Paroxysmal nocturnal hemoglobinuria (PNH) is a non-malignant clonal disease caused by mutations in 1 or several hematopoietic stem cells via the acquired somatic PIG-A gene, clinically manifested by chronic intravascular hemolysis, hematopoietic failure and recurrent thrombosis. Chen Pingbo, Department of Orthopedics, Xinjiang Uygur Autonomous Region Hospital of Traditional Chinese Medicine 1. Pathogenesis: Regarding the pathogenesis of PNH, the dual pathogenesis theory proposed by Dacie is a commonly recognized and accepted hypothesis. First, hematopoietic stem cells mutate under certain conditions to produce GPI-deficient PNH clones; second, due to certain factors (now mostly considered as immune factors), hematopoietic damage or hematopoietic failure occurs and PNH clones gain proliferative advantage over normal clones. Initially, CD55 was thought to have an important role in erythrocyte hemolysis in PNH and was used to explain the sensitivity of erythrocytes to complement in PNH. However, CD55 deficiency alone does not lead to hemolysis, which was confirmed in patients with congenital CD55 deficiency.1 A case of congenital CD59 deficiency was identified in 1990, which exhibited numerous typical manifestations of PNH, such as intravascular hemolysis, hemoglobinuria and venous thrombosis, and therefore, it is now believed that the typical manifestations of PNH – intravascular hemolysis and thrombosis – are due to CD59 deficiency 2. Diagnosis: Flow cytometry diagnosis Flow cytometry is the gold standard for the diagnosis of PNH and allows quantitative analysis of PNH blood cells. The most commonly used are anti-CD55 and CD59 antibodies, which can bind specifically to CD55 and CD59 on the cell surface when added to flow cytometry. The cells that are not bound by CD55 and CD59 are the PNH cells. The number of CD55ˉ and CD59ˉ cells can be determined by colorimetric analysis.3. Treatment Conventional treatment is mainly to control hemolytic episodes, such as immunosuppressants like dextran, sodium bicarbonate, adrenocorticosteroids, and androgens to stimulate hematopoiesis. Long-term hemoglobinuria can lead to iron deficiency. Iron can cause the production of reactive oxygen species, and PNH cells are sensitive to oxidative damage, which can easily induce hemoglobinuria. After affirmation of iron deficiency, treatment should be started at a small dose, 1/3 to 1/10 of the conventional dose, and should be discontinued if there is still a reaction. In addition to raising hemoglobin and maintaining tissue oxygen demand, blood transfusion can also inhibit erythropoiesis and indirectly reduce complement-sensitive erythrocytes. Anticoagulation Venous thrombosis is the most important complication of PNH, mainly in the vena cava and cranial veins, about 50% of PNH patients have had venous thrombosis, and about 1/3 died due to thrombosis. The patient was admitted to the hospital on August 4, 2014 with the chief complaint of “right hip pain with limitation of movement for 1 year”. Physical examination: T:36.5 degrees, P:80 beats/min, R:20 beats/min, BP:130/60 mmHg; specialized examination: mild swelling of the right hip joint, limited movement (70 degrees of flexion, 10 degrees of posterior extension, 10 degrees of abduction, 20 degrees of internal retraction, 20 degrees of internal rotation, 20 degrees of external rotation), positive percussion pain in the longitudinal axis, positive right four-letter test, relative length shortening of the affected limb compared with the healthy side about 100 px. The muscles of the affected limb were mildly atrophied, muscle strength was grade 3, and the peripheral sensory blood flow of the affected limb was normal. The patient had suffered from paroxysmal sleep hemoglobinuria in the past, so the preliminary diagnosis was: old fracture and dislocation of the right hip joint and paroxysmal sleep hemoglobinuria. Laboratory examination: According to the patient’s medical history, symptoms and signs, in order to further clarify the diagnosis, we improved the relevant examinations and checked: complete blood cell analysis + reticulocyte count (erythrocyte pressure volume 0.16L/L, hemoglobin 49.00g/L, absolute value of lymphocytes 0.73*10^9/L, mean erythrocyte hemoglobin volume 37.40pg, mean erythrocyte hemoglobin concentration 299.00g/L, mean erythrocyte volume 125.20fL, percentage of monocytes 11.70%, absolute neutrophil value 1.22*10^9/L, platelet count 114.00*10^9/L, erythrocytes 1.31*10^12/L, erythrocyte distribution width (CV) 20.80%, erythrocyte distribution width (SD) 93.60 (fL, leukocytes 2.30*10^9/L) macrobiochemistry (total bilirubin 52.70umol/L, aspartate aminotransferase 147.40U/L, total protein 64.60g/L, globulin 19.66g/L, direct bilirubin 7.00umol/L, indirect bilirubin 45.70umol/L, other indicators were not significantly abnormal), coagulation (APTTR value 1.32, D-dimer 2.50ug/ML), acid hemolysis test (-), blood smear (-), urine ferric hemoglobin qualitative test (+), hemolysis IV (-). The blood-related tests showed abnormalities, so we asked the hematology department to consult and recommend checking CD55 and CD59, and the test results showed that CD55: 68% and CD59: 42.6%. After transferring to the hematology department, the patient was given bilirubin reduction, hepatoprotection, acid suppression and gastric protection, methylprednisolone immunosuppressive therapy, and was given washed red blood cells infusion (3 times totaling 6U) for supportive treatment. The following formula is shown: Formula 1: 60g Radix Astragali, 9g Radix Angelicae Sinensis, 20g Radix Codonopsis Pilosulae, 12g Atractylodes Macrocephala, 9g Poria, 9g Glycyrrhiza Glabra, 9g Radix Paeoniae Alba, 9g Rhizoma Ligustici Chuanxiong, 9g Salviae Miltiorrhizae, 30g Radix et Rhizoma Inulae, 30g Coix Coix Seeds, 15g Radix Coix Coix Seeds, 9g Radix Rehmanniae, 7 doses, 1 dose daily. 15g Wine cornelian 12g Salt bone fructus 10g Senghu Cao 20g White flowered snake’s tongue 30g Roasted licorice 9g 6 doses 1 dose daily Decoction taken orally twice daily. After treatment, the patient’s bilirubin (total bilirubin 16.90umol/L, unconjugated bilirubin 13.50umol/L, conjugated bilirubin 0.50umol/L) and reticulocyte (reticulocyte percentage 5.43%, reticulocyte absolute value 0.15*10^12/L) levels decreased significantly, and hemoglobin increased significantly (red blood cells 2.68*10^12/L, hemoglobin 88*10^12/L). 12/L, hemoglobin 88.00 g/L,). He was transferred to our department on August 27, 2014. The patient was transferred to our department and underwent “right hip total hip arthroplasty with old fracture and dislocation” under general anesthesia on August 29, 2014, the whole operation went smoothly and the anesthesia was satisfactory, the intraoperative diagnosis was the same as before the operation, and she was admitted to ICU after the operation. Specialist examination: postoperative wound was good, dry and clean without blood ooze, no redness, swelling and heat in the periphery, and sensory blood flow at the toe end was possible. Because of the patient’s poor basic condition, he was given antibiotics to prevent infection, low molecular heparin to prevent thrombosis, famotidine to suppress acid and protect the stomach, and aminoglutethimide to reduce sputum, and rehydration for symptomatic treatment in the postoperative ICU ward. Postoperatively, 1.5 U of O-type suspended red blood cells were transfused for hemoglobin supplementation, and 250 ml of O-type fresh frozen plasma and 270 ml of O-type de-virulent fresh frozen plasma were transfused for coagulation factor supplementation. After the operation, we observed the peripheral blood flow and arterial pulsation of the affected limb, administered antibiotics to prevent postoperative infection, prevented thrombosis with low-molecular heparin, treated the patient with postoperative rehydration and pneumatic therapy to reduce swelling, took X-rays of the affected limb, and understood the patient’s condition. The wound healed well. The patient was also given oral Chinese medicine, and the following prescriptions were given: Free decoction of Sheng Di Huang (10g/bag) 1 bag Free decoction of Sheng Ma (6g/bag) 1 bag Free decoction of Bai Shen (10g/bag) 1 bag Free decoction of Gardenia (10/bag) 1 bag Free decoction of Huang Lian (3g/bag) 1 bag Free decoction of Guan Huang Bai (6g/bag) 1 bag Free decoction of Angelica Sinensis (10g/bag) 1 bag Free decoction of Red Peony (10g/bag) 1 bag Free decoction of Radix Rehmanniae (6g/bag) 1 bag Free decoction of Rhizoma Rheum (3g/bag) 1 bag Free decoction of Rhizoma Rheum ( 3g/bag) 1 bag Free decoction of licorice (3g/bag) 1 bag Free decoction of honeysuckle (10g/bag) 1 bag Free decoction of stone cassia (30g/bag) 1 bag Free decoction of chicken blood vine (15g/bag) 1 bag Free decoction of astragalus (10g/bag) 1 bag 7 doses 1 dose daily Water for oral intake twice a day. Before discharge, the complete blood cell analysis was checked (erythrocytes 2.23*10^12/L, hemoglobin 76.00g/L), which was significantly better than before admission. On September 10, 2014, the patient was discharged from the hospital with good wound pairing, dry and clean without blood oozing, no peripheral redness, swelling and heat, normal sensory blood flow at the toe end, and the mobility of the right hip joint (flexion, posterior extension, abduction, adduction, internal rotation, external rotation) was significantly improved. Due to the short postoperative period, full recovery to normal mobility requires active functional exercise after discharge. 1. advise the patient not to flex and extend the hip joint >90°, not to sit on a low stool, not to squat, not to over-rotate or externally rotate the lower limb, and not to sleep on the side. 2. avoid the following actions: crossing the legs; sitting on a low toilet or a short sofa when going to the toilet.