선택 - 화살표키/엔터키 닫기 - ESC


 

Purpura (자색반, 자색반병)

The Root of Ambulatory Care (외래 진료 지침서) 전체 목록 보기
KEYWORDS: ..피하나 점막하로 혈액이 extravasation되어 생기는 적갈색 혹은 자색의 반점 Petechiae (<2mm) - 혈소판 장애에서 흔히 발생 : 혈소판 증가증이나 감소증 모두 원인이 될 수 있으며, 감소 증에 의한 자반증은 혈소판 수치가 5만개 이상이면 잘 발생하지 않는다. Ecchymosis/Bruise(>2mm) - 혈액응고 장애에 흔히 발생 ..일반적으로 손가락 등으로 눌렀을 때 색깔이 소실되지 않는 점으로 홍반이 나 모세혈관 확장과 구분되지만, 작은 혈관종이나 각화혈관종 (angiokeratoma) 과도 구분하기 어려워, 시간을 두고 색깔변화를 관찰해야 하는 경우도 있다. i. Extramedullary erythropoiesis (blue berry muffin baby) ii. Coagulation defects : Protein C and S deficiency, Hemorrhagic disease of the newborn, Hereditary clotting deficiencies. iii. Platelet abnormalities : Immune platelet destruction (Maternal autoimmune thrombocytopenia, Drug-related immune thrombocytopenia) Primary platelet production/function defects (Wiscott-Aldrich syndrome, Fanconi's trauma, Congenital amegakaryocytic thrombocytopenia etc) Kasabach- Merritt syndrome iv. Infection : Congenital (toxoplasmosis, syphilis, viruses, rubella, cytomegalovirus, HSV) Sepsis, Human immunodeficiency virus, Parvovirus B19 v. Trauma i. Henoch-Schonlein purpura - Palpable ii. Acute hemorrhagic edema iii. Collagen-vascular disease and systemic vasculitis Purpura Neonatal 449 The Root of ambulatory care iv. Pigmented purpura v. Infection associated purpura (Bacterial, Viral, Fungal, Parasites) vi. Trauma/factitial purpura vii. Drug eruptions i. Drug effects/side effects (Antibiotics, NSAIDs, Thiazides, Allopurinol, Phenytoin etc) - Nonpalpable ii. Toxins and venoms iii. Senile/trauma iv. Infections v. Malignancy Intravascular origin : usually nonpalpable Vascular origin : usually palpable Extravascular and miscellaneous : may be palpable or nonpalpable 1. Age of onset? → Birth : Intrauterine infection, maternal idiopathic thrombocytopenic purpura, maternal systemic lupus erythematosus, maternal medication, TAR syndrome, congenital amegakaryocytic thrombocytopenia → 2~4years : Idiopathic thrombocytopenic purpura → 4~7years : Henoch-Schonlein purpura → Adult : Drug/Toxin 2. Chronicity? → Acute onset : Idiopathic thrombocytopenic purpura, Henoch-Schonlein purpura, medication, mechanical cause → Long duration : Abnormality of platelets, Coagulopathy 3. Pattern of bleeding → Mucosal bleeding : Thrombocytopenia, von Willebrand's disease → Intramuscular and intra-articular bleeding : Hemophilia 4. Associated symptoms? → Abdominal pain, blood in stools, joint pain : Henoch-Schonlein purpura → Lethargy, fever, bone pain : Leukemia → Intermittent fever, musculoskeletal symptoms : SLE → Lethargy, polyuria,polydipsia : Uremia → Purpura, but otherwise healthy : Idiopathic thrombocytopenic purpura Key questions Children Older Key tests Purpura 5. Drug use → Alkylating agents : Thrombocytopenia → Antimetabolites : Thrombocytopenia 6. Past health → Antecedent viral infection(URI) : Idiopathic thrombocytopenic purpura, Henoch-Schonlein purpura → Systemic lupus erythematosus : SLE → Liver disease : Cirrhosis or chronic hepatitis → Renal disease : Chronic renal failure 7. Family history → von Willebrand's disease : von Willebrand's disease → TAR syndrome : TAR syndrome → Wiskott-Aldrich syndrome : Wiskott-Aldrich syndrome 1. CBC/Peripheral blood smear/PT/aPTT 2. Bleeding time Other tests if indicated by history and physical examination 451 The Root of ambulatory care Fig 1. Algorithm for the diagnosis of purpura (PT=prothrombin time : aPTT=activated partial thromboplastin time) 참고 문헌 John J. Coon : Purpura, in Saunders Manual of Medical Practice, 2nd ed, Robert E. Rakel(ed). Philadelphia, Saunders, 2000, P 1192-1194 송윤미 : 자반증, in 가정의학 임상편. 서울, 계측문화사, 2002, P 1059-1065 Leung, M.B.B.S., Ka Wah Chan, M.B.B.S. : Evaluationg the child with purpura.A journal of the American Family Physicains 2001 ; 64 : 419-428 History and physical examination Etiology clear Prolonged PT and aPTT Prolonged PT and aPTT Bleeding time Sepsis/ Disseminated intravascular coagulopathy Coagulation factor deficiency/ von Willebrand’s disease/Circulating anticoagulant/ Liver disease Platelet dysfuction/ von Willebrand’s disease von Willebrand’s disease/ Vascular purpura/ Child abuse Idopathic thrombocytopenic purpura/Hemolytic-uremic syndrome/Thrombotic thrombocytopenic purpura/ Systemic lupus erythematosus/ Bone marrow aplasia/ Sequestration of platelets Thrombocytopenia CBC/Peripheral blood smear/ PT and aPTT Trauma/Drug/ Infection/Hemangioma/ Malignancy Etiology unclear Yes No Yes No Yes No Normal Prolonged