scholarly journals Kikuchi-Fujimoto Disease: A Case Report

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4189-4189
Author(s):  
Jonathan Hickman ◽  
Joseph An

Abstract Kikuchi-Fujimoto: A Case Report Hickman, JD. MD LT MC USN and An, Joseph, DO. LCDR MC USN Naval Medical Center Portsmouth 620 Johns Paul John Cir, Portsmouth VA 757-953-2223 The views expressed in this abstract are those of the authors and do not necessarily reflect the official policy or position of the Department of the Navy, Department of Defense, or the United States Government. We are military service members and employees of the U.S. Government. This work was prepared as part of my official duties. Title 17 U.S.C. 105 provides that "Copyright protection under this title is not available for any work of the United States Government." Title 17 U.S.C. 101 defines a United States Government work as a work prepared by a military service member or employee of the United States Government as part of that person's official duties. Kikuchi-Fujimoto disease is a rare benign disorder often presenting with tender cervical lymphadenopathy, fever, and malaise. While first described in Japan, its distribution is worldwide and predominantly seen in young adults. Diagnosis is based on characteristic histopathologic findings of patchy necrosis occupied by karyorrhectic debris and abundant histiocytes on node biopsy. The origin is unclear but associated with a preceding viral illness as well as cutaneous lupus erythematosus. Treatment is generally supportive and focused on managing tender lymph nodes. We present a case of a 28-year-old female presenting with a 2 month history of night sweats, fever, and weight loss in the setting of painful neck swelling. CT and PET/CT imaging demonstrated numerous hypermetabolic and enlarged nodes in the bilateral cervical and axillary regions. Lab studies were notable for leukopenia, anemia, and elevated inflammatory markers. A COVID-19 screening was negative. Excisional biopsy of a cervical node revealed extensive cortical necrosis and apoptotic debris with scattered histiocytes and plasmacytoid dendritic cells in absence of neutrophils or a monoclonal B cell or T cell population. Treatment was initiated with NSAIDs and close monitoring. The patient exhibited a complete response after two months. Our case is an important reminder that lymphadenopathy, fever, and night sweats in a young adult are not pathognomonic for lymphoma. Nonetheless, a high suspicion for lymphoma should be maintained and followed with an expedited workup. Kikuchi-Fujimoto can certainly mimic Hodgkin lymphoma or other serious conditions like lupus erythematosus and tuberculosis. The diagnosis is largely one of exclusion following a careful examination of a lymph node histopathology and must be considered in young previously healthy adults to avoid misdiagnosis and unnecessary escalation of treatment. Disclosures No relevant conflicts of interest to declare.

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5829-5829
Author(s):  
Marianne Ulcickas Yood ◽  
Susan Jick ◽  
Catherine Vasilakis-Scaramozza ◽  
Bonnie M.K. Donato ◽  
Ioannis Tomazos ◽  
...  

Abstract Background: For patients suffering from rare diseases, accurate and early diagnosis is critical and often lifesaving, whereas misdiagnosis can be fatal. While patient registries are useful and necessary, they may not provide reliable patient population denominators or sufficient longitudinal clinical follow-up. Access to complete and integrated patient records necessary to capture full clinical history can be challenging. Commonly, there is a lack of centralized and continuous care in the health care systems of many countries, including the United States (US). It is also challenging to identify a sufficient number of cases to provide robust results because of the rare nature of these diseases. We describe a healthcare system that can identify patients for research purposes, who have rare diseases, by accessing de-identified electronic clinical details. We used Paroxysmal Nocturnal Hemoglobinuria (PNH) as an example of a rare disease for this abstract. Methods: The Department of Defense (DOD) healthcare system is a US-based, longitudinal electronic health record (EHR) and claims database with health information on approximately 10 million active beneficiaries across the country. We evaluated the feasibility to conduct studies of rare diseases in the DOD healthcare system by assessing the capability to identify patients with PNH and to describe their course of disease and treatment. We used ICD 9/10 diagnosis codes, NDC and HCPCS codes, laboratory data and PNH treatment codes to identify patients with a clinical course consistent with PNH. Patients were classified as definite/likely, probable/possible or unlikely PNH based on the available clinical evidence and then findings were validated against review of patient records by a clinical expert. Individuals classified as unlikely PNH were excluded from the study. The clinical information on these patients will be used to understand the course of PNH in patients with and without treatments and to describe their treatment adherence and disease activity over time. Results: We identified 244 people with a diagnosis or treatment code that was indicative of PNH during years 2007-2017; 71% of the patients had electronic records that covered 10 or more years starting as early as 2003 and extending as far as 2017. From these 244 patients, we identified 73 patients with a definite/likely or probable/ possible PNH diagnosis. An ICD-10 code for PNH or a prescription for eculizumab (PNH treatment) were required, but not sufficient to confirm the presence of PNH. There is no ICD-9 code for PNH. Cases had no other indication for eculizumab use and had to have appropriate symptoms, comorbidities or lab results to be considered a case. Patients with only 1 code for PNH and no treatment were assumed to have unconfirmed disease and were excluded. 27 cases, including those with no eculizumab and a random sample of likely cases, were reviewed to validate the PNH diagnoses in collaboration with DOD treating physicians. Conclusion: The DOD healthcare system is a valuable and cost effective resource for the study of rare diseases in a timely manner. We have demonstrated the ability to identify a validated series of PNH cases that will provide important clinical insights for identifying and treating new PNH cases. This healthcare system provides long patient follow-up, demographics similar to the US population, and access to records in an integrated inpatient, outpatient and ER system that encompasses all patient care. Disclaimer Statement: Research data were derived from an approved Naval Medical Center, Portsmouth, VA IRB protocol (NMCP.2017.0080). The views expressed in this abstract are those of the authors and do not necessarily reflect the official policy or position of the Department of the Navy, Department of Defense or the United States Government. Copyright Notice: CAPT Brian Feldman is a military service member. This work was prepared as part of his official duties. Title 17 U.S.C. 105 provides that 'Copyright protection under this title is not available for any work of the United States Government.' Title 17 U.S.C. 101 defines a United States Government work as a work prepared by a military service member or employee of the United States Government as part of that person's official duties. Disclosures Ulcickas Yood: Alexion Pharmaceuticals, Inc.: Other: Employee of EpiSource, LLC, which was contracted by Alexion Pharmaceuticals, Inc. EpiSource had the final decision on content. . Jick:Alexion Pharmaceuticals, Inc.: Other: Employee of the Boston Collaborative Drug Surveillance Program, which was contracted and paid by Alexion Pharmaceuticals, Inc. to work on a study of PNH using DOD data. . Vasilakis-Scaramozza:Alexion Pharmaceuticals, Inc.: Other: Employee of the Boston Collaborative Drug Surveillance Program, which was contracted and paid by Alexion Pharmaceuticals, Inc. to work on a study of PNH using DOD data.. Donato:Alexion Pharmaceuticals Inc: Employment, Equity Ownership. Tomazos:Alexion Pharmaceuticals, Inc.: Employment, Equity Ownership. L'Italien:Alexion Pharmaceuticals, Inc.: Equity Ownership, Other: Former employee and current stockholder of Alexion Pharmaceuticals, Inc. . Sicignano:Alexion Pharmaceuticals, Inc.: Other: Employee of Health ResearchTx, which has a business relationship with Alexion Pharmaceuticals, Inc.. Feldman:Alexion Pharmaceuticals, Inc.: Other: Employee, Department of Navy, United States Government..


Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5830-5830
Author(s):  
Marianne Ulcickas Yood ◽  
Susan Jick ◽  
Catherine Vasilakis-Scaramozza ◽  
Bonnie M.K. Donato ◽  
Ioannis Tomazos ◽  
...  

Abstract Background: Rare diseases are often characterized by misdiagnosis resulting in delays in critical and potentially lifesaving treatment. Awareness of first signs and symptoms of rare diseases can provide clinical evidence for early and accurate diagnosis. Medical records are the primary source of clinical information from first signs and symptoms to key clinical disease related events, yet access to records can be difficult and time consuming. We conducted a study using a large electronic medical record (EMR) and claims database to identify and describe characteristics of Paroxysmal Nocturnal Hemoglobinuria (PNH) patients at the time of their PNH diagnosis. We present baseline findings of the PNH population identified in the Department of Defense (DOD) healthcare system, where all clinical details were readily available. The DOD healthcare system is a US-based, longitudinal EMR and claims database with health information on approximately 10 million active beneficiaries throughout the country. Methods: We identified all people in the DOD database from January 01, 2007 through May 31, 2017 who had an ICD-10-CM code for PNH or a NDC or HCPCS code for eculizumab, the drug used to treat PNH. There is no ICD-9-CM code for PNH. Cases had no other indication for eculizumab use and had to have appropriate symptoms, comorbidities or lab results to be considered a case. We reviewed the electronic record for each patient and classified each as definite/likely, probable/possible or unlikely PNH based on attributes of the available health data including codes for eculizumab, flow cytometry, lab results, hemoglobinuria, aplastic anemia, pancytopenia and other comorbidities. Our ruling was validated by medical record review by a clinical expert for all equivocal cases and a sample of definite/likely cases. Individuals classified as unlikely PNH were excluded. Patient characteristics at PNH diagnosis are presented using descriptive statistics. Results: We identified 73 PNH patients (55% female) after review of all available electronic data; 41 had a diagnosis of PNH and received eculizumab, 17 had a diagnosis of PNH only, and 15 received eculizumab only. From these, 61 patients were determined to have definite/likely PNH and 12 had probable/possible PNH. The use of eculizumab ranged from 0 to 378 (median=18) prescriptions, dependent in part on the amount of follow-up in the database. There were 19 patients (26%) who had prevalent PNH and 54 (74%) who were newly diagnosed with PNH during the study period. Among newly diagnosed patients, the median age at PNH diagnosis was 46 years (range 13 - 86). Most patients had codes for hemoglobinuria at or before the PNH diagnosis date (69%), and many had codes for aplastic anemia (46%), thrombocytopenia (56%), and pancytopenia (28%). At any time after the PNH diagnosis, 85% had unspecified anemia, 52% had aplastic anemia, 53% had thrombocytopenia, and 33% had pancytopenia codes in their records. See table. Conclusion: From a population of around 10 million actively enrolled patients, across a 10-year study period, we identified 73 patients with PNH. Data from these patients will be used to identify patterns of health encounters leading up to diagnosis and relevant outcomes following diagnosis. This information can be used to help diagnose other patients with this rare disease and to improve their medical outcomes. Disclaimer Statement: Research data were derived from an approved Naval Medical Center, Portsmouth, VA IRB protocol (NMCP.2017.0080). The views expressed in this abstract are those of the authors and do not necessarily reflect the official policy or position of the Department of the Navy, Department of Defense or the United States Government. Copyright Notice: CAPT Brian Feldman is a military service member. This work was prepared as part of his official duties. Title 17 U.S.C. 105 provides that 'Copyright protection under this title is not available for any work of the United States Government.' Title 17 U.S.C. 101 defines a United States Government work as a work prepared by a military service member or employee of the United States Government as part of that person's official duties. Table. Table. Disclosures Ulcickas Yood: Alexion Pharmaceuticals, Inc.: Other: Employee of EpiSource, LLC, which was contracted by Alexion Pharmaceuticals, Inc. EpiSource had the final decision on content. . Jick:Alexion Pharmaceuticals, Inc.: Other: Employee of the Boston Collaborative Drug Surveillance Program, which was contracted and paid by Alexion Pharmaceuticals, Inc. to work on a study of PNH using DOD data. . Vasilakis-Scaramozza:Alexion Pharmaceuticals, Inc.: Other: Employee of the Boston Collaborative Drug Surveillance Program, which was contracted and paid by Alexion Pharmaceuticals, Inc. to work on a study of PNH using DOD data.. Donato:Alexion Pharmaceuticals Inc: Employment, Equity Ownership. Tomazos:Alexion Pharmaceuticals, Inc.: Employment, Equity Ownership. L'Italien:Alexion Pharmaceuticals, Inc.: Equity Ownership, Other: Former employee and current stockholder of Alexion Pharmaceuticals, Inc. . Sicignano:Alexion Pharmaceuticals, Inc.: Other: Employee of Health ResearchTx, which has a business relationship with Alexion Pharmaceuticals, Inc.. Feldman:Alexion Pharmaceuticals, Inc.: Other: Employee, Department of Navy, United States Government..


2010 ◽  
Vol 43 (01) ◽  
pp. 127-131 ◽  
Author(s):  
Leonard Champney ◽  
Paul Edleman

AbstractThis study employs the Solomon Four-Group Design to measure student knowledge of the United States government and student knowledge of current events at the beginning of a U.S. government course and at the end. In both areas, knowledge improves significantly. Regarding knowledge of the U.S. government, both males and females improve at similar rates, those with higher and lower GPAs improve at similar rates, and political science majors improve at similar rates to non-majors. Regarding current events, males and females improve at similar rates. However, those with higher GPAs and political science majors improve more than others.


1963 ◽  
Vol 17 (1) ◽  
pp. 226-230

The Security Council discussed this question at its 1022nd–1025th meetings, on October 23–25, 1962. It had before it a letter dated October 22, 1962, from the permanent representative of the United States, in which it was stated that the establishment of missile bases in Cuba constituted a grave threat to the peace and security of the world; a letter of the same date from the permanent representative of Cuba, claiming that the United States naval blockade of Cuba constituted an act of war; and a letter also dated October 22 from the deputy permanent representative of the Soviet Union, emphasizing that Soviet assistance to Cuba was exclusively designed to improve Cuba's defensive capacity and that the United States government had committed a provocative act and an unprecedented violation of international law in its blockade.


Slavic Review ◽  
1969 ◽  
Vol 28 (2) ◽  
pp. 276-288 ◽  
Author(s):  
Benjamin M. Weissman

In March 1921 Lenin predicted, “If there is a harvest, everybody will hunger a little and the government will be saved. Otherwise, since we cannot take anything from people who do not have the means to satisfy their own hunger, the government will perish.“ By early summer, Russia was in the grip of one of the worst famines in its history. Lenin's gloomy forecast, however, was never put to the test. At almost the last moment, substantial help in the form of food, clothing, and medical supplies arrived from a most unexpected source —U.S. Secretary of Commerce Herbert Hoover.Hoover undertook the relief of Soviet Russia not as an official representative of the United States government but as the head of a private agency —the American Relief Administration (A.R.A.).


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