lymph node. Core needle biopsy of this node demonstrated follicular lymphoma. Lastly, indirect immunofluorescence (IIF) in rat bladder was positive (titer 1 : 10,240). This finding confirmed the diagnosis of paraneoplastic pemphigus (PNP) in the setting of follicular lymphoma. The patient's cutaneous disease was treated with a combination of intravenous immunoglobulin and methylprednisolone, along with intravenous rituximab, with a resolution of his cutaneous symptoms. His lymphoma was treated with six cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP), with an interval decrease in his tumor burden. PNP is an autoimmune-mediated mucocutaneous disease associated with underlying neoplasm, most commonly non-Hodgkin lymphoma or chronic lymphocytic leukemia. Affected patients develop variable autoantibodies to antigens on keratinocytes and the basement membrane zone. Severe intractable stomatitis is characteristic, in addition to polymorphous cutaneous eruptions including bullae and erosions. Mortality rates can reach up to 90% due to malignancy, sepsis, or bronchiolitis obliterans, an irreversible and often lethal cause of pulmonary insufficiency. We highlight PNP manifesting in a patient with lymphoma, who responded well to the skin- and malignancy-directed treatments. PNP is an exceedingly rare diagnosis that should be considered in a patient with intractable stomatitis.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":" ","pages":"3641474"},"PeriodicalIF":0.7,"publicationDate":"2022-10-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9626227/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40443777","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 Case of Secondary Hemophagocytic Lymphohistiocytosis Presenting with Severe Dyserythropoeisis in a Patient with Autoimmune Hemolysis.","authors":"Kai J Rogers, Sharathkumar Bhagavathi","doi":"10.1155/2022/8505823","DOIUrl":"https://doi.org/10.1155/2022/8505823","url":null,"abstract":"<p><p>The diagnosis of hemophagocytic lymphohistiocytosis (HLH) requires that several clinical criteria are met, and often relies on the identification of rare hemophagocytic cells in the bone marrow. Given the challenge in making the diagnosis, additional signs of immune dysregulation in the bone marrow would have practical clinical use in cases where overt hemophagocytosis is not seen. We present here a case of secondary HLH in a patient with autoimmune hemolysis ultimately diagnosed as Evans syndrome that initially presented with profound dyserythropoeisis in both the peripheral blood and bone marrow. We also explore an association between dyserythropoeisis and HLH in a series of cases previously seen at our institution.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":" ","pages":"8505823"},"PeriodicalIF":0.7,"publicationDate":"2022-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9605839/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40443805","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":"Long-Term Zidovudine Therapy and Whether It is a Trigger of Vitamin B12 Deficiency: A Case Study of Megaloblastic Anemia at the University of Zambia Teaching Hospital.","authors":"Natasha Mupeta Kaweme, Sahar Mounir Nagib Butress, Hamakwa Muluti Mantina","doi":"10.1155/2022/3827616","DOIUrl":"https://doi.org/10.1155/2022/3827616","url":null,"abstract":"<p><p>Macrocytic anemia is frequently observed in adult HIV-infected patients treated with nucleoside reverse transcriptase inhibitors and with vitamin B12 and folate deficiency. In this case report, we discuss a 52-year-old nonvegetarian male on long-term antiretroviral therapy for 5 years, presenting with severe macrocytic anemia (hemoglobin, 3.7 g/dL; mean corpuscular volume, 119.6 fL) and leukopenia (2.71<i>∗</i>10<sup>9</sup>/L), who was diagnosed with megaloblastic anemia caused by vitamin B12 deficiency following laboratory investigations. Parenteral vitamin B12 replacement therapy was initiated, with an early response observed. Notwithstanding, the treatment response was not sustained as the patient later presented with refractory anemia and persistence of macrocytosis. Discontinuation of zidovudine with concurrent vitamin B12 administration promptly improved the patient's clinical deficiency symptoms. At the end of 3 months, the patient had a complete hematological recovery. The deficiency of vitamin B12 disrupts DNA synthesis inhibiting effective hematopoiesis in all cell lines, particularly erythroid precursors and further promotes reversible bone marrow failure. Long-term ART therapy with zidovudine causes cytotoxicity in myeloid and erythroid precursors and induces bone marrow suppression. Whether long-term zidovudine consumption induced lower levels of vitamin B12 and subsequent megaloblastic anemia requires in-depth research and exploration.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":" ","pages":"3827616"},"PeriodicalIF":0.7,"publicationDate":"2022-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9568349/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33517000","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 Rare Case of BCL2-Positive Multiple Myeloma Complicated with Follicular Lymphoma.","authors":"Yutaka Tsutsumi, Shinich Ito, Mirei Kobayashi, Takanori Teshima","doi":"10.1155/2022/3076968","DOIUrl":"https://doi.org/10.1155/2022/3076968","url":null,"abstract":"<p><p>A 68-year-old woman presented with follicular lymphoma complicated by IgG kappa-positive multiple myeloma. In this case, both follicular lymphoma and plasma cells were positive for BCL2 by immunostaining. T-cell association in the FL and MM was also analyzed in this case. Some CD3-positive T-cells were found around the plasma cells. These cells were mainly CD8-positive T-cells and not CD4-positive T-cells. These results suggest that CD4-positive T-cells were not associated with the proliferation of the plasma cells in this case. Although the FL that developed was initially positive for BCL2 protein, this does not indicate that plasma cells were derived from FL cells because of the eventual complication that occurred in this case. Furthermore, in this case, rituximab and bendamustine were effective for FL. They were not effective for MM, however, demonstrating that additional treatment options are necessary for the simultaneous treatment of BCL2-positive MM with FL.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":" ","pages":"3076968"},"PeriodicalIF":0.7,"publicationDate":"2022-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9550423/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33526640","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}
David Palmer, Lauren Davis, Helena Sivaloganathan, Timothy Chevassut
{"title":"A Single-Centre Experience of Post-COVID-19 Vaccine-Related Immune-Mediated Complications.","authors":"David Palmer, Lauren Davis, Helena Sivaloganathan, Timothy Chevassut","doi":"10.1155/2022/4742639","DOIUrl":"https://doi.org/10.1155/2022/4742639","url":null,"abstract":"<p><p>The significant impact of the COVID-19 pandemic has resulted in a worldwide effort to develop effective vaccines. In the United Kingdom, the COVID-19 vaccine development and roll-out has been overwhelmingly successful in reducing infections and deaths. However, case reports have emerged of a rare syndrome of vaccine-induced immune thrombocytopenia and thrombosis (VITT), as well as cases of immune thrombocytopenia (ITP). This has necessitated a better understanding of these conditions. However, as both VITT and \"vaccine-associated ITP\" are emerging conditions, evidence on the clinical features, epidemiology, and management is still evolving. Subsequently, with the initiation of the COVID-19 vaccine booster program, it has become increasingly important to continue to collect accurate data on post-COVID-19 vaccine complications to aid with their prompt recognition and management. In this case series, we report on the presentations and management of seven cases of post-COVID-19 vaccine-related immune-mediated complications which occurred at our center between the months of March and July 2021.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":" ","pages":"4742639"},"PeriodicalIF":0.7,"publicationDate":"2022-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9546669/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33517182","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}
Jew Win Kuan, Sing Ling Chai, Pathmanathan Rajadurai, Lee Gong Lau, Joseph Uchang, Sharifah Noor Akmal Syed Husain
{"title":"A Case Report of Leptomeningeal Myelomatosis and Rapid Improvement with Regimen Consisting of Daratumumab, Pomalidomide, Vincristine, Procarbazine, and Dexamethasone.","authors":"Jew Win Kuan, Sing Ling Chai, Pathmanathan Rajadurai, Lee Gong Lau, Joseph Uchang, Sharifah Noor Akmal Syed Husain","doi":"10.1155/2022/4081971","DOIUrl":"https://doi.org/10.1155/2022/4081971","url":null,"abstract":"<p><p>Central nervous system (CNS) involvement in multiple myeloma (MM) (MM-CNS) in the form of leptomeningeal myelomatosis or brain parenchyma plasmacytoma is rare, causing challenges in clinical diagnosis and treatment. We would like to report a case of leptomeningeal myelomatosis and illustrated the challeges. A 61-year-old man was diagnosed with MM with left paravertebral plasmacytoma, R-ISS II with high suspicion of double-hit MM, either biallelic aberrancy of TP53 or del(17p) and IGH aberrancy depending on the definition chosen, treated with lenalidomide-bortezomib-dexamethasone and local radiotherapy, later developed systemic relapse and progression to MM-CNS in the form of leptomeningeal myelomatosis. A modified CNS-based treatment not reported before, consisting of daratumumab, pomalidomide, vincristine, procarbazine, and dexamethasone, brought a rapid clinical improvement and warrants a further study. Incorporation of intrathecal thiotepa into the regimen would likely increase the efficacy.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":" ","pages":"4081971"},"PeriodicalIF":0.7,"publicationDate":"2022-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9453015/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33461150","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":"Bortezomib Use for a Critically Ill Patient with Angioimmunoblastic T-Cell Lymphoma.","authors":"Motoharu Shibusawa","doi":"10.1155/2022/6079633","DOIUrl":"https://doi.org/10.1155/2022/6079633","url":null,"abstract":"<p><p>Angioimmunoblastic T-cell lymphoma (AITL) accounts for 18.5% of all peripheral T-cell lymphomas. There is still no gold standard chemotherapy for treating newly diagnosed AITL. This case describes the use of bortezomib in newly diagnosed AITL. A 53-year-old man with no previous illness presented with erythema and swelling in the left neck. A diagnosis of AITL was made based on the results of lymph node biopsies. AITL progression led the patient to a severely deteriorated general condition. Bortezomib was thus administered, which resulted in a reduction in lymphadenopathies, the disappearance of tumor fever, and a decrease in serum lactate dehydrogenase levels. Subsequently, the patient's general condition gradually improved. Despite the patient's poor condition, bortezomib was well tolerated. After bortezomib administration, the patient did not require chemotherapy for approximately 10 months. The present case indicates that bortezomib is a possible treatment option for patients with AITL.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":" ","pages":"6079633"},"PeriodicalIF":0.7,"publicationDate":"2022-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9453013/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33461149","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}
Alena Strýčková, Jakub Benko, Martin Jozef Péč, Monika Péčová, Jana Žolková, Monika Brunclíková, Tomáš Bolek, Ján Staško, Matej Samoš, Marián Mokáň
{"title":"SARS-CoV-2 Infection-Associated Aortic Thrombosis Treated with Oral Factor Xa Inhibition.","authors":"Alena Strýčková, Jakub Benko, Martin Jozef Péč, Monika Péčová, Jana Žolková, Monika Brunclíková, Tomáš Bolek, Ján Staško, Matej Samoš, Marián Mokáň","doi":"10.1155/2022/7805900","DOIUrl":"https://doi.org/10.1155/2022/7805900","url":null,"abstract":"<p><p>Coronavirus disease 2019 (COVID-19) is an acute complex systemic disorder caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2).While SARS-CoV-2 is known to cause significant pulmonary disease, various extrapulmonary manifestations of COVID-19 have also been reported. Growing evidence suggests that COVID-19 is associated with coagulopathy leading to micro and macrovascular complications. Although in patients with COVID-19, venous thromboembolic events are more frequent, arterial thrombosis also occurs at an increased rate. These often lead to acute life-threatening ischemia, which requires urgent diagnosis and treatment. We present case reports of two patients with an abnormal thrombus formation in the thoracic aorta who recently overcame COVID-19, which led to systemic embolism and splenic infarction. Ambulatory oral factor Xa inhibitor therapy led to aortic thrombosis resolution in both patients.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":" ","pages":"7805900"},"PeriodicalIF":0.7,"publicationDate":"2022-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9436625/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40348258","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}
Sulagna Das, Charles Abreu, Micah Harris, John Shrader, Satish Sarvepalli
{"title":"Severe Thrombocytopenia Associated with Dengue Fever: An Evidence-Based Approach to Management of Thrombocytopenia.","authors":"Sulagna Das, Charles Abreu, Micah Harris, John Shrader, Satish Sarvepalli","doi":"10.1155/2022/3358325","DOIUrl":"https://doi.org/10.1155/2022/3358325","url":null,"abstract":"<p><p>Dengue is a mosquito-borne viral illness common in tropical and subtropical countries but very rare in the United States. Patients infected with dengue often present with thrombocytopenia. In the setting of dengue, platelet transfusions as a treatment for thrombocytopenia have no clear benefits in reduction of severe bleeding or improvement of the platelet count. Here, we present a case of a traveler infected with dengue virus and discuss the approach to treat thrombocytopenia.</p>","PeriodicalId":46307,"journal":{"name":"Case Reports in Hematology","volume":" ","pages":"3358325"},"PeriodicalIF":0.7,"publicationDate":"2022-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9391179/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40628664","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}