Tiêu đề LEUKEMIA | HAEMOPHILIA ✅

HEMOPHILIA A 1. HISTORY TAKING 1. 4C a. Complain i. Bruising at left forearm b. Course (Natural History - If cannot found in book, write based on common case seen) i. Neonatal period : ● Bleeding is unusual (due to less movement) ● Some newborn or infant with haemophilia can present with spontaneous intracranial bleed (only 2%) ● Bleeding with circumcision (30%) ● A child may also present with post-vaccination or vitamin K injection haematoma ii. Childhood: ● Easy bruising in early childhood(start to crawl, walk) ● Excessive bleeding following trauma or surgery ● ‘Spontaneous’ bleeding particularly into the soft tissues, muscles, joints and gums ● Epistaxis iii. The natural history of untreated hemophilia is one of cyclic recurrent hemorrhages into specific joints, including hemorrhages into the same (target) joint. ● In young children : the joint distends easily and a large volume of blood may fill the joint until tamponade ensues or therapy intervenes ● Following hemarthrosis - proteolytic enzymes are released by white blood cells into the joint space, and heme iron induces macrophage proliferation, leading to synovial inflammation ● The synovium thickens and develops frond-like projections into the joint that are susceptible to being pinched and may induce further hemorrhage. ● The cartilaginous surface becomes eroded, may even expose raw bone, leaving the joint susceptible to articular fusion. ● In the older patient with advanced arthropathy, bleeding into the target joint, with its thickened synovium, causes severe pain, because the joint may have little space to accommodate blood. c. Cause i. Deficiency of factor VIII d. Complications - Musculoskeletal complications: i. Synovitis ii. Joint arthropathy
iii. Pseudotumor iv. Re-bleeding v. Compartment syndrome vi. Joint contractures - CNS : Bleeding in the head and sometimes in the brain which can cause long term problems, such as seizures and paralysis - Inhibitor formation against FVIII/FIX - Transfusion-related infections of concern in people with hemophilia i. HIV ii. HBV iii.HCV iv.HAV v.Parvovirus B19 2. Systemic Review a. Exclude complications i. MSK : ● Joint arthropathy ● Deformity ● Secondary osteoarthritis ● Leg length discrepancy (due to epiphyseal overgrowth) ● fractures ● Pseudotumor - may lead to nerve compression (femoral nerve palsy may be caused by iliacus hematomas : paraesthesia at L4 distributions ii. CNS : Seizures ( in cases with history of intracranial bleed) iii. Presence of inhibitors should be suspected in the following situations: ● Poor response to replacement therapy ● Recovery assays are not as expected
● Increase bleeding episodes despite optimal prophylaxis b. Exclude other diagnosis i. Hemophilia B ii. Thrombocytopenia iii. Platelet function disorders iv. Von Willebrand disease v. Vitamin K deficiency vi. Bernard-Soulier syndrome and Glanzmann thrombasthenia (rare) 3. Past Medical History a. Chronic Disease i. Complaint ii. Current control or status iii. Complications of disease and treatment iv. Compliance 4. Past Surgical History a. Active Complaint b. Current control or status c. Complications of disease and surgery d. Compliance to Dr recommendations 5. Social History a. Acute i. Distance from home ● If bleeding episode occur, will require immediate factor replacement ii. Who take care of the patient iii. Knowledge of the disease ● Caretaker need to be aware of patient’s condition - easy to bleed ● Extra caution in taking care of the child b. Chronic i. Distance from home for multiple visit ● If patient require prophylaxis treatment ii. Who take care of the patient and ensure compliance iii. How the disease affects patient lifestyle ● School attendance ● Can patient joins sports activity at school iv. Bantuan kewangan dari mana mana jabatan v. Any association related to this case: ● Haemophilia Society
- All haemophiliacs should be registered with a patient support group e.g. Haemophilia Society. vi. They should have a medic-alert bracelet/chain which identifies them as haemophiliacs and carry a book in which the diagnosis, classification of severity, types of bleeds and admissions can be recorded 6. PHYSICAL EXAMINATION 1. Growth a. Physical b. Mental c. Developmental 2. Nutritional Status 3. Sign of acute illness a. Ecchymosis b. Haematoma c. Active bleeding d. Joint swelling (haemarthrosis)