echemi logo
Product
  • Product
  • Supplier
  • Inquiry
    Home > Active Ingredient News > Infection > A case study of 1 pregnancy complicated by hemophagocytic syndrome

    A case study of 1 pregnancy complicated by hemophagocytic syndrome

    • Last Update: 2022-09-20
    • Source: Internet
    • Author: User
    Search more information of high quality chemicals, good prices and reliable suppliers, visit www.echemi.com

    preface

    The patient was admitted to the hospital


    Case after

    Patient, female, 24 years old, 31 weeks


    Past menstrual patterns, LMP September 15, 2020, EDC June 22, 2021


    Past and personal history: Plain and healthy


    Physical examination after admission: body temperature 36.


    Auxiliary examination: total protein checked in the hospital: 56.


    Admission diagnosis: 1.


    On the second day of admission (4.


    On the 3rd day of admission (4.


    Postoperative diagnosis: 1.


    After caesarean section, ICU treatment was transferred to the ICU, and anti-infection (cefoperazone sodium sulbactam sodium + ornidazole, antibiotics were discontinued until the 3rd day after surgery), hepatoprotective and other treatments


    Bone marrow aspiration results (5.


    (The results of the bone marrow puncture during treatment (5.


    Changes in the patient's condition during hospitalization

    Specific circumstances and handling methods

     

    Case studies

    Clinician analysis

    (1) The patient's distinctive clinical features are persistent hyperthermia and abnormal


    (2) Causes of abnormal liver function during pregnancy include:

    (1) Liver disease caused by pregnancy: a.


    (2) Pregnancy complicated by liver disease: pregnancy with chronic liver disease and new liver disease during pregnancy; Among them, pregnancy with chronic liver disease includes: a.


    (3) Drug-induced liver injury (DILI): a.
    women are more likely to develop acute liver injury; b.
    women are risk factors for certain drug-related DILI, such as minocycline and nitrofurantoin; c.
    pregnant women are prone to DILI (current evidence is preferred).

    (3) Causes of fever during pregnancy include infectious fever and non-infectious fever

    (1) Infectious fever is common in the respiratory system, urinary system or digestive system by bacteria or viruses and cause systemic or local infection, clinical often with acute fever as a clinical feature, the diagnosis needs to be combined with the patient's fever type, heat course, physical examination and comprehensive analysis of laboratory results; In addition, fever during pregnancy also needs to exclude intrauterine infection caused by premature rupture of membranes; At the same time, if the maternal fever has a long course, it is also necessary to distinguish
    it from rare clinical causes such as tuberculosis, cytomegalovirus, microvirus B19 and Brucella.

    (2) Non-infectious is common in lupus erythematosus, Still disease, polymyositis, polyarteritis nodosum, tumor fever, hyperthyroidism, functional fever, diencephalic syndrome, etc
    .

    (4) In view of the patient's clinical diagnosis and treatment, consider the secondary hemophagocytic syndrome
    after Epstein-Barr virus infection.

    Laboratory physician analysis

    1.
    After the patient was admitted to the hospital, the examination found that lymphocytes% were 9.
    40%, lymphocyte count: 0.
    40* 10^9/, erythrocyte count: 3.
    09*10^12/, hemoglobin: 93.
    0g/L, all lower than the normal reference range, considering that the blood count may be due to the patient's pregnancy, it is recommended to continue to pay attention to the patient's blood count results
    .

    2.
    Because the patient continues to have fever, the non-specific infection indicators CRP and PCT are checked, showing that the patient's two indicators are only mildly elevated, CRP is usually elevated in bacterial infection and autoimmune diseases, while viral infection is not
    .
    PCT is mainly stimulated by bacterial endotoxins, usually in the case of viral infection alone, PCT is not elevated, and the patient's blood culture results are negative, indicating that the patient is more likely
    to have viral infection.

    3.
    The patient continued to have fever after admission, and on the ninth day, the blood count was significantly lower than that of the previous days, and no abnormalities
    were found in the examination of the patient's A gong, TORCH, tuberculosis, hepatitis B, hepatitis C, hepatitis A, hepatitis E, CMV, and herpes virus.
    Patients test positive for Epstein-Barr virus, which proliferates in oropharyngeal epithelial cells and then infects B lymphocytes, which enter the blood circulation in large numbers, causing systemic infection and long-term latent in
    human lymphoid tissue.
    EBV infection can present as a proliferative infection and an underlying infection
    .
    Different infectious states express different antigens, antigens EBV early antigens, EBV capsid proteins and EBV membrane antigens expressed during proliferative infection, and antigens EBV nuclear antigens and latent membrane proteins
    expressed during latent infection.
    The common disease is infectious mononucleosis, but patients do not show a large increase in monocytes and atypical lymphocytes in the peripheral blood and are therefore not considered
    .

    4.
    After the consultation of the hematology department, it is recommended to improve the bone marrow puncture examination, the bone puncture results suggest that the bone marrow hyperplasia is active, the granulous erythroid hyperplasia is obvious, and the phagocyte phagocytosis phenomenon is occasionally seen
    .

    Knowledge development

    Hemophagocyticsyndrome (HPS), also known as hemophagocytic lymphohistrome, was first reported by Risdall in 1979, mainly due to cytotoxic killer cell (CTL) and NK cell function defects leading to antigen clearance disorders, mononuclear macrophage system receiving continuous antigen stimulation and overactivation proliferation, resulting in a large number of inflammatory cytokines resulting in a group of clinical syndromes
    .

    Hemophagocytic syndrome is divided into primary HPS and secondary HPS according to genetic factors, the former is autosomal recessive inheritance or X-linked inheritance, with a clear genetic defect or family history
    .
    The latter can be caused
    by a variety of factors, such as infection (mainly Epstein-Barr virus infection), malignancy, autoimmune diseases, drugs, acquired immunodeficiency (e.
    g.
    , transplantation), and so on.
    Secondary HPS has also been reported to occur during pregnancy, usually in the second and third trimesters
    of pregnancy [1-2].
    Immune disorders and changes due to the presence of xenogenetic fetal material during pregnancy are possible factors that precipitate HPS [3
    ].

    Presents with fever, splenomegaly, pancytopenia, elevated serum ferritin, elevated triacylglycerol, decreased fibrinogen, and blood phagocytosis may be found on bone marrow, spleen, or lymph node biopsy
    .
    The annual incidence worldwide is 1 in 800,000, with the vast majority of adult cases occurring in Asia
    .
    The disease has a high fatality rate, and timely diagnosis and treatment are essential, but due to its rare and complex clinical manifestations, as well as insufficient awareness of the disease, it often leads to delayed or missed diagnosis, resulting in high mortality [4].

    The cause of the disease is unknown, the condition is dangerous, and the prognosis is poor
    .

    The mechanism of secondary HLH is unclear
    .
    A reasonable hypothesis is that dysfunction of the T cells and NK cells that drive the HLH phenotype is caused by viral infection or chronic antigen stimulation of malignant tumors
    .
    The best example is EBV-associated HLH, where the EBV latent membrane protein (LMP1) interferes with the T cell adapter protein, i.
    e.
    , signal transduction lymphocytes to activate molecularly associated proteins, which in turn leads to overactivation of T cells and secretion of Th1 cytokines [5
    ].
    With the advent of whole-exome and whole-genome sequencing and its application in this subgroup of rare diseases, new genetic drivers and modifiers are likely to be discovered in the future [6].

    The mechanism of pregnancy-concomitant hemophagocytic syndrome has not been well understood, and one theory is that the immature placenta releases fragments of the trophoblast layer containing fetal RNA and DNA components into the maternal circulation, leading to a systemic inflammatory response [7].

    Treatment options: The basic treatment principle of current HLH is to combine immunosuppression and cytotoxic therapy to target high inflammatory states
    .
    The HLH-2004 regimen [8] is currently recognized as an HLH treatment regimen, as well as monoclonal antibody therapy and hematopoietic stem cell therapy
    .
    Rituximab has been reported [5] as a rescue treatment option for patients with EBV-associated
    HLH.
    Before initiating treatment, the HLH case fatality rate was about 95% and the median survival was 1 to 2 months
    .
    Uncertainty about the diagnosis of HLH has a serious impact on prognostic assessment and treatment decisions
    .
    Therefore, early recognition and treatment are essential, as is emergency referral to the Department of Haematology and Oncology
    .

    Case summary

    Through the treatment of this case, we summarize the following experiences:

    (1) Infection during pregnancy can cause adverse outcomes, and infection may cause lung infection, brain abscess, etc.
    , resulting in patient death, which should be highly valued
    .

    (2) Improving relevant examinations and actively performing bone marrow aspiration is the key to confirming the treatment plan, which is not only conducive to the control of inflammation as soon as possible, but also can reduce the spread of infection and facilitate the recovery
    of the whole body.

    (3) Early specification of multiple bacterial culture and drug susceptibility is very important, only to determine the type of pathogenic bacteria, choose sensitive antibacterial drugs, treatment in the shortest time to achieve results, play a multiplier effect with half the effort
    .

    (4) Improving resistance and improving the nutritional status of the whole body is of great significance for the control of local and systemic infections, and there must be a big picture view and systematic thinking, and close cooperation between medical and nursing departments is the key
    .

    (5) The establishment of patients' self-confidence is very important, and only with the cooperation of patients and their families can the treatment and nursing measures
    of medical staff be better implemented.

    (6) Attention should be paid to follow-up observation in the later stage, and risk factors and precautions should be promptly informed
    to patients and their families.

    Expert reviews

    Pregnancy complicated by hemophagocytosis syndrome is extremely rare in the clinic, clinicians, especially obstetricians and gynecologists, but the disease progresses rapidly, and if the diagnosis is delayed, it can lead to adverse pregnancy outcomes including fetal death intrauterine and maternal death, so for clinicians, how to find the real cause of the disease from many differential diagnoses is particularly critical
    .

    Through this case, we can learn that if a patient encounters unexplained fever in clinical work, accompanied by rash and decreased blood cells in two or three lines, abnormal liver function, and the effect is still poor after standardized antibiotic treatment, multidisciplinary consultations should be actively held to improve the corresponding examinations, so as to make comprehensive judgments
    .

    References

    1.
    Rousselin A,AlaviZ,LeMoigne E,etal.
    Hemophagocytic syndrome in pregnancy :casereport,diagnosis,treatment,andprognosis[J].
    Clin Case Rep,2017,5(11):1756-1764.

    2.
    Alsina L,ColobranR,deSevilla MF,etal.
    Novel and atypical splicing mutation in a compound heterozygousUNC13D defect presenting in Familial HemophagocyticLymphohistiocytosis triggered by EBV infection[J].
    Clin Immunol, 2014,153(2):292-297.

    3.
    Rajendran A,SherifAA,DivakarA,etal.
    Triple threat :pregnancy,SLE,EBVas potential triggers in secondary hemophagocytic lymphohistiocytosis[J/OL].
    http://dx.
    doi.
    org/10.
    18203/2320-6012.
    ijrms20173603.

    4.
    Jamy O,NunneryS,GiriS,etal.
    Under-recognition of hemophagocytic syndrome in United States'rural,non-teachinghospitals[J].
    Leuk Lymphoma,2016,57(12):2911-2913.

    5.
    RamoscasalsM, Britozern P, Lpezguillermo A, et al.
    Adult haemophagocytic syndrome[J].
    Lancet,2014,383(9927); 1503-1506.

    6.
    TothovaZ, Berliner N.
    Hemophagocytic syndrome and critical illness: Newinsights into diagnosis and management[J].
    Intensive Care Med,2015, 30(7): 401-412.

    7.
    Yip KP , Ali M , Avann F, et al.
    Pregnancy-induced haemophagocyticlymphohistiocytosis[J].
    Intensive Care Soc, 2020, 21(1): 87-91.

    8.
    TothovaZ, Berliner N.
    haemophagocytic syndrome and critical illness:newinsights into diagnosis and management[J] .
    intensice Care Med,2014,30(7):401-402.

    This article is an English version of an article which is originally in the Chinese language on echemi.com and is provided for information purposes only. This website makes no representation or warranty of any kind, either expressed or implied, as to the accuracy, completeness ownership or reliability of the article or any translations thereof. If you have any concerns or complaints relating to the article, please send an email, providing a detailed description of the concern or complaint, to service@echemi.com. A staff member will contact you within 5 working days. Once verified, infringing content will be removed immediately.

    Contact Us

    The source of this page with content of products and services is from Internet, which doesn't represent ECHEMI's opinion. If you have any queries, please write to service@echemi.com. It will be replied within 5 days.

    Moreover, if you find any instances of plagiarism from the page, please send email to service@echemi.com with relevant evidence.