Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder. Its occurrence secondary to hepatobiliary malignancies is even rarer, and without timely diagnosis and treatment, the mortality rate is extremely high. There is a need to raise awareness of this disease. This report describes a case of a 70-year-old female patient diagnosed with AHA 2 months after surgery for cholangiocarcinoma, admitted to the Second Affiliated Hospital of Bengbu Medical College in October 2022. The patient presented with subcutaneous hematoma in both lower limbs. Coagulation function tests showed a markedly prolonged activated partial thromboplastin time (APTT) of 74.5 seconds, with no correction in the APTT mixing test. Coagulation factor assays revealed a severely reduced coagulation factor VIII activity (FVIII:C) of 0.3%, and an inhibitor titer of 25.6 BU/mL was detected. After ruling out other potential causes, the patient was diagnosed with cholangiocarcinoma-associated AHA. With chemotherapy to control the primary tumor, alongside hemostatic and immunosuppressive therapy for inhibitor eradication, AHA was brought under control. The patient had no further coagulation abnormalities or bleeding, enabling timely and full-course chemotherapy for cholangiocarcinoma and significantly improving survival and quality of life. Therefore, in patients with malignancies who present with spontaneous bleeding or unusual bleeding following surgery, trauma, or invasive procedures, clinicians should be alert to the possibility of secondary AHA. Timely diagnosis and treatment can significantly improve prognosis.
Humans ; Cholangiocarcinoma/surgery* ; Female ; Hemophilia A/drug therapy* ; Aged ; Bile Duct Neoplasms/surgery* ; Factor VIII

BACKGROUND: Total knee arthroplasty (TKA) can reduce severe joint pain and improve functional disability in hemophilia. However, the long-term outcomes have rarely been reported in China. Therefore, this study aimed to evaluate the long-term outcomes and complications of TKA in Chinese patients with hemophilic arthropathy. METHODS: We retrospectively reviewed patients with hemophilia who underwent TKA between 2003 and 2020, with at least 10 years of follow-up. The clinical results, patellar scores, patients' overall satisfaction ratings, and radiological findings were evaluated. Revision surgery for implants during the follow-up period was recorded. RESULTS: Twenty-six patients with 36 TKAs were successfully followed up for an average of 12.4 years. Their Hospital for Special Surgery Knee Score improved from an average of 45.8 to 85.9. The average flexion contracture statistically significantly decreased from 18.1° to 4.2°. The range of motion (ROM) improved from 60.6° to 84.8°. All the patients accepted patelloplasty, and the patients' patellar score improved from 7.8 preoperatively to 24.9 at the last follow-up. There was no statistically significant difference in clinical outcomes between the unilateral and bilateral procedures, except for a better ROM at follow-up in the unilateral group. Mild and enduring anterior knee pain was reported in seven knees (19%). The annual bleeding event was 2.7 times/year at the last follow-up. A total of 25 patients with 35 TKAs were satisfied with the procedure (97%). Revision surgery was performed in seven knees, with 10- and 15-year prosthesis survival rates of 85.8% and 75.7%, respectively. CONCLUSIONS TKA is an effective procedure for patients with end-stage hemophilic arthropathy, which relieves pain, improves knee functions, decreases flexion contracture, and provides a high rate of satisfaction after more than ten years of follow-up.
Humans ; Arthroplasty, Replacement, Knee/methods* ; Hemophilia A/surgery* ; Follow-Up Studies ; Retrospective Studies ; Treatment Outcome ; Knee Joint/surgery* ; Range of Motion, Articular ; Arthritis/complications* ; Pain ; Contracture/surgery* ; Surgeons ; Knee Prosthesis

Humans ; Ankle ; Ankle Joint/surgery* ; Arthritis ; Arthrodesis ; Lower Extremity ; Hemophilia A/complications*

On December 22, 2017, a 35-year-old male hemophilia A patient with a secondary chronic refractory wound after left knee joint surgery was transferred from the Department of Hematology of Maoming People's Hospital to the Department of Burns and Plastic Surgery in the same hospital. The physical examination revealed that the patient's left knee joint was swollen, with a full-thickness skin defect wound of 4 cm×4 cm on the lateral side of the joint and a large number of dark red blood clots at the bottom of the wound. The wound bleeding was controlled by intravenous infusion of plasma, cryoprecipitate, and human coagulation factor Ⅷ. After con- ventional debridement and dressing changes until the wound infection was controlled and necrotic tissue was removed, a subcutaneous cavity wound of 2 cm×2 cm in area and 3 cm in depth remained in the left knee joint and was difficult to heal. Nineteen days after transfer, the patient received autologous platelet-rich plasma (PRP) treatment, and 32 days after PRP treatment, the wound in left knee joint was healed with epithelialization. This case suggests that autologous PRP therapy would be a good option for hemophilia complicated chronic refractory wounds when they could not be repaired by surgery.
Adult ; Hemophilia A/therapy* ; Humans ; Knee Joint/surgery* ; Male ; Platelet-Rich Plasma ; Skin Transplantation ; Soft Tissue Injuries/surgery* ; Treatment Outcome ; Wound Healing

OBJECTIVETo observe the clinical significance of postoperative personalized antithrombotic therapy for patients with hemophilic arthritis (HA) patients after arthroplasty.

METHODSFrom September 2005 to October 2013, 11 cases of arthroplasty for hemophilic arthritis in hip and knee total operation 14 times,including 1 case of double knees (calculated as one operation), operation in left knees 6 times, operation in right knees 5 times, 2 in hip. All the patients were male and the age ranged from 23 to 57 years old,with an average of (36.1 ± 11.0) years old; the average weight was (64.1 ± 8.9) kg. All the patients were preoperatively diagnosed and classified as hemophilic arthritis with the radiological images and laboratory tests. According to the function of joints, the risk of postoperative venous thromboembolism (VTE), and dynamic observation of Factor VIII:C (FVIII:C) activity, patients were treated with personalized antithrombus by adjusting the dosage of recombinant human coagulation factor VIII (Kogenate FS). All the patients were orderly divided into postoperatively distal joints moving group and none-moving group to observe the coagulation function.

RESULTSThe enrolled patients had no postoperative complication of VTE and pulmonary embolism (PE). The APTT and D-2 were different between two groups in the postoperative early stage. Length of hospital day was shorter in the moving group than none-moving group.

CONCLUSIONBecause of the self-coagulation disorder, patients with HA tended to bleed. However it doesn't mean that there is no risk of postoperative thrombosis. Therefore,it's important to determine how to control the balance between postoperative antithrombus, hemostasis,and coagulation factor replacement therapy after arthroplasty for HA. Postoperative moving has proved helpful for HA, especially in reducing the risk of hemostasis and shortening the time in hospital.

Adult ; Arthritis ; surgery ; Arthroplasty ; adverse effects ; Factor XIII ; metabolism ; Hemophilia A ; complications ; Hemostasis ; Humans ; Male ; Middle Aged ; Postoperative Complications ; prevention & control ; Thrombosis ; prevention & control ; Young Adult

Aneurysm, False ; surgery ; Factor VIII ; therapeutic use ; Hemophilia A ; drug therapy ; Humans ; Uremia

BACKGROUND: Hemophiliacs have extrinsic tightness from quadriceps and flexion contractures. We sought to examine the effect of a focused physical therapy regimen geared to hemophilic total knee arthroplasty. METHODS: Twenty-four knees undergoing intensive hemophiliac-specific physical therapy after total knee arthroplasty, at an average age of 46 years, were followed to an average 50 months. RESULTS: For all patients, flexion contracture improved from -10.5 degrees preoperatively to -5.1 degrees at final follow-up (p = 0.02). Knees with preoperative flexion less than 90 degrees were compared to knees with preoperative flexion greater than 90 degrees. Patients with preoperative flexion less than 90 degrees experienced improved flexion (p = 0.02), along with improved arc range of motion (ROM) and decreased flexion contracture. For those patients with specific twelve-month and final follow-up data points, there was a significant gain in flexion between twelve months and final follow-up (p = 0.02). CONCLUSIONS: Hemophiliacs with the poorest flexion benefited most from focused quadriceps stretching to a more functional length, with gains not usually seen in the osteoarthritic population. This data may challenge traditional views that ROM gains are not expected beyond 12-18 months.
Adult ; Aged ; Arthroplasty, Replacement, Knee/*methods ; Female ; Follow-Up Studies ; Hemophilia A/complications/*physiopathology/*therapy ; Humans ; Joint Diseases/*blood/complications/surgery ; Knee Joint/*physiopathology/surgery ; Male ; Middle Aged ; *Physical Therapy Modalities ; Range of Motion, Articular/physiology ; Retrospective Studies ; Treatment Outcome

OBJECTIVETo evaluate the amount of blood loss and the efficacy of clotting factor in controlling blood loss during total knee arthroplasty.

METHODSThe medical documents of 18 patients with haemophilic arthritis (HA) secondary to haemophilia A and 19 patients with osteoarthritis (OA) were retrospectively reviewed. Demographic data,functional and hematological test results,the amount of blood loss and transfusion,and complications were analyzed.

RESULTSThe median amounts of total and external blood loss were 2240 ml(1892-3415 ml) and 1326 ml(934-2256 ml)in the HA group, which were significant higher than those in the OA group [1746 ml(1259-2246 ml)and 846 ml (504-1217 ml), respectively]. The median amounts of external blood loss in the two groups were 680 ml(370-1330 ml)and 730 ml(200-1190 ml)and there was no significant difference(p=0.620). Moreover, more patients in the HA group required blood transfusion (84.2% vs. 47.4%), and more red cells were transfused per patient in the HA group (2.3 U vs. 0 U).

CONCLUSIONSThe total blood loss and hidden blood loss are higher in the HA patients than in OA patients during total knee arthroplasty, although the external blood loss is basically the same. Management with more clotting factor may decrease the blood loss in HA patients.

Adolescent ; Adult ; Arthritis ; etiology ; surgery ; Arthroplasty, Replacement, Knee ; Hemophilia A ; complications ; Humans ; Male ; Middle Aged ; Osteoarthritis, Knee ; surgery ; Postoperative Hemorrhage ; Retrospective Studies ; Young Adult

OBJECTIVETo study the efficacy of total knee anhmplasty (TKA) for the treatment of hemophilic knee arthropathy, and to explore the operative characteristics, the selection of prothesis, the effectiveness and safety of clotting factor replacement treatment.

METHODSFrom January 2008 to June 2010, 10 patients (12 knees)with hemophilic anhropathv underwent TKA. The average age was 33.6 years old (ranged, 17 to 49 years). There was 8 cases of type A hemophilia and 2 cases of type B hemophilia. According to Arnold and Hilgartner classification: 7 knees were IV degree and 5 knees were V degree. The level of VIII factor for replacement treatment was more than 80% on operation day, more than 60% within 3 days after operation, more than 40% from the third day to the second week after operation. Added prothrombin complex concentrate (PCC) to improve the level of IX factor, and the level of IX factor for replacement treatment was more than 40% on operation day, more than 30% within 3 days after operation, more than 20% from the third day to the second week after operation. Functional training was mainly based on continuous passive motion (CPM) device after surgery. Clinical assessment included hospital for special surgery knee score (HSS) and the individual scores (including pain, function, activity, muscle strength, flexion deformity and stability).

RESULTSTen patients (12 knees) were followed-up, and the average duration was 11 months (ranged, 6 to 24 months). The average preoperative HSS score was (44.9 +/- 12.5) (ranged, 29 to 62 scores), whereas the average postoperative HSS score was (84.4 +/- 10.2) (ranged 72 to 96 scores) (P < 0.01). The preoperative individual score including pain, function, activity, muscle strength flexion deformity and stability were significantly improved compared with pre-operation, the differences between them were statistically significant (P < 0.01 ). TKA had the distinct role in relieving pain from preoperative (8.5 +/- 4.1) to postoperative (24.5 +/- 4.4).

CONCLUSIONUnder the strict perioperative coagulation factor replacement therapy, TKA is a safe and an effective treatment for hemophmc anhmpathy of knee joint, whicht can effectively relieve pain and improve joint function.

Adolescent ; Adult ; Arthritis ; surgery ; Arthroplasty, Replacement, Knee ; methods ; Female ; Hemarthrosis ; surgery ; Hemophilia A ; complications ; drug therapy ; Humans ; Male ; Middle Aged

OBJECTIVETo evaluate the short-term effects of ankle joint replacement for the patients with hemophilic arthritis. To find out the main points for improving the joint function and the patients' life quality. To accumulate more experience for clinical treatment.

METHODSThe Kofoed evaluation system and the AOFAS evaluation system were used to follow-up 6 hemophilic patients with ankle arthritis (6 males, ranging in age from 23 to 57 years, with an average of 41.2 years), who were treated with ankle joint replacement from 2004.7 to 2007.7. The scores before operation, and the 6th,12th months after operation were compared.

RESULTSThe Kofoed evaluation system showed the joint replacement significantly improved the scores for relieving pain, joint function, range of motion and the total score (P<0.05). Except the score for relieving pain, the other three also had significant differences between the 6th month and the 12th month after the operation (P<0.05). Compared with each details of the joint function, there was significant improvement before and after the operation (P<0.05). The ankle got more extended range of motion in the 12th month after operation than the 6th month (P<0.05). The AOFAS evaluation system showed the joint replacement significantly improved the scores for relieving pain, joint function, X-ray measurement and the total score (P<0.05). There was no differences between the 6th month after operation and the 12th month in the results for relieving pain and the X-ray measurement (P>0.05). However, the joint function and the total score had significant differences (P<0.05). Compared with each details of the joint function, there was also significant improvement before and after the operation (P< 0.05), and the walk distance, as well as the joint flexion and extension activities, had differences between the 6th month and the 12th month after the operation. Both of the two evaluation system showed the operation could get good curative effects (P< 0.05), and the artificial joint could constantly be in good condition from the 6th month to the 12th month after operation (P> 0.05).

CONCLUSIONIn this short-term observation, the joint replacement can significantly improve the life quality for the hemophilic patients with ankle arthritis.

Adult ; Ankle Joint ; physiopathology ; surgery ; Arthritis ; etiology ; physiopathology ; surgery ; Arthroplasty, Replacement ; methods ; Hemophilia A ; complications ; Humans ; Male ; Middle Aged