Bleeding manifestation of Hemophilia is
Correct Answer: Hemarthrosis
Description: (B) Hemarthrosis[?]Hemophilia is an X linked disorder of coagulation caused by the deficiency in a circulating plasma protein.oHemophilia A is caused by the deficiency of factor VIII, and hemophilia B is caused by the deficiency of factor IX.oIt is PTT which is affected (increased) and not PT (unaffected).oFactor VIII is involved in the intrinsic pathway which is measured by PTT and not in extrinsic pathway which is measured as PT.oBleeding is the common manifestation of hemophilia and the common bleeding manifestations are Hemarthoses, Hematomas, Mucocutaneous bleeding, Intracranial bleeding, Hematuria and Pseudotumor.i|SITES OF BLEEDINGSeriousLife-threatening* Joints (Hemarthrosis)* Central Nervous System (CNS)* Muscle/soft tissue* Gastrointestinal (GI)* Mouth/gums/nose* Neck/throat* Hematuria* Severe trauma [?]Clinical Manifestations: HemarthrosisoPathophysiology:-Bleeding probably starts from synovial vessels into the synovial space.-Re-absorption of this blood is often incomplete leading to chronic proliferative synovitis, where the synovium is more thickened & vascular, creating a 'target joint' with recurrence of bleeding.-There is destruction of surrounding structures as well-bone necrosis & cyst formations, osteophytes.-Terminal stage: Chronic Hemophiliac arthropathy: Fibrous or bony ankylosing of the joint.oDefects of secondary hemostasis (coagulation factor defects) often present with bleeds into soft tissues (e.g., muscle) or joints. Bleeding into joints (hemarthrosis) following minor trauma is particularly characteristic of hemophilia.oIt is unknown why severe defects in secondary hemostasis present with this peculiar pattern of bleeding; as with severe platelet defects, intracranial hemorrhage, sometimes fatal, may also occur.Other Options[?]Hemoptysis:-Diseases causing repeated haemoptysis include carcinoma, bronchiectasis, carcinoid tumours and some infections.-Severe mitral stenosis is now a rare cause. Patients with repeated haemoptysis should be investigated, at the very least by chest radiography and bronchoscopy.-Haemoptysis following trauma may be from a lung contusion or injury to a major airway. Treatment depends on the underlying cause.[?]Hematomas:-If large, painful or causing neural deficit, a haematoma may require release by incision or aspiration.-In the gluteal or thigh region, there may be an associated disruption of fat in the form of a fat fracture, which results in an unsightly groove but intact skin.-An untreated haematoma may also calcify and therefore require surgical exploration if symptomatic.[?]Hematuria:-Blood in the urine (haematuria) may be the only indication of cancer and other pathology in the urinary tract.-Microscopic haematuria may be detected by dipstick testing in a routine health check. A substantial haemorrhage imparts a red or brownish tinge to the urine (macroscopic haematuria) and the patient may pass clots.
Category:
Pathology
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