scholarly journals Hemophagocytic lymphohistiocytosis and thrombotic microangiopathy after parvovirus B19 infection and renal transplantation: a case report

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
C. J. Steffen ◽  
N. Koch ◽  
K. U. Eckardt ◽  
K. Amann ◽  
E. Seelow ◽  
...  

Abstract Background Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disease characterized by hyperactivation of the immune system that causes hypercytokinemia and potentially multi organ failure. HLH can occur in patients with underlying rheumatic or autoinflammatory disorders. Additionally, HLH can develop in patients during infections or malignancies without a known genetic predisposition. Case presentation We herein report a patient, who presented with fever, both acute kidney and liver injury, anemia, thrombocytopenia and HSV stomatitis. HLH was diagnosed based on clinical criteria and qPCR revealed an acute parvovirus B19 infection as potential underlying infectious trigger. Treatment was started with both IVIG and dexamethasone. Subsequently, kidney biopsy demonstrated TMA. Conclusions In rare cases both HLH and aHUS can occur simultaneously in a patient as a consequence of viral infections. Insights from this unusual case might help physicians understand this complex symptom constellation.

Author(s):  
Katie Jeffery

Viral infection in pregnancy can lead to adverse outcomes for both the mother and the foetus. This chapter examines the steps to be taken on exposure to viruses associated with rash illness in pregnancy. Consideration is given to the important points in the history, which viral infections are of concern in pregnancy (varicella-zoster, parvovirus B19, rubella, measles, and Zika), the possible outcomes of infection, diagnostic approaches, and the management of a confirmed case of Parvovirus B19 infection.


2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Nonso Osakwe ◽  
Diane Johnson ◽  
Natalie Klein ◽  
Dalia Abdel Azim

Background. Hemophagocytic lymphohistiocytosis (HLH) is a rare condition associated with viral infections including HIV. Cases have been reported mainly in advanced HIV/AIDS. This is a rare case that reports HLH associated with human herpes virus-8 (HHV-8) associated multicentric Castleman disease in a stable HIV patient. Case Presentation. A 70-year-old Asian male patient with history of stable HIV on medications with CD 4 cell count above 200 presented with cough and fever and was initially treated for pneumonia as an outpatient. Persisting symptoms prompted presentation to the hospital. The patient was found to have anemia which persisted despite repeated transfusion of packed red cells. A bone marrow biopsy to investigate anemia revealed hemophagocytosis. A CT scan revealed multiple enlarged lymph nodes and hepatosplenomegaly. An excisional lymph node biopsy revealed HHV-8 associated multicentric Castleman disease. The patient deteriorated despite initiation of treatment. Conclusion. HLH can occur at any stage of HIV, rapid diagnosis to identify possible underlying reactive infectious etiology and prompt initiation of treatment is crucial to survival.


2014 ◽  
Vol 124 ◽  
pp. 438-441 ◽  
Author(s):  
Michinori Mayama ◽  
Masato Yoshihara ◽  
Tetsuya Kokabu ◽  
Hidenori Oguchi

2012 ◽  
Vol 2012 ◽  
pp. 1-2 ◽  
Author(s):  
Nathan D. Oliver ◽  
Auleen Millar ◽  
Adrian Pendleton

Introduction. Whilst there are reports of viral myopathies affecting children and the immunocompromised, infective myositis is a relatively rare inflammatory myopathy in adults. The clinical spectrum can range from benign myalgias to more serious complications in certain risk groups.Case Presentation. We present two cases of myositis as a result of parvovirus B19 infection.Conclusion. Viral myositis and parvovirus B19 associated myositis should be considered in adults presenting with significant myalgia.


2019 ◽  
Vol 7 ◽  
pp. 232470961988369
Author(s):  
Precious Macauley ◽  
Mohammad Abu-Hishmeh ◽  
Carissa Dumancas ◽  
Vijay Alexander-Rajan ◽  
Fernando Piedra-Chavez ◽  
...  

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening condition characterized by widespread inflammation due to massive immune activation and cytokine release. It is of 2 types, primary or familial and secondary or acquired. Diagnosis is made by fulfilling 5 of 8 criteria as determined by the Histiocyte Society. Treatment includes etoposide, dexamethasone, with or without intrathecal methotrexate in the presence of neurologic involvement as well as treating the underlying cause in secondary HLH. We present a case of a 23-year-old female with congenital human immunodeficiency virus (HIV) infection who presents with nonspecific signs and symptoms of cough, fever, leukopenia, and anemia, and a high-serum parvovirus B19 DNA, later diagnosed with HLH and treated with etoposide and dexamethasone. She made clinical improvements and was successfully discharged to home after 26 days of admission.


Hematology ◽  
2009 ◽  
Vol 2009 (1) ◽  
pp. 127-131 ◽  
Author(s):  
Alexandra H. Filipovich

Abstract Hemophagocytic lymphohistiocytosis (HLH), which has many genetic causes, is characterized by multi-system inflammation. HLH is a reactive process resulting from prolonged and excessive activation of antigen presenting cells (macrophages, histiocytes) and CD8+ T cells. Hemophagocytosis, which is mediated through the CD163 heme-scavenging receptor, is a hallmark of activated macrophages/histiocytes and is the characteristic finding for which the disorder was named. The majority of genetic causes identified to date affect the cytotoxic function of NK and T cells, crippling immunologic mechanisms that mediate natural immune contraction. The predominant clinical findings of HLH are fevers (often hectic and persistent), cytopenias, hepatitis and splenomegaly. Due to the life-threatening implications of the diagnosis of genetically determined HLH, antiinflammatory therapy, often consisting of steroids, etoposide or antithymocyte globulin (ATG), should be instituted promptly, followed by curative hematopoietic cell transplantation. Secondary HLH, associated with autoimmune disorders or viral infections in teens and adults, also carries a significant mortality rate and should be managed in consultation with specialists familiar with the diagnosis and treatment of such disorders.


2013 ◽  
Vol 32 (7) ◽  
pp. 789-792 ◽  
Author(s):  
Marine Butin ◽  
Yahia Mekki ◽  
Alice Phan ◽  
Geneviève Billaud ◽  
Sylvie Di Filippo ◽  
...  

2020 ◽  
Vol 9 (1) ◽  
Author(s):  
Marika Toscano ◽  
Elizabeth Westen ◽  
Stefanie J. Hollenbach ◽  
Archibald S. Perkins ◽  
Loralei L. Thornburg

AbstractBackgroundHemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disease. It is characterized by impaired natural killer cell and cytotoxic lymphocyte function leading to dysregulated macrophage phagocytosis of erythrocytes, leukocytes and platelets. Coupled with a massive release of cytokines this rapidly leads to multi-organ failure and mortality if not treated promptly.Case presentationWe report a case of pregnancy-associated, treatment-resistant HLH with no identified risk factors.ConclusionIt is likely that HLH is an unrecognized cause of intractable fever and disseminated intravascular coagulation (DIC) in young pregnant women and it should be included in the differential diagnosis when patients do not respond to typical therapies. High serum ferritin should further prompt consideration of this diagnosis. This rare disease is often not thought of and many gynecologist obstetricians have never heard of it, making this case report of essential importance to reignite the interest in HLH, particularly when it occurs during pregnancy.


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