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Evaluating the Child with Purpura ALEXANDER K.C Evaluating the Child with Purpura ALEXANDER K.C. LEUNG, M.B.B.S., University of Calgary Faculty of Medicine, Alberta, Canada KA WAH CHAN, M.B.B.S., University of Texas M.D. Anderson Cancer Center, Houston, Texas Purpura is the result of hemorrhage into the skin or mucosal membrane. It may rep- resent a relatively benign condition or herald the presence of a serious underlying disorder. Purpura may be secondary to thrombocytopenia, platelet dysfunction, coagulation factor deficiency or vascular defect. Investigation to confirm a diagno- sis or to seek reassurance is important. Frequently, the diagnosis can be established on the basis of a careful history and physical examination, and a few key labora- tory tests. Indicated tests include a complete blood cell count with platelet count, a peripheral blood smear, and prothrombin and activated partial thromboplastin times. (Am Fam Physician 2001;64:419-28.) urpura results from the extrava- vessel wall and, in response to the exposed sation of blood from the vascula- subendothelial collagen, release adenosine 3 ture into the skin or mucous diphosphate (ADP) and thromboxane A2. membranes. Therefore, purpuric The released ADP and thromboxane A2 cause lesions do not blanch with pres- further platelet aggregation and the formation Psure. Depending on their size, purpuric of a platelet plug that is responsible for pri- lesions are traditionally classified as petechiae mary hemostasis.2,3 (pinpoint hemorrhages less than 2 mm in Secondary hemostatic mechanisms consist greatest diameter), purpura (2 mm to 1 cm) of a series of sequential enzymatic reactions or ecchymoses (more than 1 cm).1 Although involving various coagulation factors and purpura itself is not dangerous, it may be the leading to the formation of a fibrin clot. The sign of an underlying life-threatening disor- intrinsic pathway is activated by the exposed der. This article reviews the etiology of pur- collagen, and the extrinsic pathway is activated pura in children and suggests an approach to by tissue thromboplastin (Figure 1).3 evaluating the problem. The integrity of the vascular system depends on three interacting elements: platelets, plasma Normal Hemostasis coagulation factors and blood vessels. All three The normal hemostatic mechanisms are elements are required for proper hemostasis, vascular response, platelet plug formation and but the pattern of bleeding depends to some activation of coagulation factors with the for- extent on the specific defect. In general, platelet mation of fibrin to stabilize the platelet plug. disorders manifest petechiae, mucosal bleed- Following a vascular injury, vasoconstric- ing (wet purpura) or, rarely, central nervous tion and retraction usually occur immediately system bleeding; coagulation disorders present and decrease blood flow to the affected area. as ecchymoses or hemarthrosis; and vasculitic Factor VIII–von Willebrand’s factor (factor disorders present with palpable purpura.1 VIII–vWF) is released from endothelial cells and adheres to the exposed collagen matrix.2 Platelet Disorders Facilitated by factor VIII–vWF, platelets Simple purpura strongly indicates the pres- adhere to the endothelial cells of the damaged ence of a qualitative or quantitative platelet disorder. THROMBOCYTOPENIA Because purpura results from extravasation of blood into the Thrombocytopenia may be caused by in- skin, the lesions do not blanch with pressure. creased platelet destruction, decreased platelet production or sequestration of platelets. AUGUST 1, 2001 / VOLUME 64, NUMBER 3 www.aafp.org/afp AMERICAN FAMILY PHYSICIAN 419 Increased Platelet Destruction. Immune respiratory tract infection, is common. The thrombocytopenia is the most frequent cause peak incidence is between two and four years of increased platelet destruction. Idiopathic of age. Both genders are equally affected. (immune) thrombocytopenic purpura is by Fever, lethargy, weight loss, bone pain, joint far the most common etiology of thrombocy- pain, pallor, lymphadenopathy and hepato- topenia in childhood. The disorder is caused splenomegaly are characteristically absent. by the development of IgG autoantibodies to Minimal splenomegaly occurs in about 5 to 10 platelet membrane antigens as a result of an percent of symptomatic children.5 Idiopathic unbalanced response to an infectious agent or thrombocytopenic purpura is usually a tem- autoimmunity.4 The characteristic presenta- porary disorder, with 80 to 90 percent of chil- tion is the sudden onset of bruises, purpura, dren recovering within six to 12 months, usu- mucosal hemorrhage and petechiae in a child ally within a few weeks.6 Chronic idiopathic who is otherwise in excellent health. An thrombocytopenic purpura is more likely to antecedent viral infection, especially an upper present in teenage girls and children with Coagulation Cascade Intrinsic pathway Extrinsic pathway Vascular surface changes Tissue thromboplastin XII XIIa VII VIIa XI XIa IX IXa VIII Common pathway X Prothrombin (II) Xa + V Thrombin XIIIa XIII Fibrinogen (I) Fibrin monomer Fibrin polymer Stable fibrin Clot formation FIGURE 1. A simplified version of the coagulation “cascade.” An abnormality in the extrinsic pathway results in a prolonged prothrombin time (PT). An abnormality in the intrinsic pathway results in a prolonged activated partial thromboplastin time (aPTT). An abnormality in the com- mon pathway results in prolongation of PT and aPTT. Adapted with permission from Cohen AR. Rash—purpura. In: Fleisher GA, Ludwig S, et al., eds. Textbook of pediatric emergency medicine. 3d ed. Baltimore: Williams & Wilkins, 1993:430-8. 420 AMERICAN FAMILY PHYSICIAN www.aafp.org/afp VOLUME 64, NUMBER 3 / AUGUST 1, 2001 Purpura underlying immune disorders. It has a more insidious onset. Idiopathic thrombocytopenic purpura is the most common Drugs that act as a hapten with platelet sur- cause of thrombocytopenia in children. face antigens to form an immunologic moiety can also cause an immune thrombocytopenia. This has been shown to occur with penicillin, valproic acid (Depakene), quinidine, sulfon- Escherichia coli O157:H7. The pathologic amides, cimetidine (Tagamet) and heparin. process is initiated by toxin-induced endothe- Thrombocytopenia secondary to immune lial injury and is followed by fibrin deposition destruction may rarely be the presenting fea- in the renal microvasculature and destruction ture of human immunodeficiency virus, of red blood cells and platelets. In addition, cytomegalovirus and herpesvirus infections.5 vasoactive and platelet-aggregating substances It also occurs in approximately 10 percent of are released from damaged endothelial cells patients with systemic lupus erythematosus; and result in the formation of platelet at times, thrombocytopenia may be the pre- thrombi.8 senting manifestation. In hemolytic-uremic syndrome, thrombo- Post-transfusion purpura is characterized cytopenia may result from platelet destruc- by the acute onset of thrombocytopenia tion, increased consumption of platelets, approximately five to 14 days after a transfu- intrarenal aggregation of platelets, sequestra- sion.7 Previous transfusions may sensitize tion of platelets in the liver or spleen, or a patients to foreign platelet antigens. combination of these factors.8 The remaining Neonatal isoimmune (alloimmune) throm- platelets in the circulation appear to be bocytopenia develops when the mother pro- “exhausted” and circulate in a degranulated duces alloantibodies in response to a fetal form, depleted of nucleotide and granule con- platelet antigen, most commonly P1A1, which tents. From a functional perspective, these is not present on maternal platelets. These IgG platelets demonstrate a pattern characteristic antibodies cross the placenta and cause of impaired aggregation. thrombocytopenia in the fetus. The condition Thrombotic thrombocytopenic purpura occurs most commonly in P1A1-negative and hemolytic-uremic syndrome have gener- women who have been previously sensitized ally similar clinical and laboratory findings. to P1A1-positive platelets.2,5 However, thrombotic thrombocytopenic pur- Neonatal autoimmune thrombocytopenia pura occurs more often in adults, and neuro- may be caused by idiopathic thrombocyto- logic (rather than renal) symptoms are more penic purpura, systemic lupus erythematosus prominent. or drug-related immune-mediated purpura. Disseminated intravascular coagulopathy is In these disorders, maternal autoantibodies characterized by generalized activation of the cross the placenta, bind to fetal platelets and plasma coagulation pathways within small cause their destruction.5 blood vessels, with the formation of fibrin Nonimmune thrombocytopenia may occur and the depletion of all coagulation factors because of hemolytic-uremic syndrome, and platelets. This disorder may result from thrombotic thrombocytopenic purpura or overwhelming sepsis, incompatible blood disseminated intravascular coagulopathy. transfusion, snake bite, giant hemangioma Hemolytic-uremic syndrome is characterized and malignancy. Children with disseminated by the triad of microangiopathic hemolytic intravascular coagulopathy are generally anemia, thrombocytopenia and acute renal quite ill and may present with extensive pur- injury. The syndrome is most often associated puric lesions and petechiae, as well as multi- with infection by verocytotoxin-producing focal hemorrhage.9 AUGUST 1, 2001 / VOLUME 64, NUMBER 3 www.aafp.org/afp AMERICAN FAMILY PHYSICIAN 421 Purpura fulminans is an acute, often lethal recurrent infections
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