Secondary hemophagocytic lymphohistiocytosis characterized by liver injury

Zeng, Zhan; Li, Minghui; Xie, Yao

doi:10.1016/j.medcle.2020.11.035

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The disease progresses rapidly, the condition is dangerous and the prognosis is poor.<a class="elsevierStyleCrossRefs" href="#bib0030">1–3</a> The clinical manifestations are diversified and non-specific, and it is easy to be misdiagnosed.A married woman, 33 years old, she had taken 7 doses of traditional Chinese medicine from January 4 to January 11, 2017, about one month later, symptoms of asthenia, poor appetite and yellow urine appeared. She was later hospitalized in our hospital. Physical examination: 39.1°C, severe jaundice of bilateral sclera, paler eyelid conjunctiva, obvious tenderness and rebound pain in the whole abdomen, negative Murphy's sign, positive moving dullness, mild pitting edema in both lower limbs. She had a history of hypertension for 2 years, and was treated with captopril.We further performed several biochemical and imaging tests on her. Routine blood tests suggested the presence of anemia and leukopenia; liver function test suggested elevated transaminases, bilirubin, and decreased albumin. The changes in the patient's blood profile and liver function are shown in <a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>. Abdominal ultrasound revealed a large liver, diffuse liver disease, splenomegaly and ascites.<elsevierMultimedia ident="fig0005"></elsevierMultimedia>Considering the clinical manifestations and laboratory results of the patient, we consider that the patient may have acute infectious diseases or hematological diseases. Clinically, if patients have symptoms such as fever, blood cell reduction, hepatosplenomegaly and so on, we should be alert to the possibility of hemophagocytic syndrome.<a class="elsevierStyleCrossRef" href="#bib0035">2</a> We performed a bone marrow biopsy. Bone marrow aspiration showed that the diagnosis was highly suspicious of HLH hemophagocytic syndrome.Reviewing the course of disease development, we sorted out the diagnosis and treatment ideas, looking for the etiology of HLH, thinking as follows: The patient had taken Chinese medicine, a month later began to appear fatigue, anorexia, yellow urine, yellow eyes, dizziness and other symptoms. Chinese medicine may be the cause of HLH or the incentive to aggravate HLH. While the patient signed on admission on March 14, the peritoneal irritation was positive, and there was still abdominal infection, which may be the factor inducing or aggravating HLH.This is a patient whose liver function is seriously damaged for unknown reasons, young female, acute onset, rapid progress, history of taking Chinese medicine 1 month before the onset, the course already lasting a month, with progressive fatigue, anorexia, yellow urine, severe damage to liver function, and in the late course of high fever. Under thorough examination and symptomatic liver protection, anti-infection, supportive treatment, has excluded A, B, D and E viral hepatitis, EBV and CMV hepatitis, has excluded drug, alcoholic, fatty, autoimmune, hereditary hepatitis. The patient was diagnosed as HLH with fever, splenomegaly, peripheral blood cytopenia and abnormal increase of ferritin, combined with bone puncture pathological examination, and without excluding pure red cell aplasia. The patient's condition progresses rapidly, and the cause of HLH is unknown, which may be related to traditional Chinese medicine and abdominal infection. Our treatment continued, and she died two days after the diagnosis. The diagnosis and treatment of this patient provides us with ideas to deal with similar problems in clinical practice.In this case, the patient had an acute onset and rapid progression of the disease, and came to our hospital late, which only gave us two days for treatment, although the symptomatic support therapy such as anti-infection, hepatoprotective, and blood transfusion was given, but the results were not satisfactory. The patient's clinical manifestation of predominantly hepatic impairment increased the difficulty of diagnosis, which in turn led to a delay in treatment, while early recognition of HLH and timely treatment is the key to improving prognosis.<a class="elsevierStyleCrossRefs" href="#bib0045">4,5</a> From this case, we should draw conclusions. Firstly, took as little as possible Chinese medicine with unknown composition. Secondly, early use of powerful antibiotics to control abdominal infection. Thirdly, for patients with liver injury as the main manifestation, the thinking of diagnosis and treatment should be broadened, but not limited to liver disease. In addition to liver injury, when facing patients with fever, splenomegaly and other manifestations, we should consider other system diseases, especially blood system diseases." 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Cron" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s40272-019-00367-1" "Revista" => array:6 [ "tituloSerie" => "Paediatr Drugs" "fecha" => "2020" "volumen" => "22" "paginaInicial" => "29" "paginaFinal" => "44" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/31732958" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0035" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Hemophagocytic lymphohistiocytosis in adults" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "N.S. Bhatt" 1 => "B. Oshrine" 2 => "J. 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Berliner" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1182/blood-2015-01-551622" "Revista" => array:6 [ "tituloSerie" => "Blood" "fecha" => "2015" "volumen" => "125" "paginaInicial" => "2908" "paginaFinal" => "2914" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/25758828" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0050" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Hemophagocytic lymphohistiocytosis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "J. Skinner" 1 => "B. Yankey" 2 => "B.K. Shelton" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.4037/aacnacc2019463" "Revista" => array:6 [ "tituloSerie" => "AACN Advanced Critical Care" "fecha" => "2019" "volumen" => "30" "paginaInicial" => "151" "paginaFinal" => "164" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/31151946" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] "agradecimientos" => array:1 [ 0 => array:4 [ "identificador" => "xack571967" "titulo" => "Acknowledgment" "texto" => "This study was funded in part by the National Science and Technology Major Project of China (2017ZX10201201-001-006 and 2017ZX10201201-002-006) and the Digestive Medical Coordinated Development Center of Beijing Hospitals Authority (XXZ0302)." 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Vol. 157. Issue 11.