1 case of combined heptopha cytopha syndrome during pregnancy.
Last Update: 2020-08-01
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. Phagocytophatic lymphatic tissue hyperplasia, also known as phage cell syndrome, is caused by out-of-control immune response after overactivation of the disease, the causes are complex and diverse, clinically relatively rare, high fatality rate? This case report analyzed 1 case of phagocytosis-induced phagocytosis syndrome to improve awareness and understanding of the disease?
1 Clinical cases
case characteristics Patients, female, 31 years old, 20 weeks pregnant, G3P1, medium pregnancy? On April 1, 2018, there was no obvious cause of fever, fluctuating at 38.0 to 40.0 degrees C, accompanied by the fear of cold? Cough? To the local hospital, blood routine show three series of mild reduction, C-reactive protein increase, alanine amino metastase (ALT) significantly increased, bilirubin mild lysin increase, clear protein (ALB) 27g/L, urine routine white blood cells 1 plus, no abnormality? On the 13th of the month transferred to the hospital's infection department, check blood routine show three-line reduction, bilirubin increase, liver enzyme increase, plasma clotting enzyme progenitor time (PT) and activation part of the clotting enzyme time (APTT) extended, D-dipolymer significantly increased, ferritin? Lactate dehydrogenase (LDH)? For a clear diagnosis, further bone-piercing suggests that nuclear cells are active, and that blood-eating cells are 1% to 2%, and that bone marrow flow is not visible with obvious abnormal phenotype cells, and is initially diagnosed as phage cell syndrome? Low proteinemia? Dispersive intravascular clotting (DIC) to be discharged? Moderate anemia? Liver damage, etc. After admission to the hospital to give mena (complex licolic glycolic poop tablets)? Smetai (injection of adenosine methionine) liver protection, shup deep (injection of cephalosporine ketone sodium) snr. Love anti-viral, Oak gastric protection, while supplementing human blood ALB? plasma, etc., from April 18, 2018 to give human immunoglobulin 20g / L - Astrong dragon 60 to 80 mg control inflammation treatment, the patient's body temperature returned to normal?
hospitalization white blood cells 4.2 x 109/L, red blood cells 3.22 x 1012/L, hemoglobin 99g/L, platelets 78 x 109/L, hypersensitivity C reactive protein 18.28mg /L?Blood Biochemical Tips Total Bilirubin (TBIL) 57.6 smol/L, Direct bilirubin (DBIL) 34.1 ?mol/L, indirect bilirubin (IBIL) 23.5 smol/L, ALT 3 73U/L, Tianmen domide amino transferase (AST) 880U/L, glutamate transfer enzyme (GGT) 145U/L, LDH 1 052U/L, ALB 21g/L, bile Acid 70.4 smol/L, glycolic acid 29.25mg/L, alkaline phosphatase 306U/L, calcium 1.79mmol/L, phosphorus 0.84mmol/L? Blood clotting function: PT 18.9s, APTT 45.4s, D-dipolymer s.20 000?g/mL, fibrin degradation product 68.5mg/L?A-fetal protein 120 .70 sg/L, cancer antigen 125 (CA125) 101.30U/mL, ferritin.2 000?g/L, vitamin B121 476pmol/L , calcitonin progeny 20.10 ng/mL?lipids: triamide 3.32mmol/L, total cholesterol 2.22mmol/L? bone marrow elephant (April 16, 2018): bone marrow has nuclear cell growth activity, granulation ratio 1.5:1; granular hyperplout is active, nuclear mild left shift, red line increase is significantly active, dividing phase, with late-to-late-growth, dominant, dominant, dominant, dominant in the form? The proportion did not see obvious abnormality; the megagen proliferation, platelets scattered in clusters visible, smears to see the phage cells 1% to 2%? Peripheral blood visible rod-like nuclear cells slightly higher proportion? Blood clotting function (April 19, 2018): 3P test positive, D-dipolymer. 20 000 ?g/L? Bone marrow elephant (April 20, 2018): the bone marrow has nuclear cell hyperplasia, granuloma ratio 1.0:1, the tablet can be seen large, shape-like lymphoma abnormal cells, see Figure 1A, nuclear malformation is obvious, nuclear renition is large and obvious, cytoplasm deep infection, there is a trailing phenomenon? Grain system? The red system proliferates, the morphological proportion did not see significant abnormality? Giant growth, platelets scattered in small clusters rare? A large number of phage cells (3% to 5%) can be seen in the picture, see Figure 1B
? Treatment process April 20, 2018, after the hospital consultation and Shanghai high-risk maternity center consultation opinion, pay close attention to the hematological-related changes, B ultra-clear fetal condition, first consider the treatment of phage syndrome, and then consider whether to continue pregnancy or induced delivery? Further peripheral blood search for broken red blood cells? Net-woven red blood cells? CD25R, PET-CT definitive cause? Closely monitor blood routine, DIC, liver function, blood culture, pharynx swab culture, continue propylene globulin? Dexamethasone 10mg/m2 d), Vitamin K1, Plasma Therapy, Liver Protection? Anti-infective treatment, etc. April 20, 2018, recommended that patients review bone wear to clearly diagnose, bone wear see a large number of blood-eating cells, see lymphocyoma cells, cells larger, abnormally obvious; chest tightness; B super show liver and spleen is not big, liver dysfunction (Child-C grade), review blood clotting show further extension of PT and APTT, fibrin raw still decline, continue to infusion plasma, supplement human fibrin correction DIC, and follow lymphoma chemotherapy, first add VP-16? dissemine 10mg/ (m2?d)? Blood clotting? Blood routine no significant increase, expected to gradually increase CTX? VCR, etc.? Again inform the patient's family of the condition, follow-up treatment requires the use of a large number of chemotherapy toxic drugs, the family and patients expressed understanding, agree to the current treatment, and said that if necessary, according to the patient's condition to terminate the pregnancy? April 21-23, 2018 continue the treatment of the semisepine and VP-16 program, monitoring blood routine? Blood biochemistry? Blood clotting? Liver enzymes? LDH significantly lower than before, normal body temperature? Pt and APTT carrying time is shorter, and fibrinogen has rebounded from the previous one? ALB 19g/L, hemoglobin 62g/L? But fibrin is still low, poor clotting function, give serum protein to correct hypoproteinemia, infusion plasma to correct clotting function? April 23, 2018, 10:00 pm sudden consciousness disorder, irritability and drowsiness alternate, complain headache? Blurred vision? Not the answer? Unretarmof light reflection, eyes staring to the left, sdurashima and yellow ingress of the whole body? Cervical resistance (-, involuntary mobility of limbs), mild increase in muscle tone in the limbs, pathological signs of bipedal foot (plus), blood pressure 130/96mm Hg (1mm Hg-0.133kPa), heart rate 120 times/min? No contractions? No vaginal bleeding? Good heart? Urgently consult neurology? Gynecologic? Emergency department? Digestive consultation? Emergency blood routine 2.0 x 109/L, hemoglobin 74g/L, platelet count 36 x 109/L; Blood Biochemical TBIL 166.9 smol/L, DBL 80.5 mol/L, ALB 23g/L, ALT 199U/L, AST214U/L, GGT 7U9/L Glucose 5.0mmol/L, potassium 3.28mmol/L, calcium 1.85mmol/L; clotting function shows PT 20.8s, APTT 47.1s, fibrinogen 1.86g/L, D-dipolymer 36 840 ?g/L, hemolysmic 103 smol/L? Consider the possibility of hepatic encephalopathy leading to metabolic encephalopathy, while acute cerebral ischemia? Transient cerebral ischemic episodes? Lymphoma and central conditions are no exception? Treatment to give glycol dehydration treatment, supplement alB diuretic reduction cranial pressure? Abos? Arginine improves liver encephalopathy? Potassium supplementation? Low-molecule heparin subcutaneous injection and other treatment? Can the patient fall asleep intermittently at night, but recur irritability? On April 24, 2018, the patient was still confused, emotionally more stable than last night, sensitive to light reflection, sleaze and whole body skin yellow dye, limb involuntary activity, slightly increased muscle tone of the limbs, double-sided Babinski positive, no nausea? Vomiting? Treatment continues to glycol dehydration to supplement ALB diuretic reduction cranial pressure? Abos? Arginine improves liver encephalopathy? Potassium supplementation? Low-molecular heparin subcutaneous injection and other treatment, and suspended chemotherapy, patients still repeated irritability? Symptoms such as delirium? The patient's family strongly requested to go back to the local hospital, inform the patient's family of the condition and related risks, sign out of the hospital, to the local hospital?
Figure 1 Bone marrow elephant staining results (x 100)
primary haemophile syndrome is a rare disease, is an autosomal recessive genetic disease, mainly found in infants and young children, 90% of children under 2 years of age? Secondary hemophilic cell syndrome is common in adults, by viruses? Bacteria? Fungi? Infections such as protozoa? Tumor? Autoimmune diseases? A reactive disease caused by a variety of pathogenic factors, such as drugs, initiates the activation mechanism of the immune system. Increased liver enzyme? PT and APTT extension, D-dipolymer significantly increased? Ferrite? LDH?triceratlygine is significantly elevated, the bone marrow has macrophages and other manifestations, patients with blood cell syndrome diagnosis is clear, but sustained high fever for more than 2 weeks, the cause is still unclear, infection has not been ruled out? Are there the following possibilities for primary disease? (1) Infection-related: the patient complains of fever before there is a fear of cold, there is cough, calcitonin primary 28.18 ng/mL, blood culture negative, red blood cell deposition rate is normal, endotoxin? Fungal D-glucan normal, A? B? C? Ding? Hepatitis E is negative, EB virus negative, anti-cytomegalovirus IgG positive, HSVI type IgG positive, cytomegavirus DNA negative? B overshow liver and spleen no swelling, deny the history of contact with epidemic water? (2) Malignant tumor: the patient's first bone wear? The flow did not detect the relevant blood system disease basis, the second bone through a large number of macrophages and lymphoma cells, can review PET-CT?related fusion gene? Genetic mutation? IgH? T-cell rearrangement and other clear causes? (3) Autoimmune Diseases: Patients are middle-aged women, denied that there is a history of related diseases, denied the history of pain, infection department during the examination of autoimmune anomalies, do not rule out pregnancy period? Is it possible for drugs such as induced phage cell syndrome? This case of patients for pregnancy 20 weeks fever 2 weeks of illness, the rapid development of the disease, infection? The evidence of autoimmune disease is insufficient, there is a clear reduction of peripheral blood three lines, abnormal liver function? Blood clotting disorder? High-iron protein? High triamcinolone? High lactic acid dehydrogenase and low fibrinogen performance, and according to the results of bone marrow smear splicing confirmed as phage cell syndrome combined lymphoma, using VP-16 plus disemisone program for treatment, but the patient's liver damage is serious, the emergence of metabolic encephalopathy, also does not exclude lymphoma tired resulting in mental symptoms, and finally suspended chemotherapy and according to the requirements of the patient transferred to a local hospital for treatment? Is phage cell syndrome a kind in the bone marrow? Spleen? Benign in hematopoietic tissue such as lymph nodes? Reactive hyperplasia of tissue cell disease, accompanied by an active phagocytopharycal phenomenon? Mainly NK cells and cytotoxic T lymphocyte function or lack of activity lead to tissue cell division and proliferation of cytokines disorders, histology is seen in the mesh endothelial cell system mixed lymphocyte cell accumulation, mainly manifested in T-cell functional defects, T-cells and mononucleocys activity enhancement? Reduced or lack of hypercytokinemia and selective cytotoxic activity? NK cell energy loss, CD3? CD4 cell reduction, CD8 increase? T-cell dysfunction stimulates macrophages to secrete a large number of cytokines, cell immune function is disordered, thus damaging organ tissue, the development of phage cell syndrome is a dynamic process, the early bone marrow like only a small number of blood-eating cells, inner phytocells and/or red blood cells and/or platelet cells, very easily missing, therefore, suspected that hepophycell syndrome often needs multiple parts of the bone-eating, and requires a serious study of the cell form of the body of patients? Does the bone marrow find the full form of phagocytosis with nuclear cells? Erythrocyte? Platelet phage cells can be used as an important basis for early diagnosis. Spleen? Lymph nodes are swollen, peripheral blood systems or three lines are reduced, liver function is abnormal? Blood clotting disorder? High-iron protein? High triamcinolone? High lactic acid dehydrogenase and low fibrinogen, manifested as lipid metabolism? Ferrinin metabolism and coagulation dysfunction? Hepatitis C virus? A megacell virus? Bacteria? Systemic lupus? Bone marrow hyperplasia syndrome? Tumors, among them infections, EB virus and cytomegalovirus infection for the common, early diagnosis and give the corresponding treatment is very critical? High iron iron iron protein is due to macrophage phagocytosis and digestion of a large number of red blood cells and decomposition to produce a large number of hemoglobin, hemoglobin is further broken down into iron and globulin, resulting in ferritin increases, tricerbriyrate is likely to be macrophage and digestion of white blood cell decomposition release a large number of tricergang? In addition to liver damage, fibrinogen reduction is also associated with macrophage secretion that causes serum fibrosis to activate and break down fibrinogen in the blood? High LDH is related to systemic organ tissue damage. At present, the recognized diagnostic criteria for phage cell syndrome were revised by the International Association of Cells in 2004 and can be diagnosed when any of the following criteria are met. (1) Molecular diagnosis conforms to heptopha syndrome: the relevant disease-causing genes known to be phagocytosis syndrome, such as PRF1? UNC13D? STX11? STXBP2? Rab27a? LYST? SH2D1A? BIRC4? ITK? AP3 beta1? MAGT1? Cd27 and other pathological mutations? (2) 5 of the following 8 indicators: (1) fever, body temperature greater than 38.5 degrees C, continuous spleen, (2) large spleen; (3) reduction of blood cells (tired and peripheral blood two or three), hemoglobin 90g/L, platelets 100 x 10 9/L, neutrophils 1.0 x 109/L and not a reduction in bone marrow hematopoietic function; (4) high trialycerin or low fibrinemia, triamide glycerin 3mmol/L or 3 standard deviations higher than peers, fibrillingens 1.5g/L or less.
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