Hemophilic Arthropathy—Pathophysiology and Advances in Treatment
Hemophilia is an X-linked genetic disorder that predominantly affects males, with females typically serving as asymptomatic carriers. Hemophilia A results from a deficiency or dysfunction of coagulation factor VIII, while a deficiency in factor IX causes hemophilia B. A less common condition, factor...
Saved in:
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2025-04-01
|
| Series: | Rheumato |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2674-0621/5/2/5 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849431775068028928 |
|---|---|
| author | Katarina Kovač Ivan Ljudevit Caktaš Nataša Kalebota Porin Perić |
| author_facet | Katarina Kovač Ivan Ljudevit Caktaš Nataša Kalebota Porin Perić |
| author_sort | Katarina Kovač |
| collection | DOAJ |
| description | Hemophilia is an X-linked genetic disorder that predominantly affects males, with females typically serving as asymptomatic carriers. Hemophilia A results from a deficiency or dysfunction of coagulation factor VIII, while a deficiency in factor IX causes hemophilia B. A less common condition, factor XI deficiency (formerly hemophilia C), is categorized as a rare bleeding disorder. The severity of hemophilia is classified based on the activity concentration of factors VIII and IX: severe (<1 IU/dL), moderate (1–5 IU/dL), and mild (6–<40 IU/dL). One of the most prevalent complications of hemophilia is hemarthrosis, bleeding into joint cavities, which, if unrecognized or untreated, can lead to hemophilic arthropathy. The pathophysiology of hemophilic arthropathy involves two key mechanisms: the accumulation of iron from blood in synovial joints, which cannot be cleared due to repeated bleeding, and the inflammatory response, resulting in synovial hyperplasia and the progressive destruction of cartilage and bone. Hemophilic arthropathy significantly impairs quality of life, causing chronic pain, joint deformities, and sometimes requiring surgical intervention. This thesis will examine the pathophysiology and management strategies for hemophilic arthropathy. |
| format | Article |
| id | doaj-art-7f136e72a2914562aaabb36dcdca5b5f |
| institution | Kabale University |
| issn | 2674-0621 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Rheumato |
| spelling | doaj-art-7f136e72a2914562aaabb36dcdca5b5f2025-08-20T03:27:32ZengMDPI AGRheumato2674-06212025-04-0152510.3390/rheumato5020005Hemophilic Arthropathy—Pathophysiology and Advances in TreatmentKatarina Kovač0Ivan Ljudevit Caktaš1Nataša Kalebota2Porin Perić3School of Medicine Zagreb, University of Zagreb, 10 000 Zagreb, CroatiaClinic for Rheumatic Diseases and Rehabilitation, University Hospital Centre Zagreb, 10 000 Zagreb, CroatiaSchool of Medicine Zagreb, University of Zagreb, 10 000 Zagreb, CroatiaSchool of Medicine Zagreb, University of Zagreb, 10 000 Zagreb, CroatiaHemophilia is an X-linked genetic disorder that predominantly affects males, with females typically serving as asymptomatic carriers. Hemophilia A results from a deficiency or dysfunction of coagulation factor VIII, while a deficiency in factor IX causes hemophilia B. A less common condition, factor XI deficiency (formerly hemophilia C), is categorized as a rare bleeding disorder. The severity of hemophilia is classified based on the activity concentration of factors VIII and IX: severe (<1 IU/dL), moderate (1–5 IU/dL), and mild (6–<40 IU/dL). One of the most prevalent complications of hemophilia is hemarthrosis, bleeding into joint cavities, which, if unrecognized or untreated, can lead to hemophilic arthropathy. The pathophysiology of hemophilic arthropathy involves two key mechanisms: the accumulation of iron from blood in synovial joints, which cannot be cleared due to repeated bleeding, and the inflammatory response, resulting in synovial hyperplasia and the progressive destruction of cartilage and bone. Hemophilic arthropathy significantly impairs quality of life, causing chronic pain, joint deformities, and sometimes requiring surgical intervention. This thesis will examine the pathophysiology and management strategies for hemophilic arthropathy.https://www.mdpi.com/2674-0621/5/2/5joint diseasesfactor VIII deficiencyfactor IX deficiencyhemophilia Ahemophilia Bsynovitis |
| spellingShingle | Katarina Kovač Ivan Ljudevit Caktaš Nataša Kalebota Porin Perić Hemophilic Arthropathy—Pathophysiology and Advances in Treatment Rheumato joint diseases factor VIII deficiency factor IX deficiency hemophilia A hemophilia B synovitis |
| title | Hemophilic Arthropathy—Pathophysiology and Advances in Treatment |
| title_full | Hemophilic Arthropathy—Pathophysiology and Advances in Treatment |
| title_fullStr | Hemophilic Arthropathy—Pathophysiology and Advances in Treatment |
| title_full_unstemmed | Hemophilic Arthropathy—Pathophysiology and Advances in Treatment |
| title_short | Hemophilic Arthropathy—Pathophysiology and Advances in Treatment |
| title_sort | hemophilic arthropathy pathophysiology and advances in treatment |
| topic | joint diseases factor VIII deficiency factor IX deficiency hemophilia A hemophilia B synovitis |
| url | https://www.mdpi.com/2674-0621/5/2/5 |
| work_keys_str_mv | AT katarinakovac hemophilicarthropathypathophysiologyandadvancesintreatment AT ivanljudevitcaktas hemophilicarthropathypathophysiologyandadvancesintreatment AT natasakalebota hemophilicarthropathypathophysiologyandadvancesintreatment AT porinperic hemophilicarthropathypathophysiologyandadvancesintreatment |