Chapter 19


Note: Page numbers followed by "f" and "t" indicate figures and tables, respectively.

Acanthocytes, 307 Accuracy/precision clarification of, 9, 10f Activated partial thromboplastin time (aPTT), 238 automated procedure for, 315-318 in hemophilia A diagnosis, 260 Activated protein C resistance (Factor V Leiden), 285 laboratory diagnosis, 285-286 pathway, 285f Acute basophilic leukemia, 175 Acute erythroid leukemia, 173-174, 173f Acute hemolytic anemia, 103-104 Acute lymphoblastic leukemia (ALL), 175 clinical features, 175-176 epidemiology, 175

morphological classification, 176, 176t precursor B/lymphoblastic lymphoma, 176-178

precursor T lymphoblastic leukemia/lymphoblastic lymphoma,

178-179 prognostic factors, 178f, 179-180 Acute megakaryoblastic leukemia, 174, 174f Acute monoblastic leukemia, 172-173, 172f Acute monocytic leukemia, 172, 172f Acute myelofibrosis, 175 Acute myeloid leukemia (AML), 161 chromosomal alterations in, 168t clinical features, 162, 163t conditions with increased risk for, 162t cytochemical staining, 163-166 with 11q23, 169, 170f epidemiology of, 161-162 immunophenotypic classification, 166-167, 166t with inv(16)(p13q22), 168, 168f laboratory features, 163 with maturation, 171, 171f minimally differentiated, 170, 171f with myelodysplasia, 170 not otherwise categorized, 170 with recurrent genetic abnormalities, 167-168 with t(8;21)(q22;q22), 168, 168f with t(15;17)(q22;q21), 168-169 with t(16;16)(p13q22), 168 therapy-related, 170 WHO classification of, 166-167, 167t without maturation, 170-171, 171f Acute myelomonocytic leukemia (AMML), 171-172, 172f Acute promyelocytic leukemia, 168-169, 169f Adenosine diphosphate, 234-235 Adult bone marrow formation in, vs. in fetus, 16f WBC differential values in, 139t, 307t Afibrinogenemia, 270 Agglutination vs. rouleaux, 213f AIDS. See HIV-AIDS

Alder's anomaly (Alder-Reilly anomaly), 149, 149f ALL. See Acute lymphoblastic leukemia Allosteric changes, in Hgb, 53 Alpha-1-antitrypsin, 273 Alpha-2-antiplasmin, 239, 272-273, 283 Alpha-2-macroglobulin, 239-240, 273 Alpha-naphthyl butyrate/acetate esterase stains, 165-166 AML. See Acute myeloid leukemia AMML. See Acute myelomonocytic leukemia Anemias. See also specific types acute hemolytic, 103-104 bone marrow response to, 18 of chronic disease/inflammation, 71-72

conditions leading to, 72t hemolytic classification by intrinsic/extrinsic defects, 58t terminology, 58 iron-deficiency, 68-70 macrocytic vs. megaloblastic, 86 morphological classification of, 23-25 pathophysiology-linked symptoms, 26t sickle cell, 115-120 sideroblastic, 72-74 Anisocytosis, 39 Ankyrin, 38

Antiangiogenic therapies, 223 Anticardiolipin (ACA), 286-287 Anticoagulant drugs, 289 Anticoagulant therapy, 288 Antiglobulin test, direct, 207 Antihemophilic factor (Factor VIII), 236 complex formation with vWF, 258, 260f thrombotic disorder associated with, 286 Antiphospholipid antibodies laboratory assays for, 287 Antiphospholipid syndrome, 286-287 Antiplatelet drugs, 288 Antithrombin (AT) deficiency of, 284 effects on serine proteases, 284f in thrombosis, 283 Aplastic anemia, 105 Arachidonic acid (AA), 235

338 Index

Aristotle, 230 Aspirin, 288

platelet function and, 251 AT. See Antithrombin Auer rods, 163, 168f, 308 Autoimmune hemolytic anemia, 207

Bacteria in cerebrospinal fluid, 316f extracellular, in peripheral smears, 153f intracellular, in segmented neutrophil, 153f in peripheral smear, 153 phagocytosis of, 145 in precipitated stain, 153f Baldes, 230

B-ALL. See Precursor B lymphoblastic leukemia Bands, 132f hypogranular, 221f identification criteria, 132 toxic granulation in, 308 Barts hydrops fetalis, 76 Basophilic normoblast, 36, 36f Basophilic normoblast-nucleated, 36f Basophilic stippling, 45, 46f, 308 Basophils, 133f identification criteria, 133 increase in, conditions associated with, 144 Sysmex scatterplot of, 324f B cells, 136

Beckman-Coulter instrumentation, 322-323 Bence Jones protein, 212 Bennett, John, 161

Bernard Soulier syndrome (BSS), 250

Biohazard shield, 7f

Bite cells (helmet cells), 44f, 45f, 103f formation, 104f Bizzozero, 232 Blast crisis in CML, 189, 190 Blasts excess, refractory anemia with, 222 Bleeding evaluation of, 258 iron deficiency and, 66, 68, 70-71 open vs. closed, 258 secondary to chronic disease, 263 B lymphocytes, 137 Bone marrow in acute myeloid leukemia, 163

in chronic lymphocytic leukemia, 206f in chronic myelogenous leukemia, 190, 190t dysplasia, in MDSs, 220

formation in fetus, vs. adult, 16f

Gaucher's cell, 152f in hematopoiesis, 16

incorporation of vitamin B12 into, 88

internal structure, 19t in leukemia, 160

in lipid storage diseases, 152-153

M:E ratio and, 18 Niemann-Pick cell, 152f responses to anemic stress, 87t ringed sideroblasts in, 72f Bone marrow aspiration, 21, 21f Bone marrow report, 21-22, 23f Brodie, 230 Burr cells, 43, 45f, 307

C1 activator, 273 Cabot rings, 308

Calcium, ionized (Factor IV), 235 Calmodulin, 38 Capillary tubes, 299f, 300f Carboxyhemoglobin, 54, 55 CD. See Cluster designation CD markers, 329t in acute leukemias, 174, 175, 177 in hairy cell leukemia, 208 of lymphocytes, 134t, 137 in T-cell development, 179f CEL. See Chronic eosinophilic leukemia Cell-Dyne technology, 325-326, 326f Cellular immunity, 19 Centrifuge standard microhematocrit, 299f Cerebrospinal fluid (CSF) bacteria in, 316f causes of abnormal cells in, 317t cell count and differential, 313-315 normal values of, 317t reference factors for calculations, 315t color and appearance of, 318t CFU-GEMM. See Committed stem cells CFU-S. See Colony-forming units-spleen Charache, Samuel, 117

Chediak-Higashi syndrome, 149-150, 149f, 251 Cheilitis, 69 Chemical hazards, 8 Chemotaxis in white cell phagocytosis, 145 Cholelithiasis, 99

Christmas disease. See Hemophilia B, factor IX

deficiency Chronic diseases anemia of, 71-72 Chronic eosinophilic leukemia (CEL), 192 Chronic lymphocytic leukemia (CLL) bone marrow view in, 206f disease progression in, 206-207 immunological function/treatment options, 207 modified Rai staging for, 207t smudge cells of, 206f Chronic myelogenous leukemia (CML) clinical features/symptoms, 189-190 diagnosis, 191 key facts of, 189t neutrophilic leukemoid reaction vs., 191t overview, 189

pathophysiology of, 189 peripheral blood/bone marrow findings, 190, 190t prognosis, 191-192 treatment, 191-192 Chronic myeloproliferative disorders (CMPDs). See also specific disorders characteristics of, 189t differentiation of, 198t-199t interrelationships of, 188f terminology of, 188 WHO classification of, 188t Chronic neutrophilic leukemia (CNL), 192 CLL. See Chronic lymphocytic leukemia Cluster designation (CD), 137. See also CD markers CML. See Chronic myelogenous leukemia CMPDs. See Chronic myeloproliferative disorders CNL. See Chronic neutrophilic leukemia Coagulation, blood abnormalities in pathogenesis of thrombosis, 283 extrinsic pathway, 237-238 inhibitors of, 240, 240f, 283-284 intrinsic system, 238 laboratory model of, 237 study of, 230 in vitro cascade, 237f in vivo cascade, 237f Coagulation factors, 236t classification of, 235-237 congenital deficiencies of with absent/mild bleeding, 262 with bleeding manifestations, 262 Coefficient of variation, 9 Cold agglutinin syndrome (CAS), 107-108 Collagen, 231

as aggregating agent, 235 Collagen vascular disease anemia associated with, 71 Colony-forming units-spleen (CFU-S), 19 Committed stem cells (CFU-GEMM), 19, 20 Complement system, 240 Complete blood count (CBC), 22-23 critical values, 26 in HIV disease, 151 normal values, 25t sample report, 24f Congenital nonspherocytic hemolytic anemia, 105

Cooley, Thomas, 74-75 Cooley's anemia, 78-79 Coulter principle, 321, 321f Coumadin, 238, 264, 289 COX-2 inhibitors platelet function and, 251 Critical results/values for CBC, 26 definition of, 11 sample, 139t for WBC, 22 Cryoprecipitate, 261, 270 CSF See Cerebrospinal fluid

Cubilin, 89 Cytokines, 19-20, 21t in phagocytosis, 147t Cytomegalovirus (CMV), 150-151 Cytoplasm red cell, 43 Cytospin method in body fluid differentials, 314-315

d-dimers, 273-274

qualitative test for, 318-320 Deleted 5q, 222 Delta checks, 10 Dextran platelet function and, 251 Diamond-Blackfan anemia, 106 DIC. See Disseminated intravascular coagulation Direct antiglobulin test, 207

Disseminated intravascular coagulation (DIC), 43, 273 acute, mechanism of, 273-274 conditions precipitating, 273f events triggering, 274t laboratory profile in, 275t treatment, 276 DNA synthesis, 87 Dohle bodies, 146f, 148, 148f, 308 Donath-Landsteiner test, 108 Down syndrome, 161, 175 Duckert, F., 230 Dysfibrinogenemia, 271

Ehrlich, Paul, 148, 161 Ehrlichiosis. See Human ehrlichiosis Electrical hazards, 8 Electrophoresis alkaline, hemoglobin, points in analysis of, 120f hemoglobin, 119-120 serum protein, 210

patterns in serum of normal vs. multiple myeloma patients, 210f

Elliptocytes, 42-43, 43f, 101f Elliptocytosis. See Hereditary elliptocytosis Embden-Meyerhof pathway, 40f in pyruvate kinase deficiency, 105 in RBC metabolism, 38-39 Endothelium, 231 Environmental hazards, 8 Eosinophilia definition of, 144 Eosinophils, 133f allergy and, 144 identification criteria, 132 Epinephrine as aggregating agent, 234 EPO. See Erythropoietin Epstein, 161

Epstein-Barr virus (EBV), 150

340 Index

Erythrocyte sedimentation rate (ESR), 300

conditions associated with increase/decrease in, 302 normal values, 302 Sediplast ESR rack, 301f Erythrocytosis secondary, causes of, 193 Erythroid hyperplasia, 75f Erythropoiesis, 34, 34f ineffective consequences of, 87t in megaloblastic anemia, 86-87 Erythropoietin (EPO), 18, 20-21, 34

increased production of, 27 ESR. See Erythrocyte sedimentation rate Essential thrombocythemia (ET)

clinical features/symptoms, 196-197 diagnosis, 197 diagnostic criteria for, 198t key facts for, 196t pathophysiology of, 196 peripheral blood/bone marrow findings, 197 prognosis, 197 treatment, 197 Esterase stains, 165-166 ET. See Essential thrombocythemia Extramedullary hematopoiesis, 17 Eye protection, 7f

Factor I. See Fibrinogen

Factor II. See Prothrombin

Factor III. See Thromboplastin

Factor IV See Calcium, ionized

Factor V Leiden, 262

Factor VII. See Proconvertin

Factor VIII. See Antihemophilic factor

Factor IX. See Thromboplastin, plasma component

Factor X. See Stuart-Prower factor

Factor XI. See Thromboplastin, plasma antecedent

Factor XII. See Hageman factor

Factor XIII. See Fibrin-stabilizing factor

Fanconi's anemia, 105-106, 161-162

Fauvism, 104

Ferritin, 66, 70-71

Fibrin conditions elevating, 273t degradation products of, 273f formation of, 270 measurement of, 273-274 Fibrinogen (Factor I), 235 in clot formation, 231 disorders of, 270-271 in hemostasis, 270 thrombin's activity on, 271f Fibrinolysis abnormalities, in pathogenesis of thrombosis, 283 in disseminated intravascular coagulation, 275 natural inhibitors of, 272-273 physiological activators of, 272 Fibrinolytic system, 239-240

disorders of, in thrombotic disease, 286 measurable products of, 273-274 Fibrin-stabilizing factor (Factor XIII), 236

deficiency of, 263 Fibrometer, 230 Fibronectin receptors, 234 Flagging signals, 10 Flame cells, 211, 211f Fletcher factor, 237 Flow cytometer internal components of, 328f Flow cytometry, 326-327 applications of, 327t data interpretation, 327-328 in diagnosis of PNH, 107 graphing of data from, 329f Fluids, body cell count and differential, 313-315 normal values of, 317t reference factors for calculations, 315t worksheet, 315t Folic acid, 41 in DNA synthesis, 87 nutrition requirements of, 87-88 Folic acid deficiency, 89-90 in megaloblastic anemia, 86

Gaucher's cells, 152f Gaucher's disease, 152

G-CSF See Granulocyte-colony-stimulating factor Glanzmann's thrombasthenia, 250-251 Gloves, 6, 7f

Glucose-6-phosphate dehydrogenase (G6PD) deficiency, 102 agents causing hemolysis in, 104 clinical manifestations, 103-105 diagnosis of, 105 genetics of, 102-103 genotypes of, 103t Glutamic acid carboxylation of, 264f GM-CSF See Granulocyte-macrophage colony-stimulating factor Golhorm, 230 Granulation abnormal, platelet, 221f toxic, 308

Granulocyte-colony-stimulating factor (G-CSF), 19 Granulocyte-macrophage colony-stimulating factor (GM-CSF), 19 Gray platelet syndrome, 251 Growth factors, 21t

G6PD deficiency. See Glucose-6-phosphate dehydrogenase deficiency

Hageman, John, 230

Hageman factor (Factor XII), 236, 237

deficiency of, 262 Hairy cell leukemia, 207-208, 207f CD markers in, 208t

Ham's test, 106, 107f Handwashing, 7 Hct. See Hematocrit HE. See Hereditary elliptocytosis Heinz bodies, 17, 43, 45, 46f, 104f, 308 formation, 46f in G6PD deficiency, 103 Helmet cells. See Bite cells Hemarthrosis, 260, 260f Hematocrit (Hct), 22. See also Microhematocrit normal values, 300 Hematology, 4 automated data on, interpreting, 320 Coulter principle, 321, 321f flow cells, 322

hydrodynamic focusing, 321 instruments, 322-326 multiple polarized scatter separations, 322 optical scatter, 321 radiofrequency resistance, 321 VSC technology, 321 automated instruments in, knowledge necessary for operator of, 323 Hematopoiesis, 20f in acute myeloid leukemia, 162 definition of, 16

intramedullary vs. extramedullary, 17 Heme molecule, 52

Hemochromatosis. See Hereditary hemochromatosis Hemoglobin (Hgb), 22 abnormal, 54-55, 55f chromosomes specific to formation of, 53f electrophoresis, 119-120, 119f alkaline, points in analysis of, 120t embryonic, 16 function, 53-54 structure of, 52, 52f synthesis of, 52-53 iron in, 39 in thalassemias, 75 types of, 52-53

variants of, in US vs. worldwide, 121f Hemoglobin A (HgbA) gene states of, 76-77, 76f normal concentrations, by age, 119t postpartum, 16 Hemoglobin Barts (74), 76, 77 Hemoglobin C crystals, 308 Hemoglobin C disease, 120-121 Hemoglobin CC (HgbCC), 120f Hemoglobin D Punjab, 122 Hemoglobin E (HgE), 121 Hemoglobin F (HgbF) normal concentrations, by age, 119t in sickle cell anemia, 114-115, 117 Hemoglobin G phila, 122 Hemoglobin H disease, 76-77

peripheral smear in, 77f Hemoglobin O Arab, 122 Hemoglobin S (HgbS), 42 Hemoglobin S-beta thalassemia, 121

Hemoglobin SC (HgbSC), 120f, 121f haplotypes of, 114-115 solubility screen for, 118, 312, 313f Hemoglobinemia, 58 Hemoglobinopathies, 114 Hemolysis clinical events and, 55t extravascular, 56f intravascular, 57f laboratory evidence of, 56-57 physiology of, 57-58 types of, 55-56 Hemolytic uremic syndrome (HUS), 248

vs. thrombotic thrombocytopenic purpura, 249t Hemophilia A, factor VIII deficiency, 230 laboratory diagnosis of, 260 quality of life issues in, 261-262, 261t symptoms of, 258, 259 treatment for, 261 Hemophilia B, factor IX deficiency quality of life issues in, 261t Hemophilia C, factor X deficiency, 262 Hemorrhage. See Bleeding

Hemorrhagic telangiectasia. See Hereditary hemorrhagic telangiectasia Hemosiderin, 66 Hemostasis basis of, 230 fibrin in, 271-272, 272f fibrinogen in, 270 normal, 282

primary system of, 230-231 platelet aggregation, 234-235 platelet development, 232-235 platelet function/kinetics, 233-234 secondary system of, 231

coagulation factors, 235-237, 235-241, 236t common pathway, 238 complement system, 240 extrinsic pathway, 237-238 feedback inhibition, 239 fibrinolysis, 239-240 interrelationships between systems, 241 intrinsic pathway, 238 kinin system, 240 physiological coagulation, 237 thrombin formation, 238-239 vitamin K in, 263 Henoch-Schonlein anemia, 252 Heparin, 289

low-molecular-weight, 289 Heparin cofactor II deficiency of, 284 in thrombosis, 284 Heparin-induced thrombocytopenia (HIT), 287 diagnosis, 287-288 pathophysiology of, 287f Hepatosplenomegaly, 17

in leukemia, 160 Hepcidin, 71-72

342 Index

Hereditary elliptocytosis (HE), 100-101

variants of, 101t Hereditary hemochromatosis (HH), 72 laboratory diagnosis, 73 symptoms of, 73, 73t treatment, 73-74 Hereditary hemorrhagic telangiectasia, 252, 252f Hereditary pyropoikilocytosis, 101-102, 102f Hereditary spherocytosis (HS) clinical presentation, 98-100 genetics of, 98 laboratory diagnosis of, 100 pathophysiology of, 98 Hereditary stomatocytosis, 102 Hereditary xerocytosis, 102 Hermansky-Pudlak syndrome, 251 Herrick, James B., 117 Hewson, William, 230 Hexosaminidase A, 153 Hexose monophosphate shunt in RBC metabolism, 39 Hgb. See Hemoglobin HH. See Hereditary hemochromatosis High-molecular-weight kininogen (HMWK), 235 Hippocrates, 230 Histiocytes in peritoneal fluid, 316f HIT. See Heparin-induced thrombocytopenia HIV-AIDS

effects on hematology parameters, 151 Hodgkin's lymphoma, 206, 208-209 Homocysteine, 286

Howell-Jolly bodies, 17, 43, 45, 45f, 308

in megaloblastic anemias, 88f Human ehrlichiosis, 148, 148f Humoral immunity, 19 Hypercoagulability, 282 Hypercoagulable states conditions requiring evaluation for, 288t laboratory screening tests for, 288t Hyperhomocysteinemia, 286 Hyperviscosity syndrome, 252 Hypochromia, 39, 41, 41f, 308 Hypofibrinogenemia, 270

IDA. See Iron-deficiency anemia (IDA) Idiopathic thrombocytopenic purpura (ITP), 247

acute vs. chronic, 247f Immunity cellular, 19 humoral, 19 Immunoglobulins basic structure, 209f IgM, 214

serum protein electrophoresis patterns, 210f type and activity range of, 209t Immunophenotype acute myeloid leukemia, 166-167, 166t lymphoblastic leukemia, 176-177

precursor B lymphoblastic leukemia, 176-177 precursor T lymphoblastic leukemia, 178-179 Inclusions, red cell, 17, 43, 45, 45t diseases matched to, 310t grading, 310t recording, 308 Infants/newborns jaundice in, 104-105 red blood cell values in, 25t WBC differential values, 138t, 307t Inflammation anemia of, 71-72 Intramedullary hematopoiesis, 17 Iron absorption, 66 enhancers of, 68t inhibitors of, 68t available recycled, vs. need, 69f deficiency, prevention/control recommendations, 71t foods high in, 68t forms of, 68t in hemoglobin formation, 39

intake, 66

overload anemias related to, 72-74 organs damaged by, 74f storage/recycling, 66, 68 Iron-chelating agents, 74

rotation of injection sites, 75f Iron cycle, 67f

Iron-deficiency anemia (IDA) causes of, 70-71 diagnostic tests, 70

stages of, matched to diagnostic signals, 69t causes of, 69t treatment, 71 Isoelectric focusing, 120 ITP. See Idiopathic thrombocytopenic purpura

Kasabach-Merritt syndrome, 252 Kidneys. See Renal disease

Kininogen, high-molecular-weight (HMWK), 235, 237 Kinin system, 240 Koilonychia, 69, 70f Kottman, 230

Labile factor. See Proaccelerin Lactate dehydrogenase (LDH), 58 LDH. See Lactate dehydrogenase Lee, Pearl, 74-75 Left shift definition of, 144 Leukemia. See also specific types acute of ambiguous lineage, 175 vs. chronic, 160, 160t clinical findings in, 163t cytochemical reactions in, 165t FAB classification, 166, 167t definition of, 160 early research on, 160-161 Leukocytes (WBCs), 133f agranular cell series, 133 bands, 132, 132f basophils, 133, 133f Coulter scatterplots of, 322f eosinophils, 132, 133f hereditary disorders of, 149-150 life span of, 130 lymphocytic series, 133-134 maturation stages, 130 metamyelocytes, 131-132, 132f myeloblasts, 130-131, 131f myelocytes, 131, 131f promyelocytes (progranulocyte), 131, 131f qualitative defects of, 145-146 quantitative changes in, 144

terminology related to, 144-145 segmented neutrophils, 132, 132f terminology of, 130, 146t toxic changes in, 146 toxic granulation in, 146f, 147, 147f toxic vacuolization in, 147, 147f Leukocytosis definition of, 144, 146 Leukoerythroblastic picture, 146

definition of, 144 Leukopoiesis, 130 Lipid storage diseases, 152-153 Liver disease, bleeding associated with, 263 in fetal hematopoiesis, 16 Loeliger, E.A., 230

LSC. See Lymphocytic committed cell Lungs in sickle cell disease, 116 Lupus anticoagulant (LA), 286-287

diagnostic criteria for, 287t Lymphatic system, 136

in lymphocyte development/differentiation, 134-135, 135f Lymph nodes in fetal hematopoiesis, 16 Lymphoblastic leukemia (B-LBL), 176 antigen expression in, 177f cytogenetic findings, 178 immunophenotype, 176-177 laboratory findings, 176 Lymphoblasts identification criteria, 133-134 morphology, vs. myeloblasts, 164, 164f Lymphocytes antigen markers, 134t development of immunocompetency and, 137 large, 134, 134f origins of, 134 reactive, 151f morphology of, vs. resting lymphocyte, 151t response to antigenic stimulation, 137

small, 134, 134f subpopulations of, 136, 136f Sysmex scatterplot of, 324f travel path of, 136-137 Lymphocytic committed cell (LSC), 19 Lymphocytosis reactive, 150-151 Lymphoid cell lineage, 160 Lymphoproliferative disorders, 206 overview of, 215t

Macrocytes polychromatophilic, 41; see also Reticulocytes.

shape of, pathologies associated with, 92f size of, 39 Macrocytic anemias categories of, 86 clinical features of, 88 erythropoiesis in, 86-87 hematological features of, 88-89 laboratory diagnosis of, 90 non-megaloblastic, 91-92 red cell precursors in, 86 treatment, 90-91 Macrocytic normochromic anemia, 24-25 May-Hegglin anomaly, 149 MCH. See Mean corpuscular Hgb MCHC. See Mean corpuscular Hgb content MCV See Mean corpuscular volume MDSs. See Myelodysplastic syndromes Mean corpuscular Hgb content (MCHC), 22, 25

normal values, 300 Mean corpuscular Hgb (MCH), 22, 25

normal values, 300 Mean corpuscular volume (MCV), 22 conditions related to shifts in, 25t in macrocytic anemias, 80f normal values, 300 Mediterranean fever, 78-79 Megakaryocytes, 232, 232f Megaloblastic anemias clinical features of, 88 erythropoiesis in, 86-87 hematological features of, 88-89 laboratory diagnosis of, 90 peripheral blood smear in, 89f pernicious anemia, 89 red cell precursors in, 86, 86f treatment, 90 M:E ratio. See Myeloid-erythroid ratio Mercurialis, 230 Metamyelocytes, 132f identification criteria, 131-132 Methemoglobin, 54, 55 Methemoglobin reductase pathway, 39 MHA. See Microangiopathic hemolytic anemia Microangiopathic hemolytic anemia (MHA), 248

after acute DIC, 275

344 Index

Microcytes, 39, 41

in thalassemia major, 80f Microcytic disorders differential diagnosis of, 80 Microcytic hypochromic anemia, 24 Microhematocrit, 298 interpretation, 299-300 procedure, 299

reagents and equipment for, 298 Microscope, 4 care of, 5-6 parts of, 4-5, 5f Microscopy innovations in, 6 light, corrective actions in, 6 Miller eye disc, 303, 303f Minot, George, 89

Modified Westergren sedimentation rate, 300 Monoclonal gammopathy, 211 Monocytes, 133f identification criteria, 133 increase in, conditions associated with, 144 in phagocytosis, 145 Sysmex scatterplot of, 324f Morawitz, Paul, 230 Multiple myeloma, 210

chromosomal aberrations in, 210t disturbed platelet function in, 251, 252 laboratory findings in, 212t pathophysiology of, 210-212 prognosis/treatment, 212-213 symptoms/screening for, 212 Murphy, William, 89 Myeloblasts, 131f identification criteria, 130-131 morphology, vs. lymphoblasts, 164, 164f Myelocytes, 131f identification criteria, 131 Myelodysplasia acute myeloid leukemia with, 170 Myelodysplastic syndromes (MDSs), 164, 220 dysplastic changes in, 222t FAB classification of, 222t factors indicating progression to leukemia in, 223t pathophysiology of, 220

prognostic factors/clinical management, 222-223 recognizing, 220-221 therapy-related, 170 unclassifiable, 222

WHO classification of, 221-222, 222t Myelofibrosis with myeloid metaplasia (MMM), 194 clinical features/symptoms, 195 diagnosis, 195 diagnostic criteria for, 196t key facts of, 194t pathophysiology of, 194-195 peripheral blood/bone marrow findings, 195 teardrop RBCs in, 195f treatment, 195-196 Myeloid cell lineage, 160 Myeloid-erythroid ratio (M:E ratio), 18, 21, 130

Myeloperoxidase, 165 Myelosuppressive therapy in CML, 191

Naegeli, 161

Naphthol AS-D chloroacetate esterase, 165 Neonatal jaundice, 104-105 Neumann, Ernst, 161 Neutrophilia, 146

definition of, 144 Neutrophils alterations in, in peripheral smears, 147t hypersegmentation of, 148, 148f increase in, conditions associated with, 144 maturation spectrum, in CML, 190f in phagocytosis, 145 segmented bacteria in, 153f identification criteria, 132, 132f toxic granulation in, 146f, 147, 308 Niemann-Pick cell, 152f Niemann-Pick disease, 152 NK cells, 137

Non-Hodgkin's lymphoma, 206, 209 Normoblastic erythropoiesis, 86f Normoblasts polychromatic, 86f Normocytic normochromic anemia, 24 NSAIDs platelet function and, 246-247 Nucleus:cytoplasm ratio (N:C ratio), 34 in leukocytes, 130 red cell maturation and, 35 Nygaard, 230

OD curve. See Oxygen dissociation curve Opsonization in white cell phagocytosis, 145 Orthochromic normoblast, 36f Orthochromic normoblast-nucleated (nRBC), 36 Osler-Weber-Rendu disease, 252 Osmotic fragility curve, 100f Ovalocytes, 42-43, 307 Ovalocytosis, Southeast Asian, 101, 101f Overwhelming postsplenectomy infections (OPSIs), 17 Owren, Paul, 230

Oxygen dissociation curve (OD curve), 53-54, 54f

Pappenheimer bodies, 17, 45, 72f, 308 Paroxysmal cold hemoglobinuria (PCH), 107-108 Paroxysmal nocturnal hemoglobinuria (PNH), 106-107 Pauling, Linus, 114 Pavlosky, 230

PCH. See Paroxysmal cold hemoglobinuria Pelger-Huet anomaly, 149, 149f

Pentose-phosphate shunt, 40f Peritoneal fluid cell count and differential, 313-315 histiocyte in, 316f Pernicious anemia, 89

Personal protective equipment (PPE), 6-7, 8t Phagocytosis, white cell essential elements leading to, 147 t mechanism of, 145, 145f Philadelphia chromosome in CML, 189, 191, 192 Pica, 69

PK deficiency. See Pyruvate kinase deficiency Plasma cell leukemia, 214, 214f Plasma cells, 210f, 211 with inclusion, 211f sheets of, 211f structure/function of, 209-210 Plasmin, 239, 272, 273 in disseminated intravascular coagulation, 275 inhibitors of, 283 Plasminogen activator inhibitor 1 (PAI-1), 272-273 Plasminogen activator system, 239 Platelet(s) abnormalities in myelodysplastic syndromes, 220 in pathogenesis of thrombosis, 282 aggregation, 234f principle of, 234-235 Coulter scatterplots of, 322f count, 22

estimate, from peripheral smear, 306t Unopette system for, 309-311 normal values in, 312 development of, 232

dysfunction of, due to vascular disorders, 252 function acquired defects in, 251-252 drugs affecting, 252t kinetics and, 233-234 zones of, 233t giant, 221f abnormal granulation of, 221f inherited qualitative disorders of adhesion disorders, 249-251 release defects, 251 quantitative disorders of, 246-249 response of, to vascular injury, 232f satellitism, 153-154, 153f formation, 154f structure of, 232-233, 233f Pleural fluid cell count and differential, 313-315 mesothelial cell in, 316f PLL. See Prolymphocytic leukemia PNH. See Paroxysmal nocturnal hemoglobinuria Poikilocytosis, 39, 307-308 Polychromasia, 18, 41, 308 Polychromatophilic macrocytes, 41f Polychromatophilic normoblasts, 36, 36f Polycythemia vera (PV), 192

clinical features/symptoms, 192 diagnosis, 193 diagnostic criteria for, 194t increased RBCs in, 193f key facts of, 192t pathophysiology of, 192t peripheral blood/bone marrow findings, 192-193 treatment, 193-194 Postanalytic variables, 10, 11t Preanalytic variables, 10, 10f thrombocytopenia related to, 246 Precursor B lymphoblastic leukemia (B-ALL), 176, 177f antigen expression in, 177f cytogenetic findings, 178 immunophenotype, 176-177 laboratory findings, 176 Precursor T lymphoblastic leukemia (T-ALL), 179f antigen expression in, 179f cytogenetic findings, 179 immunophenotype, 178-179 laboratory findings, 178 Prekallikrein, 235, 237 Priapism in sickle cell disease, 116-117 Proaccelerin (labile factor, Factor V), 235-236 Proconvertin (stable factor, Factor VII), 236, 237

deficiency of, 262 Prolymphocytes, 134 Prolymphocyte leukemia (PLL), 208 Promonocytes identification criteria, 133 Promyelocytes (progranulocyte), 131f abnormal, 169f identification criteria, 131 Protein C, 284f deficiency of, 284-285 pathway, 284f Protein S, 284

deficiency of, 285 Prothrombin (Factor II), 235 deficiency of, 262 mutation, 286 Prothrombin time (PT), 238, 270 automated procedure for, 315-318 procedure, 318f Protonormoblast, 35, 35f Punnett square, 114f Purpura, 252, 252f Pyknosis, 153-154

Pyropoikilocytosis. See Hereditary pyropoikilocytosis Pyruvate kinase (PK) deficiency, 105

Quality assurance indicators of, 9t monitoring, 9-10 normal (reference) values, 9-10 plans, basic concepts of, 8-9 monitoring, 7

346 Index

RA. See Refractory anemia Radioactive hazards, 8

RAEB. See Refractory anemia with excess blasts

RARS. See Refractory anemia with ringed sideroblasts

RBCs. See Red blood cells

RCDW See Red cell distribution width

RCM. See Red cell mass

Red blood cell indices. See also Mean corpuscular Hgb; Mean corpuscular Hgb content; Mean corpuscular volume calculating, 300 Red blood cells (RBCs), 37f abnormal morphologies, 39

clinical conditions matched to, 309t color variations, 41 qualitative grading of, 309t recording, 307-308 shape variations, 41-43 size variations, 39-41 bone marrow production of, 16 Coulter scatterplots of, 322f elliptocytes, 42-43 fragmented, 43 hypochromic, 41f, 70f dimorphism in, 72f inclusions, 17, 43, 45, 45t recording, 308 life span of, 17 macrocytic, 221f maturation, 34 key features of, 35t terminology, 35t metabolism, 38-39 microcytic, 66, 70f in microcytic anemias, 66 normal values, in newborn, 25t nucleated in chronic lymphocytic leukemia, 206f observing/recording, 307 ovalocytes, 42-43 polychromatophilic macrocyte, 41f in polycythemia vera, 193, 193f precursors in megaloblastic anemia, 86, 86f sickle cell, 42, 42f spherocytes, 42, 42f Red cell distribution width (RCDW), 22

value of, 25-26 Red cell mass (RCM)

in polycythemia vera, 192, 193 Red cell membrane, 37f cytoskeleton, 38 development and function, 37 disorders of role of spleen in, 98 lipid composition, 37-38 protein composition in lipid bilayers, 38 Reed-Sternberg cells, 208 Reference intervals, 9 Reflex testing, 10 Refractory anemia (RA), 221 Refractory anemia with excess blasts (RAEB), 222 Refractory anemia with multilineage dysplasia, 221-222

Refractory anemia with ringed sideroblasts (RARS), 221 Renal disease anemia associated with, 71 bleeding associated with, 263 disturbed platelet function in, 252-253 Reticulocyte count with automated instrumentation, 326 conditions associated with increase/decrease in, 304 manual, procedure, 302 with Miller eye disc procedure, 302 normal values, 304 value of, 26-27 Reticulocytes, 36, 36f definition of, 26 Reticuloendothelial system (RES), 17, 66 in leukemia, 160 in lipid storage diseases, 152 Retinopathy in sickle cell disease, 117 Ringed sideroblasts, 72, 72f Ristocetin co-factor assay, 250 Rouleaux, 212, 212f vs. agglutination, 213f Russell bodies, 211f

Safety precautions, 6-9 Scatterplots/scattergrams, 322, 322f Coulter of leukocytes, 322f of platelets, 322f of RBCs, 322f Sysmex, 324f, 325f Schilling, 161 Schilling test, 90, 91f Schistocytes, 43, 248f, 308

in microangiopathic hemolytic anemia after acute DIC, 275

Sedimentation rate, modified Westergren, 300

Sediplast ESR rack, 301f

Serine protease, effects of antithrombin on, 284f

Serine protease inhibitors, 240f

Serous fluids causes of abnormal cells in, 318t cell count and differential, 313-315 normal values of, 317t reference factors for calculations, 315t Sézary cells, 208f Sézary syndrome, 208 SH. See Hereditary spherocytosis Sickle cell anemia clinical considerations, 115-117 genetics/incidence of, 114-115 laboratory diagnosis, 117-120 management, 117, 118t pathophysiology of sickling process, 115 prognosis, 117 Sickle cell gene haplotypes of, 114-115 Sickle cells, 42, 42f, 118f, 308 Sickle cell screen, 309-311

Sickle cell trait, 120 Sickle solubility test, 119f Sideroblastic anemias, 72-74 Sideroblasts ringed, 220, 222f refractory anemia with, 221 Siderotic granules, 45, 45f Smears wedge-type preparation of, 304, 305f three zones of, 306f zigzag method for differential, 306f Southeast Asian ovalocytosis, 101f Spectrin, 38

Spherocytes, 42, 42f, 98f, 308 formation mechanisms, 99f treatment/management of, 100 Spherocytosis. See Hereditary spherocytosis Spleen in fetal hematopoiesis, 16 functions of, 17, 18t role in red cell membrane disorders, 98 in sickle cell disease, 116 in thrombocytopenia, 246 Splenectomy in hereditary spherocytosis, 100 Howell-Jolly bodies observed following, 43, 45 overwhelming infections following, 17 in thalassemia major, 79 Stable factor. See Proconvertin Standard deviation, 9 Statistical quality control, 9-11 Stem cells, 18-19 Stomatocytes, 102f, 308 Stomatocytosis. See Hereditary stomatocytosis Streptokinase, 272, 290 Stroke in sickle cell disease, 117 Stuart-Prower factor (Factor X), 236

deficiency of, 262 Sudan black B, 165 Sugar water test, 106 Sulfhemoglobin, 54, 55 Synovial fluid causes of abnormal cells in, 318t cell count and differential, 313-315 normal values of, 317t reference factors for calculations, 315t Sysmex instrumentation, 324f

T-ALL. See Precursor T lymphoblastic leukemia Target cells, 42-43, 44f, 308

formation of, 44f Tay-Sachs disease, 152, 153 T-cell lymphomas, 208 T cells, 19, 136, 137

development, CD markers in, 179f in lymphoproliferative disorders, 206 T cytotoxic/suppressor cells (CD8), 137 in HIV disease, 151

Teardrop cells, 308

in myelofibrosis with myeloid metaplasia, 195f Telangiectasia, 252, 252f Terminal deoxynucleotidyl transferase, 165-166 TFPI. See Tissue factor pathway inhibitor Thalassemia gene alpha, 75 beta, 75

expression in population, 76t lineage of, from Queen Victoria, 259f Thalassemia syndromes, 74-75, 75f alpha, 76-77

clinical states of, 76f beta major, 78

treatment/management of, 79 beta thalassemia trait, 78-79 diagnosis, 76 diagnostic clues for, 80t microcytic cells in, 39-40 minor microcytic hypochromic blood smear, 80f pathophysiology of, 75-76 RBC morphology in, 39 thalassemia intermedia, 78 treatment, 76 Thalidomide, 213 T helper cells (CD4), 137

in HIV disease, 151 Thrombin activity on fibrinogen, 271f role in hemostasis, 271-272, 272f Thrombocythemia, 196t. See Essential thrombocythemia Thrombocytopenia drug-induced, 246-247 related to altered platelet distribution, 246 related to decreased platelet production, 246 related to sample integrity/ preanalytic variables, 246 Thrombocytopenia with absent radii (TAR), 251 Thrombocytosis, 249

relative, causes of, 197t Thrombolytic drugs, 289-290 Thrombolytic therapy, 272

Thromboplastin, plasma antecedent (Factor XI), 236 Thromboplastin, plasma component (Factor IX), 236 Thromboplastin (Factor III), 235 Thrombosis acquired, conditions associated with, 283t anticoagulant therapy in, 288-290 arterial vs. venous, 282 definition of, 282

inherited, conditions associated with, 283t pathogenesis of, 282

antithrombotic factors, 283-284 coagulation abnormalities, 283 fibrinolytic abnormalities, 283 platelet abnormalities, 282 vascular injury, 282 risk factors for, 283f Thrombotic disorders acquired, 286-288 inherited, 284-286 laboratory diagnosis, 288

How To Deal With Rosacea and Eczema

How To Deal With Rosacea and Eczema

Rosacea and Eczema are two skin conditions that are fairly commonly found throughout the world. Each of them is characterized by different features, and can be both discomfiting as well as result in undesirable appearance features. In a nutshell, theyre problems that many would want to deal with.

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