Fatal, Rapidly Progressive EBV-Associated Warm Autoantibody Hemolytic Anemia (WAIHA): A Rare Case Study

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S158-S158
Author(s):  
C E Kanakis ◽  
K Gvozdjan ◽  
L Sereseanu ◽  
D Rodheim ◽  
P J DeChristopher
Keyword(s):  
Organ Failure ◽  
Cervical Lymphadenopathy ◽  
Altered Mental Status ◽  
Lymph Node Biopsy ◽  
Blood Bank ◽  
Rapid Progression ◽  
Barr Virus ◽  
Relative Paucity ◽  
Night Sweats

Abstract Introduction/Objective Separately WAIHA and Epstein-Barr Virus-infectious mononucleosis (EBV-IM) are well- studied clinical entities. EBV-IM cases are commonly linked with cold autoagglutinins (IgMs with anti-i specificity), but uncommonly associated with WAIHA. More rarely, EBV-IM can be associated with rapidly fatal AIHA. Prompt and appropriate diagnostic Blood Bank testing for WAIHA in EBV-infected patients should include work-ups to prevent missing the possibility of treating complicating WAIHA. Methods/Case Report A 24-year-old woman presented to the ED with a month of cough/cold symptoms, night sweats, cervical lymphadenopathy, and imaging showing diffuse thoracic lymphadenopathy. DNA/PCR testing was positive for active EBV infection and a lymph node biopsy was diagnostic for EBV lymphadenitis. Increasing respiratory distress developed, coupled with renal and liver insufficiency, hypotension, and possible DIC, and (suspected septic) shock. On hospital day (HD) 5, altered mental status worsened, cardiac arrest occurred requiring resuscitation and vasopressors, and acidosis trended rapidly higher. The patient rapidly decompensated and she was compassionately extubated, expiring on HD 6. There were significant laboratory interval changes in the final 20 hours of life. New positive DATs (with anti-IgG and - C3b/d), a pan-reactive warm autoantibody of broad specificity (in plasma & eluate), onset of acute hemolysis (nadir Hgb to 4.6 g/dL with net drop of 4.7 g/dL & LDH 23, 582 U/L), coagulopathy (peak PT 96.6 sec) and evidnece of organ failure. Blood Bank testing, received on HD 5 at 21:45, was resulted in the EMR on HD 6 at 00:30 and the patient expired at 05:15--further illustrating the rapid clinical course presented in this case. Results (if a Case Study enter NA) N/A Conclusion This patient died of complications directly associated with acute, immune-mediated intravascular hemolysis leading to high-output cardiac failure and resulting in multi-organ failure. With a relative paucity of published EBV-IM WAIHA associations, the rapidly evolving unexpected hematologic sequelae observed complicated the clinical recognition and treatment of severe WAIHA during the course of treatment for clinically suspected DIC. The rapid progression of this patients’ symptoms and laboratory values did not allow for adequate recognition of WAIHA-related pathogenesis and appropriate interventions.

scholarly journals A Case of Relapsing Kikuchi-Fujimoto Disease

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Talayeh Rezayat ◽  
Matthew B. Carroll ◽  
Bryan C. Ramsey ◽  
Andria Smith
Keyword(s):  
Lupus Erythematosus ◽  
Symptomatic Relief ◽  
Specific Treatment ◽  
Cervical Lymphadenopathy ◽  
Severe Case ◽  
Lymph Node Biopsy ◽  
Barr Virus ◽  
Cell Mediated Immune Response ◽  
Night Sweats ◽  
Epstein Barr

Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis was first described in Japan in 1972. It is described as a benign syndrome most commonly involving cervical lymphadenopathy, fever, and night sweats. The etiology of KFD is unknown but it is thought to be triggered by an autoimmune or viral process with an exaggerated T-cell-mediated immune response. KFD can mimic other serious conditions such as lymphoma, systemic lupus erythematosus (SLE), herpes simplex, and Epstein Barr virus. Diagnosis is confirmed histopathologically. Kikuchi’s disease is typically reported to have a self-limiting course, resolving within several months and with a low recurrence rate between 3% and 4%. There is no specific treatment for KFD but any treatment is generally directed towards symptomatic relief with antipyretics and anti-inflammatory medications. In severe cases corticosteroids have been used. Here we describe a case of a previously healthy 26-year-old female that presented with fever and cervical lymphadenopathy. Malignancy and infections were ruled, and she was diagnosed with KFD histopathologically by lymph node biopsy. Her case is a severe case of KFD that despite treatment with multiple courses of corticosteroids and an immune modulating agent, relapsed.

scholarly journals Kikuchi-Fujimoto Disease: A Report of 3 Cases

2007 ◽  
Vol 86 (7) ◽  
pp. 412-413 ◽  
Author(s):  
Eimear Phelan ◽  
Emer Lang ◽  
Peter Gormley ◽  
John Lang
Keyword(s):  
Weight Loss ◽  
Cervical Lymphadenopathy ◽  
Lymph Node Biopsy ◽  
Accurate Diagnosis ◽  
Histiocytic Necrotizing Lymphadenitis ◽  
Kikuchi’S Disease ◽  
Node Biopsy ◽  
Night Sweats ◽  
Necrotizing Lymphadenitis ◽  
Kikuchi's Disease

Cervical lymphadenopathy has many underlying etiologies. One of its rare causes is Kikuchi-Fujimoto disease (Kikuchi's disease, histiocytic necrotizing lymphadenitis). We discovered such a cause in a 37-year-old woman who had presented with malaise, night sweats, and weight loss in addition to cervical lymphadenopathy. We based our diagnosis on excisional lymph node biopsy. We also review 2 other cases of Kikuchi's disease that were diagnosed by others at our institution. Clinically and histologically, Kikuchi's disease is very similar to lymphoma, and distinguishing the two is difficult. However, despite the fact that Kikuchi's disease is benign, an accurate diagnosis is important because misdiagnosis might lead to unnecessary surgery and/or chemotherapy.

scholarly journals Case Report: Kikuchi-Fujimoto disease: a diagnostic and therapeutic dilemma following pretransplant nephrectomy for a 2.35 Kg kidney

F1000Research ◽  
2016 ◽  
Vol 5 ◽  
pp. 1407
Author(s):  
Arvind P. Ganpule ◽  
Jaspreet Singh Chabra ◽  
Abhishek G. Singh ◽  
Gopal R. Tak ◽  
Shailesh Soni ◽  
...  
Keyword(s):  
Lymph Node ◽  
Lupus Erythematosus ◽  
Cervical Lymphadenopathy ◽  
Lymph Node Biopsy ◽  
Adult Polycystic Kidney Disease ◽  
Worldwide Distribution ◽  
Night Sweats ◽  
Necrotizing Lymphadenitis ◽  
Stage Renal Disease ◽  
End Stage

Kikuchi-Fujimoto disease (KFD) is an extremely rare disease with a worldwide distribution and higher prevalence in Asians. It is a benign and self-limiting disorder, characterized by regional cervical lymphadenopathy accompanied with mild fever and night sweats. Lymph node histopathology is diagnostic and treating physicians should be aware of this entity as it may mimic other systemic diseases like systemic lupus erythematosus, tuberculosis, malignant lymphoma, and more rarely adenocarcinoma. Key features on lymph node biopsy are fragmentation, necrosis and karyorrhexis. Treatment includes symptomatic care, analgesics-antipyretics, corticosteroids and spontaneous recovery occurs in 1 to 4 months. We report a case of adult polycystic kidney disease (ADPKD) with end stage renal disease and episodes of fever and cervical lymphadenopathy. The infectious screen was negative and on extensive workup, the patient was found to have histiocytic-necrotizing lymphadenitis, which clinched the diagnosis of KFD.

scholarly journals Castleman’s Disease and Posttransplant Lymphoproliferative Disorder after Liver Transplant: 3-Year Follow-Up

2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Lokesh K. Jha ◽  
Laura L. Ulmer ◽  
Marco A. Olivera-Martinez ◽  
Timothy M. McCashland ◽  
Kai Fu ◽  
...  
Keyword(s):  
Liver Transplant ◽  
Castleman’S Disease ◽  
Lymph Node Biopsy ◽  
Castleman's Disease ◽  
Human Herpes Virus 8 ◽  
Barr Virus ◽  
Night Sweats ◽  
History Of ◽  
Epstein Barr

A 59-year-old male with a history of hepatitis C cirrhosis and history of hepatitis B exposure presented 8 months after orthotopic liver transplant (LT) with fever, fatigue, myalgia, night sweats, nonproductive cough, and shortness of breath. Bone marrow biopsy for pancytopenia was positive for Epstein-Barr virus (EBV) DNA. Lymph node biopsy for lymphadenopathy on imaging showed human herpes virus 8 (HHV8) associated Castleman’s disease. Treatment included valganciclovir, rituximab, and prednisone taper with eventual discontinuation. Quantitative HHV8 DNA was initially 611,000 DNA copies/mL and was later undetectable at 6 months following treatment and remained undetectable at 3-year follow-up.

Merkel Cell Carcinoma of the Lower Extremity

2014 ◽  
Vol 104 (4) ◽  
pp. 422-425 ◽  
Author(s):  
Dominic J. Roda ◽  
Brian Albano ◽  
Bharti Rathore ◽  
Linda Zhou
Keyword(s):  
Lower Extremity ◽  
Cell Carcinoma ◽  
Merkel Cell Carcinoma ◽  
Clinical Presentation ◽  
Treatment Guidelines ◽  
Excisional Biopsy ◽  
Lymph Node Biopsy ◽  
Rapid Progression ◽  
Merkel Cell ◽  
Unusual Lesion

Merkel cell carcinoma is a rare, often fatal, neuroendocrine cutaneous malignancy with a highly variable clinical presentation. Due to the rapid progression of the disease, early detection and treatment is vital to survival. Here, we report the case of a 45-year-old woman whose podiatrist noticed an unusual lesion on her lower left leg and referred her to a dermatologist for work-up. A diagnostic excisional biopsy confirmed the diagnosis and was followed by treatment that included wide local excision of the primary lesion with subsequent chemotherapy and radiation. At the time of diagnosis, sentinel lymph node biopsy was positive. Due to the increasing prevalence of Merkel cell carcinoma in the past decade and its propensity to present on the lower extremity, podiatric physicians need to be aware of the clinical presentation and treatment guidelines for this elusive disease.

scholarly journals Simultaneous Occurrence of Choriocarcinoma in an Infant and Mother

2021 ◽  
Vol 18 (4) ◽  
pp. 1934
Author(s):  
Małgorzata Rzanny-Owczarzak ◽  
Joanna Sawicka-Metkowska ◽  
Katarzyna Jończyk-Potoczna ◽  
Ewelina Gowin ◽  
Patrycja Sosnowska-Sienkiewicz ◽  
...  
Keyword(s):  
Rare Disease ◽  
Organ Failure ◽  
Liver Tumor ◽  
Serum Levels ◽  
Simultaneous Occurrence ◽  
Positive History ◽  
Multi Organ Failure ◽  
History Of

Infantile choriocarcinoma is an extremely rare disease. We present a case study of a 1-month-old male with choriocarcinoma diagnosed simultaneously with his mother. On admission to hospital, the disease was very advanced and massive progression and multi-organ failure caused the death of the patient despite the implemented treatment. It was too late to save the child’s life, but early enough to save his mother. The authors believe that the serum levels of hCG should be determined in every newborn with anemia and liver tumor, especially when the mother has a positive history of miscarriage.

Epstein–Barr virus infection as a cause of cervical lymphadenopathy in children

2011 ◽  
Vol 75 (4) ◽  
pp. 564-567 ◽  
Author(s):  
Mosaad Abdel-Aziz ◽  
Hassan El-Hoshy ◽  
Mohammed Rashed ◽  
Mohamed Qotb ◽  
Seham Awad ◽  
...  
Keyword(s):  
Virus Infection ◽  
Epstein Barr Virus ◽  
Cervical Lymphadenopathy ◽  
Epstein Barr Virus Infection ◽  
Barr Virus ◽  
Epstein Barr ◽  
Barr Virus Infection

scholarly journals Angioimmunoblastic T-cell lymphoma presenting with an acute serologic Epstein-Barr virus profile

2015 ◽  
Vol 7 (2) ◽  
Author(s):  
Timothy Beer ◽  
Patrick Dorion
Keyword(s):  
T Cell ◽  
Epstein Barr Virus ◽  
Cell Lymphoma ◽  
Lymph Node Biopsy ◽  
T Cell Lymphoma ◽  
High Endothelial Venules ◽  
Angioimmunoblastic T Cell Lymphoma ◽  
Barr Virus ◽  
Epstein Barr ◽  
High Index Of Suspicion

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive peripheral T-cell lymphoma typically characterized by prominent lymphadenopathy and B-symptoms at the time of presentation, polyclonal hypergammaglobulinemia, autoimmune hemolysis and frequent but highly variable involvement of Epstein- Barr virus (EBV). Lymph node biopsy findings typically include effacement of nodal architecture, polymorphic infiltrate, atypical T-cells (usually CD4+/CD10+/PD1+) and prominent proliferations of high endothelial venules and follicular dendritic cells. However, this classic constellation of pathologic findings is often initially obscured by a prominence of EBV+ B-immunoblasts with or without associated peripherally circulating EBV DNA. Here we document the first reported case of an acute serologic EBV profile (VCA-IgM) in a patient with AITL, and we recommend that clinicians maintain a high index of suspicion for AITL in the appropriate clinical scenario, irrespective of Epstein-Barr related findings.

scholarly journals Giving Dracula the Keys to the Blood bank? Interrogating the Fifth Crown Casino Licensing Regulatory Review

2013 ◽  
Vol 8 (1) ◽  
Author(s):  
Linda Hancock
Keyword(s):  
European Commission ◽  
Narrative Analysis ◽  
Conflicts Of Interest ◽  
Code Of Conduct ◽  
Responsible Gambling ◽  
Blood Bank ◽  
Regulatory Review ◽  
Policy Priority ◽  
Recent Policy

Drawing on narrative analysis, this paper analyses the 2013 Fifth Regulatory Review of the license of an Australian casino as a case study focused on the framing and articulation of ‘responsible gambling’ (RG) in the Review. Part 1 sets out the policy and regulatory context for the licensing review of Melbourne’s Crown Casino. Part 2 overviews the structure/content of the Review; the key messages of the Reviewers’ narrative and its main recommendations. In reflecting on the Review in Part 3, analysis focuses on the investigation and recommendations regarding Responsible Gambling, which has gained recent policy priority. The analysis interrogates the Review’s findings, narratives, processes and evidentiary base in relation to how it presents and assesses casino performance on RG. In doing so, it focuses on the Victorian Commission for Gambling and Liquor Regulation’s Review’s framing of RG; sources of evidence drawn on by the Review; an assessment of the casino’s loyalty club feature ‘Play Safe’, as an RG measure; the Review’s assessment of casino performance on RG and its Code of Conduct in particular; and the Review’s framing of RG recommendations. It concludes with reflections on governance issues raised by the Review, the need for more focus on the neglected area of regulatory licensing and enforcement (OECD, 2011; 2012; OECD & European Commission, 2009) and the need for independent regulatory reviews that address conflicts of interest on the part of both Government and the Regulator.

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