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Lecture 17

Introduction to Benign & Malignant WBC Disorders

What is the Nuclear:Cytoplasmic ratio (N:C) of myeloblasts? 4:1
What is the Nuclear:Cytoplasmic ratio (N:C) of promyelocytes? 3:1
What is the Nuclear:Cytoplasmic ratio (N:C) of myelocyte? 2:1 or 1:1
What is the Nuclear:Cytoplasmic ratio (N:C) of metamyelocyte? 1:1
What is the Nuclear:Cytoplasmic ratio (N:C) of band neutrophils? 1:1
What is the Nuclear:Cytoplasmic ratio (N:C) of segmented neutrophils? 1:1
What is the shape of the nucleus of a segmented neutrophil? 2-5 distinct nuclear lobes
During neutrophilic maturation, when do cytoplasmic granules first appear? Promyelocyte stage
What is the half life of neutrophils after leaving the peripheral blood? 6-7 hours
What WBC has 2-5 nuclear lobes with lilac cytoplasmic granules containing numerous enzymes for killing. This is an active phagocyte during inflammation and infections. Neutrophils
What WBC is characterized by dark purple cytoplasmic granules that obscures the segmented nucleus. The nucleus consists of 2 lobes. This cell relesases histamine, elastase, and brdaykinin when stimualted by IgE, C5a, and C3. Basophils
What is the life span of basophils? 2 weeks
What WBC contains orange-red refractile cytoplasmic granules with a 2-3 lobed nucleus. Stimulation by IgA, IgE, IgG and other compounds causes release of major basic protein, esoinophilic peroxidase, substance P, PAF, and cytokines. Eosinophils
What is the life span of esoinophils? Days
What WBC is characterzied to have abundant cytoplasm with vacuoles. Small red granules may be present. The nucleus is indented with reticular chromatin. Monocytes
What is the life span of platelets? 8-10 days
(T or F) Iron deficiency decreases the number of platelets. False. Iron deficiency increases platelets.
Definition of neutropenia < 1.5 x 10^9/L neutrophils
Definition of agranulocytosis <0.5 x 10^9/L neutrophils
A condition described as decreased neutrophils in the peripheral blood. This is transient and the total number of neutrophils is normal as is the neutrophil kinetics. Common causes include hemodynamic changes, viremia, hypersensitivity, and hemolysis. Increased margination or storage of neutrophils
At what level of neutrophils is the ability to fight infection compromised? <1.0 x 10^9/L
What is the inheritance pattern of cyclic neutropenia? Autosomal dominant
A condition characterized by 3-6 days of neutropenia occuring every 21-30 days in a periodic pattern. During periods of neutropenia the patient may develop fever and infections. Cyclic neutropenia
What are main mechanisms of drug-induced neutropenia? (1) general marrow depression (2) Immunologic mechanisms
How is peripheral destruction of granulocytes distinguished from marrow failure? Periperhal destruction of granulocytes has marrow with normal number of erythroid and megakaryocytic elements and will often show myeloid hyperplasia whereas marrow failure has a hypoplasia of marrow elements.
What are the initial symptoms of neutropenia? 1-3 days of malaise, chills, sore throat, fever, fatigability and weakenss.
Criteria for eosinopenia <0.04 x 10^9/L
What conditions cause eosinopenia? Acute stress (glucocorticoid or epinephrine stimulation), acute inflammation (interleukin-5), Cushing's syndrome, or corticosteroid administration
Criteria for basopenia <0.01 x 10^8/L
What conditions cause basopenia? Corticosteroid administraiton, stress, acute inflammation, and hyperthryoidism
Criteria for monocytopenia <0.2 x 10^9?L
What conditions cause monocytopenia? May be seen in the first few hours after giving predisone, but by 12 hours returns to normal.
Criteria for lymphocytopenia <1.5 x 10^9/L for adults; <3.0 x 10^9/L for children
What conditions cause lymphocytopenia? Corticosteroids, chemotherapy, irradiation, Hodgkin's disease, HIV infection, and chronic diseases (ie sarcoid, TB, lupus)
Definition of myeloid "left shift" Release into the peripheral blood of immature neutrophils (bands, metamyelocytes, myelocytes, and promyelocytes)
Criteria for lymphocytosis lymphocytes > 4.0 x 10^9/L in adults and >9.0 x 10^9/L in children
What is the appearance of atypical lymphocytes in infectious mononucleosis? Large lymphocytes, reactive with abundant basophilic cytoplasm. Some may appear almost blast-like.
What organism causes infectious mononuclesosis? Epstein-Barr virus infeciton of B lymphoctyes
A bacterial infection commonly found in children characterized by spells of uncontrollable coughing with characteristic bark-like sound. This is frequently accompanied by lymphocytosis usually 20.0-30.0 x 10^9/L of small mature lymphocytes. Pertussis or whooping cough
Diseases associated with atypical lymphocytes Toxoplasmosis, infectious mononucleosis, CMV, actue viral hepatitis, and human herpes virus 6.
Conditions associated with eosinophilia Parasitic infections, Scarlet fever, ruberculosis, brucellosis, leprosy, Loeffler's PIE (pulmonary infiltration with eosinophilia), and tropical pulmonary eosinophilic syndromes
Criteria for hypersegmentation of granulocytes More than 3 cells having 4 lobes or a single cell with 6 lobes foudn in the course of a 100 cell differential
What type of anemia is associated with hypersegmentation? Megaloblastic anemia
What is the inheritance pattern of Myeloperoxidase deficiency? Autosomal recessive
What is myeloperoxidase deficiency? Absence of myeloperoxidase enzyme in neutrophils and monocyte granules. MPO deficiency in most people has no clinical consequence.
A congential condition in which granulocyte nuceli fail to segment normally. In the homozygote state the nucleus is round. In the heterozygote state, most granulocytes have a bilobed nuclei. Pelger-Huet anomaly
Is there granulocyte impairment in Pelger-Huet anomaly? It is a benign condition with no clinical consequence.
What is toxic granulation? Found in severe inflammatory states. Toxic granules are azurophilic granules found in promyelocytes, metamyelocytes, bands, and segmented stages. This is due to impaired cytoplasmic maturation in an effort to generate large number of granulocytes.
Cellular components described as single or multiple blue cytoplasmic inclusions representing remnants of rough endoplasmic reticulum from earlier maturational stages. Dohle bodies
An inherited conditions characterized by large pale basophilic inclusions, giant platelets, and sometimes thrombocytopenia. This anomaly is usually benign but may be associated with bleeding (thrombocytopenia). May-Hegglin anomaly
What makes up the May-Hegglin bodies? Altered RNA
A condition associated with skin hypopigmentation and abnormally large leukocyte granules. Platelets function is abnormal along with impaired chemotaxis and phagocytosis of leukocytes. Chediak-Higashi syndrome
What is the inheritance of Chediak-Higashi syndrome? Autosomal recessive
A condition characterized by the absence of lysozymal enazymes necessary to break down mucopolysaccharides. Lymphocytes often have metachromatic granules surrounded by a clear zone. Alder-Reilly anomaly
What is the inheritance pattern of Alder-Reilly anomaly? Autosomal dominant
Created by: UVAPATH4