Hematologic Disorders - Anemia (Part 2) PDF

DISTURBANCES IN OXYGENATION: HEMATOLOGIC DISORDERS PART 2: Hemolytic Anemias (Anemias of Increased Destruction) INHERITED HEMOLYTIC ANEMIAS Abnormal Hemoglobin Sickle Cell Disease Thalassemia Enzyme Deficiency G6PD Deficiency Blood Cell Membrane Abnormality Acanthocy...

DISTURBANCES IN OXYGENATION: HEMATOLOGIC DISORDERS PART 2: Hemolytic Anemias (Anemias of Increased Destruction) INHERITED HEMOLYTIC ANEMIAS Abnormal Hemoglobin Sickle Cell Disease Thalassemia Enzyme Deficiency G6PD Deficiency Blood Cell Membrane Abnormality Acanthocytosis Hereditary Elliptocytosis Hereditary Spherocytosis Stomatocytosis https://www.hopkinsmedicine.org/-/media/images/health/1_- conditions/heart-and-vascular/hemolytic-anemia-teaser-image.ashx SICKLE CELL ANEMIA An autosomal codominant inherited disease Abnormal hemoglobin (HbS) Sickle shaped RBCs Globin fraction abnormality – valine is substituted for glutamic acid in the 6th position of the beta chain Sickle Cell Trait (heterozygous) Sickle Cell Disease (homozygous) Sickle cells have a shortened life span of 7-20 days and is Trivia: Sickle Cell Gene offers a protective destroyed by the spleen effect against Plasmodium spp.  Pathophysiology OXYGEN TENSION DECREASES HbS POLYMERIZES RBC BECOMES SICKLE SHAPE INCREASED BLOOD VISCOSITY/ PROLONGED CIRCULATION TIME/ISCHEMIA CNS: THROMBOSIS/CVA, PARALYSIS, HEPATIC: HEPATOMEGALY, GALL STONES CEREBRAL DEFICITS, DEATH CARDIAC: SYSTOLIC MURMUR, SKELETAL: DACTYLITIS, DEFORMITIES, CARDIOMEGALY, HEART FAILURE/MI OSTEOMYELITIS, OSTEOPOROSIS, FRACTURES RESPI: ACUTE CHEST SYNDROME, GENITAL: PENAL PRIAPISM HYPERTENSION, PNEUMONIA RENAL: HEMATURIA, RENAL FAILURE OPTIC: HEMORRHAGE, RETINOPATHY, BLINDNESS SPLEEN: SPLENOMEGALY, SPLENIC ATROPHY DERMIS: STASIS ULCERS https://3.bp.blogspot.com/_ZWqgY BROGHw/TRkTQY4714I/AAAAAAA ACEA/P7k3L-vNCQQ/w1200-h630- p-k-no-nu/17.jpg  ▪ Three Types: Causes of Crisis: ▪ Vaso-occlusive crisis stress ▪ Aplastic Crisis dehydration ▪ Splenic Sequestration crisis change in oxygen tension in the body ▪ Others: infection ▪ Infectious crisis fever SICKLE CELL ▪ bone, joint and other crisis anesthesia CRISIS ▪ Megaloblastic Crisis over-exertion exposure to cold high altitudes ▪ Two major consequences of high hemoglobin levels rbc sickling: ingestion of alcohol ▪ Chronic Hemolytic Anemia smoking ▪ Blood Vessel Occlusion  https://static01.nyt.com /images/2019/12/07/sci ence/07SICKLECELL1/07S ICKLECELL1- ASSESSMENT & DIAGNOSIS mobileMasterAt3x.jpg Monitor for: Heart Failure https://www.microscopyu.com/assets/gallery- images/pathology_sicklec ellanemia40x02.jpg Fluid Retention Neurologic: Paresthesia, confusion, balance Diagnostic Tests: Screening: Metabisulfite Test (Sickling Test/Sickledex) Confirmatory: Hemoglobin Electrophoresis MANAGEMENT Hydroxyurea Erythropoietin Supplemental Iron, Folic Acid and Vitamin B12 Antibiotics Opioids (Morphine – drug of choice) Antihistamines, NSAIDS Hydration Pain Management O2 Therapy Frequent Transfusions Bone Marrow Transplantation Genetic Counseling Diet: increased CHON, calcium, https://science.sciencemag.org/content/sci/367/6483/1198/F1.large.jpg?width=800&height=600&carousel=1 vitamins and adequate liquids  NURSING MANAGEMENT Manage Pain Manage Fatigue Prevent / Manage Infection Antibiotics and Wound Care Promote Coping Skills Patient Education (Minimize Knowledge Deficit) Hydroxyurea is teratogenic Monitor and Manage Potential Complications Home and Community Based Care https://i.pinimg.com/originals/27/52/32/275232dad5a25489826b1a257a7ea688.jpg  https://i.ytimg.com/vi/o1YubvuTISY/maxresdefault.jpg THALASSEMIA A diverse group of genetic disorder characterized by a primary, quantitative reduction in globin chain synthesis for hemoglobin. Homozygous – Thalassemia Major (Disease) Heterozygous – Thalassemia Minor (Trait) – milder forms Types: Alpha Thalassemia – Barts Hydrops Fetalis, Hb H disease, Constant Spring disease, Silent Carrier Beta Thalassemia – Cooley’s Anemia, Hb Lepore https://www.stepwards.com/wp-content/uploads/2016/01/05_21gProteinHemoglobin-L.jpg PATHOPHYSIOLOGY defective hemoglobin chain synthesis imbalance in hemoglobin configuration precipitates in the erythroid precursors increased rigidity premature destruction of the rbc/ hemolysis in the spleen MANIFESTATIONS Hydrops Fetalis marked skeletal deformities (frontal bossing); cheek bone and jaw protrusions DIAGNOSIS AND MANAGEMENT Diagnostic Findings: Hypochromia and microcytosis, hemolysis Splenomegaly Management: Blood Transfusions; Bone Marrow Transplantation (for patients https://www.stepwards.com/wp-content/uploads/2016/01/img0028.jpg under 5 yrs. old); Splenectomy Consent Pre-Op teaching Monitor for Hemorrhage Prevent pneumonia and atelectasis Monitor platelet count (elevated first 2 weeks) https://www.limamemorial.org/TransAdam/doc/graphics/images/en/17212.jpg INHERITED HEMOLYTIC ANEMIAS Abnormal Hemoglobin Sickle Cell Disease Thalassemia Enzyme Deficiency G6PD Deficiency Blood Cell Membrane Abnormality Acanthocytosis Hereditary Elliptocytosis Hereditary Spherocytosis Stomatocytosis https://www.hopkinsmedicine.org/-/media/images/health/1_- conditions/heart-and-vascular/hemolytic-anemia-teaser-image.ashx  G6PD DEFICIENCY X-linked inherited disease (ENZYME DEFICIENCY Common in African and ANEMIA) Mediterranean Descents https://i.pinimg.com/originals/ 5b/7c/5d/5b7c 5d80780b1be5 726de14cb8ed ed0a.jpg https://images.medindia.net/patientinfo/950_400/symptoms-of-g6pd-deficiency.jpg  Pathophysiology of G6PD Deficiency Deficiency in G6PD enzyme that metabolizes glucose and generate ATP decreased GSH (glutathione) oxidative denaturation of hemoglobin (Methemoglobin) formation of Heinz bodies increased cell rigidity lysis in the spleen MANAGEMENT Recognition of the disorder (Newborn Screening) Avoidance/Cessation of offending substances Hydration Blood transfusion Prompt treatment of infection https://pbs.twimg.com/media/D5gRdY6WAAEcVVe.jpg https://sites.google.com/site/livestrongwithg6pddeficiency/_/rsrc/14 93450217924/website-builder/G6PDD_Prohibited%20items.png Blood Cell Membrane Abnormalities HEREDITARY SPHEROCYTOSIS Autosomal Dominant genetic disorder Alteration in the shape of erythrocytes characterized by a membrane abnormality that leads to osmotic swelling of the RBCs. Manifestations: Anemia – pallor, fatigue, dyspnea Jaundice splenomegaly https://image.slidesharecdn.com/haem14-hemolyticcongenital-150415203434-conversion-gate02/95/haem14- hemolytic-anemia-congenital-4-638.jpg?cb=1429148140  DIAGNOSIS AND MANAGEMENT DIAGNOSIS MANAGEMENT: -Diagnostic Test: Chromium -Splenectomy Survival -Cholecystectomy (due -Laboratory Findings: to increased incidence Increased Osmotic Fragility (Erythrocyte of gall stones) Fragility Test) -Genetic Counseling Increased Reticulocyte count -Energy Conservation Increased Bilirubin Techniques  OTHERS: (Research Work) Hereditary Stomatocytosis Acanthocytosis Elliptocytosis  ACQUIRED HEMOLYTIC ANEMIA Antibody Related Not Antibody Related Autoimmune Hemolytic Anemia Disseminated Intravascular Coagulation Iso-Antibody/Transfusion Reaction Hypersplenism Cold Agglutinin Disease Infection Liver Disease Mechanical Heart Valve Microangiopathic hemolytic anemia Paroxysmal Nocturnal hemoglobinuria Toxins Trauma Uremia IMMUNE MEDIATED HEMOLYTIC ANEMIAS Types of Immune Mediated Antibodies: Autoantibodies – produced by a person in response to drugs and disease Alloantibodies – comes from an exogenous source Classifications of autoantibodies: Warm Reacting Cold Reacting Acquired HEMOLYTIC ANEMIA Causes: chemicals, EXPOSURE HEMOLYSIS OF RBCS toxins, venom, infections (malaria) SIGNS AND SYMPTOMS OF ANEMIA  Warm Reacting Autoimmune Hemolytic Anemia usually idiopathic or may ANTIBODIES (IgG/IgA) ACT ON RBCS AT TEMP 37C UP be associated with SLE, RA, Chronic Lymphocytic Leukemia and myeloma ANTIBODIES REACT WITH ANTIGENS ON RBC MEMBRANE DESTRUCTIVE MEMBRANE CHANGES (SPHEROCYTOSIS) RBC HEMOLYSIS IN THE SPLEEN Cold Reacting Autoimmune Hemolytic Anemia ANTIBODIES (IgM) REACT WITH RBC ANTIGENS AT Etiology: Raynaud’s TEMP 31C BELOW disease, Infectious Mononucleosis, ACTIVATION OF Mycoplasma COMPLEMENT SYSTEM Pneumoniae infection, EBV, mumps and SOME CELLS CLUMP HEMOLYSIS Legionnaire’s Disease IN CAPILLARY BEDS VASCULAR OBSTRUCTION CYANOSIS, PAIN, PARESTHESIA Drug Induced Hemolytic Anemia Reaction to drugs (e.g. Methyldopa, penicillin, quinine and quinidine) production of autoantibody destruction of erythrocyte (HEMOLYSIS) Manifestations  DIAGNOSIS: Positive Coomb’s Test (antiglobulin test) decreased hematocrit increased reticulocyte count increased bilirubin MANAGEMENT Immunosuppressive Agents [cyclophosphamide (Cytoxan) or azathioprine (Imuran)] Corticosteroids (Cyclomen) Splenectomy Blood Transfusions Plasmapheresis Teach patient about drug therapy Avoid exposure to cold (for patients with cold reacting anemias) Help patient cope with illness