Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Alterations of Hematologic Function in Children Chapter 21

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Acquired Disorders of Erythrocytes  Iron deficiency anemia  Most common blood disorder of infancy and childhood  Lack of iron intake or blood loss  Manifestations Irritability, decreased activity tolerance, weakness, and lack of interest in play Irritability, decreased activity tolerance, weakness, and lack of interest in play

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Acquired Disorders of Erythrocytes  Acquired congenital hemolytic anemia  Hemolytic disease of the newborn (HDN) Alloimmune disease Alloimmune disease Maternal antibody directed against fetal antigens Maternal antibody directed against fetal antigens ABO incompatibility occurs in 20% to 25% of cases ABO incompatibility occurs in 20% to 25% of cases Rh incompatibility occurs in less than 10% Rh incompatibility occurs in less than 10% Also termed erythroblastosis fetalis Also termed erythroblastosis fetalis

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Acquired Disorders of Erythrocytes  Hemolytic disease of the newborn (HDN)  Manifestations Anemia Anemia Hyperbilirubinemia Hyperbilirubinemia Icterus neonatorum Icterus neonatorum Kernicterus Kernicterus Glucose-6-phosphate dehydrogenase deficiency (G6PD) Glucose-6-phosphate dehydrogenase deficiency (G6PD)

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Hemolytic Disease of the Newborn

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Inherited Disorders of Erythrocytes  Sickle cell disease  Disorders characterized by the presence of an abnormal hemoglobin (HbS) Mutation causes valine to be replaced by glutamic acid Mutation causes valine to be replaced by glutamic acid  Deoxygenation and dehydration cause the red cells to solidify and stretch into an elongated sickle shape

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Inherited Disorders of Erythrocytes  Sickle cell disease  Sickle cell trait Child inherits HbS from one parent and HbA from another Child inherits HbS from one parent and HbA from another  Can result in: Vaso-occlusive crisis (thrombotic crisis), aplastic crisis, sequestration crisis, and hyperhemolytic crisis Vaso-occlusive crisis (thrombotic crisis), aplastic crisis, sequestration crisis, and hyperhemolytic crisis  Other forms Sickle cell-thalassemia disease and sickle cell-HbC disease Sickle cell-thalassemia disease and sickle cell-HbC disease

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Sickle Cell Disease

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Sickle Cell Disease

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Sickle Cell Disease

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Inherited Disorders of Erythrocytes  Thalassemias  Autosomal recessive disorders  Synthesis of the globin chains of the hemoglobin molecule is slowed or defective  Major—homozygous inheritance  Minor—heterozygous inheritance

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Inherited Disorders of Erythrocytes  In alpha thalassemia, the alpha chains are affected; beta chains in beta thalassemia  Beta-thalassemia minor  Beta-thalassemia major (Cooley anemia) Life-threatening condition Life-threatening condition

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Inherited Disorders of Erythrocytes  Alpha trait  Alpha-thalassemia minor  Hemoglobin H disease  Alpha-thalassemia major Fatal condition Fatal condition

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Beta Thalassemia Major

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Inherited Coagulation and Platelet Disorders  Hemophilias  Serious bleeding disorders  Hemophilia A (factor VIII deficiency) von Willebrand disease von Willebrand disease  Hemophilia B (factor IX deficiency)  Hemophilia C (factor XI deficiency)

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Antibody-Mediated Hemorrhagic Disease  Idiopathic thrombocytopenic purpura  Autoimmune or primary thrombocytopenic purpura  Platelet destruction rate that exceeds production  70% with antecedent viral disease

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Antibody-Mediated Hemorrhagic Disease  Idiopathic thrombocytopenic purpura  Manifestations of bruising and petechial rash  Prognosis excellent with 80% regaining normal platelet counts within 6 months after onset

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Leukemia  Most common malignancy of childhood  80% to 85% are acute lymphoblastic leukemias (ALL); remainder acute myelogenous leukemia (AML)  Result of ecogenetics  Manifestations of pallor, fatigue, purpura, and fever  Blast cell is hallmark of ALL

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Leukemia

Lymphoma  Non-Hodgkin lymphoma (NHL)  Nodular or diffuse  Childhood NHL subdivided Large cell (histiocytic) Large cell (histiocytic) Lymphoblastic Lymphoblastic Small noncleaved cell (Burkitt or non-Burkitt) Small noncleaved cell (Burkitt or non-Burkitt)

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Lymphoma  Non-Hodgkin lymphoma (NHL)  Etiology viral, chronic immunostimulation, congenital immunodeficiency syndromes, and genetics  Manifestations specific to site involved  Treatment of chemotherapy and radiation

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Lymphoma  Hodgkin lymphoma  Rare in childhood  Infectious mode of transmission  Many children with Hodgkin lymphoma demonstrate a high antibody titer to Epstein- Barr virus (EBV)  Manifestations of painless adenopathy, anorexia, malaise, fever, and weight loss  Treatment by MOPP or ABVD

Elsevier items and derived items © 2008 by Mosby, Inc., an affiliate of Elsevier Inc. Lymphoma

Lymphoma