530 likes | 724 Views
Extern conference. 5 July 2007. History. An 11-year-old boy without known underlying disease CC : Continuous bleeding at traumatic wound 9 days. Present history. An 11-year-old boy was in his usual state of good health.
E N D
Extern conference 5 July 2007
History • An 11-year-old boy without known underlying disease • CC : Continuous bleeding at traumatic wound 9 days
Present history An 11-year-old boy was in his usual state of good health. 10 days PTA, he had a bicycle accident. He suffered from abrasions at his right elbow without bleeding. Film Rt. Elbow suggested a right supracondylar fracture of humerus. The fracture was fixed by using slab and arm sling. He got ibuprofen tid for relieving pain (10 days).
Present history 9 days PTA, he has had a continuous bleeding from his traumatic wound so the wound was dressed once daily. 3 days PTA, he was referred to provincial hospital for proper management. CBC : Hb 8.9 g/dl, Hct 28%, WBC11400/ul, (N73% L17% E14%)Plt 72,000/ul. The initial diagnosis was ITP Thus, he was refered to Siriraj hospital for further diagnosis and treatment.
History • PMH: No history of abnormal bleeding. No previous hospitalization, no prior surgery. • FH: 2nd child. No family history of bleeding disorder. No family history of hereditary disease. • No anticoagulant drug use.
Physical examination • V/S : T 36.8ºc, RR 40/min, PR 96/min, regular, BP 100/56 mmHg • BW 40.8 kg (P75),Height 151.5 cm (P90) • GA : A 11-year-old Thai boy. good consciousness, mildly pale, no jaundice, • Skin : no petechiae, purpura or ecchymosis
Physical examination • HEENT : pharynx and tonsils not injected, TM intact both ears, no mucosal bleeding. • RS : normal breath sounds, no adventitious sound • CVS : normal S1&S2 , no murmur • Abdomen : soft, no distension, active bowel sounds, no mass, liver& spleen not palpable, bimanual palpation negative
Physical examination • NS : muscle power grade V all extremites, normotonia, no sensory deficit DTR 2+ all, no stiff neck • Extremities: Rt arm: abrasion at medial side of right elbow, size 1x2 cm, active bleeding, swelling from elbow to fingers
Problem list • Moderate bleeding symptom at abrasion site at right elbow 9 days • Supracondylar fracture of right humerus from bicycle accident S/P slab 10 days
Bleeding Patient • Clinical Evaluation • History taking • Physical examination • Clinical Evaluation • History taking • Physical examination • Laboratory Investigation • Diagnosis and treatment
Bleeding disorder Local vs. systemic defect Location: single vs. multiple sites Severity: Spontaneous? Appropriate to trauma? Hereditary vs. acquired disorder Onset Family history Underlying disease Medication Primary vs. secondary hemostatic disoder
Primary Hemostasis vessel • Platelet • Adequate number • Normal function • Blood vessel • von Willebrand factor platelet platelet
Secondary Hemostasis • Initiation • Propagation • Fibrin clot formation
Secondary Hemostasis Primary Hemostasis • Onset • Sites • Skin • Mucosal • Others • Immediate • Superficial • Petechiae, superficial ecchymosis • Common • Rare • Delayed • Deep • Deep ecchymosis, hematoma • Rare • Retroperitoneal Hematoma, hemarthrosis
In this patient Local vs. systemic defect Location: single vs. multiple sites Severity: Spontaneous? Inppropriate to trauma Hereditary vs. acquired disorder Onset: 11 years old Family history: none Underlying disease: none Medication: Ibuprofen for 1 day Primary vs. secondary hemostatic disoder
Secondary Hemostasis Primary Hemostasis • Onset • Sites • Skin • Mucosal • Others • Immediate • Superficial • Petechiae, superficial ecchymosis • Common • Rare • Delayed • Deep • Deep ecchymosis, hematoma • Rare • Retroperitoneal Hematoma, hemarthrosis
Bleeding Patient • Clinical Evaluation • History taking • Physical examination • Laboratory Investigation • Diagnosis and treatment
Investigation • CBC at Siriraj hospital: Hb 7.1g/dL, Hct 23%, MCV 81 WBC 8880 /mm3, N 44.2%, L28.2%, Mo 7.7%, Eo19.4%, Ba 0.5% Platelet : 144,000/mm3 • CBCat provincial hospital, 9 day ago: Hb 8.9 g/dl, Hct 28%, WBC11400/ul, (N73% L17% E14%) Plt 72,000/ul.)
Investigation Interpretation of CBC : normochromic normocytic anemia Eosinophilia (>500/ ul) normal platelet count (150,000-400,000/ul) > 100,000/uLBleeding unlikely < 20,000/uL ↑ risk for spontaneous bleeding
Investigation • UA : pH 7, Sp.gr. 1.015, WBC 0-1/HPF, RBC 0 /HPF, • Coagulogram: PT 13.5 sec aPTT 27.5 sec PT-INR 1.17 • Interpretation of coagulogram: no significant prolongation of coagulogram
Primary Hemostatic Disorders Platelet disorder Thrombocytopenia Platelet dysfunction Blood vessel disorder von Willebrand disease
Bleeding tendency Hx, PE Primary hemostatic disorder Secondary hemostatic disorder CBC + platelet count CBC + platelet count+ coagulogram Normal decreased Bleeding time pancytopenia isolated thrombocytopenia Normal prolonged
Bleeding Time: Interpretation Normal value* : 2-7 min: in this patient is >30min Prolonged bleeding time: Thrombocytopenia/ anemia (Hct < 20%) Hereditary platelet dysfunction von Willebrand disease Severe hypofibrinogenemia Blood vessels disorders Uremia Myeloproliferative disorders Medication: Aspirin, NSAIDs, other antiplatelets
Bleeding Time • Limitation • Screening test for platelet function • Increased yield in patient suspected of bleeding disorder • - Operator dependent • - Not very sensitive nor • specific in general • population • - Poor predictor of • surgical bleeding
Differential diagnosis 1. Acquired platelet dysfunction with Eosinophilia 2. von Willebrand Disease (vWD) 3. Drug induced platelet dysfunction
Diagnosis Acquired platelet dysfunction with Eosinophilia (APDE)
APDE • A syndrome characterized by • Reversible spontaneous hemorrhagicdiathesis. • Platelet: bizarre shape, varying in size, pale staining and poor granulation. • Eosinophilia • First described by Mitrakul, 1975. • “Allergic vascular purpura” or “Non-thrombocytopenic purpura with eosinophilia” • Most common bleeding diathesis in children in Thailand (Suvatte, 1979).
Platelet aggregation test • High ADP 12.5 % (55.0-90.0) Low ADP 0.0 %(5.0-15.0) Epinephine 0.0 % (60.0 - 100.0) Collagen 7.5 % (70.0 - 100.0) Arachidonic acid 52.5 % (70.0 - 100.0) Ristocetin 67.5 % (50.0 - 100.0)
Proposed pathophysiology of APDE (Lim, 1989 and Laosombat, 2001) Parasitic infection Eosinophilia + IgE level & IgM production (Hathirat, 1993) Ag-Ab cpx bound plt PAF released from mast cell Plt activation Plt degranulation Storage Pool Deficiency
Certain facts of APDE • Clinical course can intermittently last from a few days to 1 year. • Self-limiting course and spontaneous recovery within 6-12 months. • Recurrence is apparently rare once resolved. • No correlation among number of eosinophil, IgE level and severity of bleeding.
Diagnostic investigation • CBC: • normal plt count, decreased granules, pale giant pltand no aggregation. • eosinophilia • Prolonged bleeding time. • Normal coagulogram and clot retraction. • Abnormal plt aggregation test.
Treatmentin APDE • No specific treatment • Treatment of parasitic infestation. • Symptomatic and supportive treatment • If severe bleeding occurred, platelet transfusion should be performed : 0.4-0.6 u/kg/dose of plt conc every 2-3 d as guided by bleeding time (Hathirat, 1982). • Oral vitamin C supplement.