Alterations of Hematologic Function

Alterations of Hematologic Function
Chapter 20

Hematopoiesis

Anemia Reduction in the total number of erythrocytes in the circulating blood or in the quality or quantity of hemoglobin Impaired erythrocyte production Acute or chronic blood loss Increased erythrocyte destruction Combination of the above

RBCs in a Blood Vessel

Anemia Classifications Etiology Morphology
Based on MCV, MCH, and MCHC values Size Identified by terms that end in “-CYTIC Macrocytic, microcytic, normocytic Hemoglobin content Identified by terms that end in “-CHROMIC Normochromic and hypochromic

Anemia Anisocytosis Poikilocytosis
Red cells are present in various sizes Poikilocytosis Red cells are present in various shapes

Anemia Fatigue, weakness, dyspnea, and pallor
Physiologic manifestation Reduced oxygen-carrying capacity Variable symptoms based on severity and the ability for the body to compensate Classic anemia symptoms Fatigue, weakness, dyspnea, and pallor

Pallor and Iron Deficiency

Koilonychia

Glossitis

Macrocytic-Normochromic Anemias
Also termed megaloblastic anemias Characterized by defective DNA synthesis Caused by deficiencies in vitamin B12 or folate Coenzymes for nuclear maturation and the DNA synthesis pathway

Ribonucleic acid (RNA) processes occur at a normal rate Results in the unequal growth of the nucleus and cytoplasm

Pernicious anemia Caused by a lack of intrinsic factor from the gastric parietal cells Required for vitamin B12 absorption Results in vitamin B12 deficiency

Pernicious anemia Typical anemia symptoms Neurologic manifestations Nerve demyelination Absence of intrinsic factor Others Loss of appetite, abdominal pain, beefy red tongue (atrophic glossitis), icterus, and splenic enlargement

Pernicious anemia Treatment Parenteral or high oral doses of vitamin B12

Folate deficiency anemia Absorption of folate occurs in the upper small intestine Not dependent on any other factor Similar symptoms to pernicious anemia except neurologic manifestations generally not seen Treatment requires daily oral administration of folate

Microcytic-Hypochromic Anemias
Characterized by red cells that are abnormally small and contain reduced amounts of hemoglobin Related to: Disorders of iron metabolism Disorders of porphyrin and heme synthesis Disorders of globin synthesis

Iron deficiency anemia Most common type of anemia worldwide Nutritional iron deficiency Metabolic or functional deficiency Progression of iron deficiency causes: Brittle, thin, coarsely ridged, and spoon-shaped nails A red, sore, and painful tongue

Sideroblastic anemia Group of disorders characterized by anemia Altered mitochondrial metabolism causing ineffective iron uptake and resulting in dysfunctional hemoglobin synthesis Ringed sideroblasts within the bone marrow are diagnostic Sideroblasts are erythroblasts that contain iron granules that have not been synthesized into hemoglobin

Sideroblastic anemia Dimorphism Myelodysplastic syndrome Erythropoietic hemochromatosis

Normocytic-Normochromic Anemias
Characterized by red cells that are relatively normal in size and hemoglobin content but insufficient in number Table 20-2

Aplastic anemia Pancytopenia Pure red cell aplasia Fanconi anemia Posthemorrhagic anemia Acute blood loss from the vascular space

Hemolytic anemia Accelerated destruction of red blood cells Autoimmune hemolytic anemias Immunohemolytic anemia Warm antibody immunohemolytic anemia Drug-induced hemolytic anemia Cold agglutinin immunohemolytic anemia Cold hemolysin hemolytic anemia

Sickle cell anemia Anemia of chronic inflammation Mild to moderate anemia seen in: AIDS, rheumatoid arthritis, lupus erythematosus, hepatitis, renal failure, and malignancies

Anemia of chronic inflammation Pathologic mechanisms Decreased erythrocyte life span Ineffective bone marrow response to erythropoietin Altered iron metabolism

Myeloproliferative RBC Disorders
Polycythemia Overproduction of red blood cells Relative polycythemia Result of dehydration Fluid loss results in relative increases of red cell counts and Hgb and Hct values

Polycythemia* Absolute polycythemia Primary Secondary Familial
Abnormality of stem cells in the bone marrow Polycythemia vera (PV) Secondary Increase in erythropoietin as a normal response to chronic hypoxia or an inappropriate response to erythropoietin-secreting tumors Familial * Table 20-3

Alterations of Leukocyte Function
Quantitative disorders Increases or decreases in cell numbers Bone marrow disorders or premature destruction of cells Response to infectious microorganism invasion Qualitative disorders Disruption of cellular function

Quantitative Alterations of Leukocytes
Leukocytosis Leukocytosis is a normal protective physiologic response to physiologic stressors Leukopenia Leukopenia is not normal and not beneficial A low white count predisposes a patient to infections

Granulocytosis (Neutrophilia)
Neutrophilia is evident in the first stages of an infection or inflammation If the need for neutrophils increases beyond the supply, immature neutrophils (banded neutrophils) are released into the blood

Granulocytosis (Neutrophilia)
This premature release is detected in the manual WBC differential and is termed a shift to the left When the population returns to normal, it is termed a shift to the right

Neutropenia Reduction in circulating neutrophils Causes
Prolonged severe infection Decreased production Reduced survival Abnormal neutrophil distribution and sequestration

Granulocytopenia (Agranulocytosis)
Causes Interference with hematopoiesis Immune mechanisms Chemotherapy destruction Ionizing radiation

Eosinophilia Hypersensitivity reactions trigger the release of eosinophilic chemotactic factor of anaphylaxis from mast cells Increased in allergic disorders Increased in parasitic invasions

Eosinopenia Decrease in circulation numbers of eosinophils
Usually caused by migration of cells to inflammatory sites Other causes Surgery, shock, trauma, burns, or mental distress

Basophils Basophils account for only 0% to 1% of the circulating WBCs
Basophilia Response to inflammation and hypersensitivity reactions Basopenia Occurs in acute infections, hyperthyroidism, and long-term steroid therapy

Monocytes Monocytosis Monocytopenia Poor correlation with disease
Usually occurs with neutropenia in later stages of infections Monocytes are needed to phagocytize organisms and debris Monocytopenia Very little known about this condition

Lymphocytes Lymphocytosis Lymphocytopenia Acute viral infections
Epstein-Barr virus Lymphocytopenia Immune deficiencies, drug destruction, viral destruction

Infectious Mononucleosis
Acute, self-limiting infection of B-lymphocytes transmitted by saliva through personal contact Commonly caused by the Epstein-Barr virus (EBV)—85% B cells have an EBV receptor site Others viral agents resembling IM Cytomegalovirus (CMV), hepatitis, influenza, HIV

Symptoms: fever, sore throat, swollen cervical lymph nodes, increased lymphocyte count, and atypical (activated) lymphocytes Serious complications are infrequent (<5%) Splenic rupture is the most common cause of death

>50% lymphocytes and at least 10% atypical lymphocytes: CBC Diagnostic test Monospot qualitative test for heterophilic antibodies Treatment: symptomatic

Atypical Lymphocytes

Leukemias Malignant disorder of the blood and blood-forming organs
Excessive accumulation of leukemic cells Acute leukemia Presence of undifferentiated or immature cells, usually blast cells Chronic leukemia Predominant cell is mature but does not function normally

Leukemias Acute lymphocytic leukemia (ALL)
Acute myelogenous leukemia (AML) Chronic myelogenous leukemia (CML) Chronic lymphocytic leukemia (CLL)

Leukemias

Origins of Leukemias & Lymphomas

Leukemias Signs and symptoms of leukemia
Anemia, bleeding purpura, petechiae, ecchymosis, thrombosis, hemorrhage, DIC, infection, weight loss, bone pain, elevated uric acid, and liver, spleen, and lymph node enlargement

Philadelphia Chromosome

Gleevec (imatinib)

Lymphadenopathy Enlarged lymph nodes that become palpable and tender
Local lymphadenopathy Drainage of an inflammatory lesion located near the enlarged node General lymphadenopathy Occurs in the presence of malignant or nonmalignant disease

Lymphadenopathy

Malignant Lymphomas Malignant transformation of a lymphocyte and proliferation of lymphocytes, histiocytes, their precursors, and derivatives in lymphoid tissues Two major categories Hodgkin lymphoma Non-Hodgkin lymphomas

Hodgkin Lymphoma Characterized by the presence of Reed-Sternberg cells in the lymph nodes Reed-Sternberg cells are necessary for diagnosis, but they are not specific to Hodgkin lymphoma Classical Hodgkin lymphoma Nodular lymphocyte predominant Hodgkin lymphoma

Hodgkin Lymphoma Physical findings Symptoms Laboratory findings
Adenopathy, mediastinal mass, splenomegaly, and abdominal mass Symptoms Fever, weight loss, night sweats, pruritus Laboratory findings Thrombocytosis, leukocytosis, eosinophilia, elevated ESR, and elevated alkaline phosphatase Paraneoplastic syndromes

Hodgkin Lymphoma

Non-Hodgkin Lymphoma Generic term for a diverse group of lymphomas
The lymphomas can be differentiated based on etiology, unique features, and response to therapies

Non-Hodgkin Lymphoma Non-Hodgkin lymphomas are linked to chromosome translocations, viral and bacterial infections, environmental agents, immunodeficiencies, and autoimmune disorders

Non-Hodgkin Lymphoma Clonal expansion of B cells, T cells, and/or NK cells Changes in proto-oncogenes and tumor-suppressor genes contribute to cell immortality and thus an increase in malignant cells

Non-Hodgkin’s Lymphoma

Burkitt Lymphoma Most common type of non-Hodgkin lymphoma in children
Burkitt lymphoma is a very fast-growing tumor of the jaw and facial bones Epstein-Barr virus is found in nasopharyngeal secretions of patients

Burkitt Lymphoma

Multiple Myeloma (MM) Infiltrate bone marrow and aggregate into tumor masses in skeletal system Plasma Cells M-proteins Fig Hypercalcemia, renal failure, bone lesions

Multiple Myeloma (MM)

Bone Marrow Aspiration

Multiple Myleoma

Lymphoblastic Lymphoma (LL)
Clone of immature T cells that become malignant in the thymus Lymphadenopathy of the neck

Alterations in Splenic Function
Splenomegaly Hypersplenism Congestive splenomegaly Infiltrative splenomegaly

Disorders of Platelets
Thrombocytopenia Platelet count <150,000/mm3 <50,000/mm3—hemorrhage from minor trauma <15,000/mm3—spontaneous bleeding <10,000/mm3—severe bleeding

Thrombocytopenia Causes Hypersplenism, autoimmune disease, hypothermia, and viral or bacterial infections that cause disseminated intravascular coagulation (DIC), HIT

Immune thrombocytopenic purpura (ITP) IgG antibody that targets platelet glycoproteins Antibody-coated platelets are sequestered and removed from the circulation The acute form of ITP that often develops after a viral infection is one of the most common childhood bleeding disorders

Immune thrombocytopenic purpura (ITP) Manifestations Petechiae and purpura, progressing to major hemorrhage

Thrombotic thrombocytopenic purpura (TTP) A thrombotic microangiopathy Platelets aggregate, form microthrombi, and cause occlusion of arterioles and capillaries Chronic relapsing TTP Acute idiopathic TTP

Essential (primary) thrombocythemia Thrombocythemia is characterized by platelet counts >400,000/mm3 Myeloproliferative disorder of platelet precursor cells Megakaryocytes in the bone marrow are produced in excess Microvasculature thrombosis occurs

Alterations of Platelet Function
Qualitative alterations in platelet function demonstrate an increased bleeding time in the presence of a normal platelet count Platelet function disorders result from platelet membrane glycoprotein and von Willebrand factor deficiencies

Alterations of Platelet Function
Manifestations Petechiae, purpura, mucosal bleeding, gingival bleeding, and spontaneous bruising Disorders can be congenital or acquired

Alterations of Coagulation
Vitamin K deficiency Vitamin K is necessary for synthesis and regulation of prothrombin, the prothrombin factors (II, VII, IX, X), and proteins C and S (anticoagulants) Liver disease Liver disease causes a broad range of hemostasis disorders Defects in coagulation, fibrinolysis, and platelet number and function

Alterations in Coagulation
Hemophilia A -1/5000 -sex linked : Xp28 -loss of factor VIII -female : modest reduction…X inactivation (Lyon Hypothesis) Hemophilia B -loss of factor IX, Xp27…Christmas disease

Alterations in Coagulation
Intrinsic Pathway -initiated: negatively charged surface a. damaged endothelium b. surface contact with foreign substances c. PTT Extrinsic Pathway -initiated: activation tissue thromboplastin a. cell injury endothelium/others b.PT

Queen Victoria

Disseminated Intravascular Coagulation (DIC)
Complex, acquired disorder in which “clotting and hemorrhage simultaneously occur” DIC is the result of increased protease activity in the blood caused by unregulated release of thrombin with subsequent fibrin formation and accelerated fibrinolysis

Endothelial damage is the primary initiator of DIC

The amount of activated thrombin exceeds the body’s antithrombins and the thrombin does not remain localized The widespread thromboses created cause widespread infarction ischemia,, and organ hypoperfusion

By activating the fibrinolytic system (plasmin), the patient’s fibrin degradation product (FDP) and D-dimer levels will increase Because of the patient’s clinical state, the disorder has a high mortality rate Treatment is to remove the stimulus

Clinical signs and symptoms demonstrate wide variability Bleeding from venipuncture sites Bleeding from arterial lines Purpura, petechiae, and hematomas Symmetric cyanosis of the fingers and toes

Alterations of Hematologic Function

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