Authors: Mary A. Williamson Mt(ascp) Phd,L. Michael Snyder Md
Hereditary Stomatocytosis
Hemolytic Extrinsic Red Blood Cell Defects
Autoimmune Hemolytic Anemias (AIHAS)
Paroxysmal Nocturnal Hemoglobinuria (PNH)
Paroxysmal Cold Hemoglobinuria (PCH)
Hemolytic Disease of the Newborn
Mechanical Hemolysis
Evans Syndrome
Erythrocytosis
Leukocytosis and Leukopenias
Neutropenia
Agranulocytosis
Lymphocytosis
Lymphocytopenia
Monocytosis
Eosinophilia
Persistent Eosinopenia
Basophilia
Leukemoid Reactions
B Lymphoblastic Leukemia/ Lymphoma (B-ALL)
Acute Myeloid Leukemia (AML)
T Lymphoblastic Leukemia/ Lymphoma (T-ALL)
Chronic Myelogenous Leukemia
Chronic Eosinophilic Leukemia (CEL) and Hypereosinophilic (HES) Syndrome
Chronic Lymphocytic Leukemia (CLL)/Small Lymphocytic Lymphoma (SLL)
Prolymphocytic (PLL) Leukemia of B- and T-Cell Subtype
Hairy Cell Leukemia
T-Cell Large Granular Lymphocytic Leukemia (T-LGL)
Chronic Neutrophilic Leukemia
Myeloproliferative Neoplasms (MPNS)
Chronic Myelogenous Leukemia (CML)
Polycythemia Vera (PV)
Essential Thrombocythemia (ET)
Primary Myelofibrosis (PMF)
Myelodysplastic Syndrome (MDS)
Chronic Myelomonocytic Leukemia (CMML)
Splenomegaly
Non-Hodgkin Lymphomas
Burkitt Lymphoma (BL)
Cutaneous T-Cell Lymphomas: Mycosis Fungoides (MF) and Sézary SYNDROME (SS)
Diffuse Large B-Cell Lymphoma (DLBCL)
Follicular Lymphoma (FL)
Mantle Cell Lymphoma (MCL)
Marginal Zone Lymphoma (MZL)
Posttransplant Lymphoproliferative Disorder (PTLD)
Lymphoplasmacytic Lymphoma (LPL)/Waldenström Macroglobulinemia (WM)
Hodgkin Lymphoma (HL)
Plasma Cell Myeloma
Monoclonal Gammopathy of Undetermined Significance (MGUS)
Plasma Cell Leukemia (PCL)
Monoclonal Light and Heavy Chain Deposition Diseases
Plasmacytoma
Immunoglobulin Light Chain Amyloidosis (ILCA)
Cryoglobulinemia
Cryofibrinogenemia
Disorders of Hemostasis and Thrombosis
Disorders of Platelets: Thrombocytopenias
Immune Thrombocytopenic Purpura (ITP)
Drug-Induced Thrombocytopenia, Immune
Heparin-Induced Thrombocytopenia (HIT)
Neonatal Thrombocytopenia
Pseudo (Spurious) Thrombocytopenia
Disorders of Platelet Function: Inherited and Acquired
Inherited Thrombocytopathies
Acquired Thrombocytopathies
Disorders Due to Coagulation Factor Deficiencies: Congenital Clotting Defects
Hemophilia
von Willebrand Disease (VWD)
Factor XII (F XII) Deficiency
Factor XI (F XI) Deficiency
Factor XIII (F XIII) Deficiency
Acquired Hemorrhagic Disorders of Multifactorial Etiology
Disseminated Intravascular Coagulation (DIC)
Hereditary Hemorrhagic Telangiectasia (HHT)
Hemostatic Failure in Cardiopulmonary Bypass Surgery
The Coagulopathy of Liver Disease
Anticoagulants, Circulating
Thrombophilia
Antiphospholipid Antibody Syndrome (APS)
Thrombotic Thrombocytopenic Purpura/Hemolytic Uremic Syndrome (TTP/HUS)
Miscellaneous Disorders
Iron Overload Disorders (IOD) and Hereditary Hemochromatosis (HH)
This Chapter covers blood diseases encompassing pathology of the formed elements of blood (red cells, white cells, and platelets), monoclonal plasmatic dyscrasias, hemorrhagic and thrombotic diseases, and lastly metabolic disorders that have a major impact on hematologic parameters.
RED BLOOD CELL DISORDERS
ANEMIAS
Definition
Anemia is a reduction in Hb leading to decrease in oxygen supply to peripheral tissues. Normal hemoglobin (Hb) range is established by population studies, but range should be adjusted for different age groups, especially for children, and levels are lower in women and in African Americans. There is some debate whether people of older ages have
physiologically
lower Hg levels. Most likely lower values reflect underlying pathology. Hb values are more accurate than hematocrit (Hct) values, because Hb is measured directly by automated analyzers, whereas the Hct is a calculated value.
Diagnosis
There are many ways to classify anemias, but the differential diagnosis of anemia can be narrowed by using the RBC size, as reflected in the MCV and the reticulocyte count. See Figure
9-1
.
In addition, insight into mechanism and etiology complements the differential diagnosis.
Onset of anemia has a great impact on symptoms and diagnosis.