PATH2220 Study Guide - Final Guide: Volume Overload, Leukocytosis, B-Cell Chronic Lymphocytic Leukemia

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Haematology
What is Haematology?
Science/medicine of blood
Disorders of the blood/bone marrow
Blood transfusion
Blood:
Cells, water, proteins and electrolytes
Cells erythrocytes, leukocytes, thrombocytes
Water plasma
Average volume 5L
Erythrocytes:
Made in bone marrow through erythropoiesis
Small flexible biconcave disc shaped cells
No nucleus
Membrane made up of lipids and proteins
o Membrane must be deformable and stabile
o Lipid bilayer with embedded proteins
o Membrane proteins 50%
Peripheral membrane elasticity
Integral embedded with lipids
Carry haemoglobin (Hb) to tissues
Require iron, folate, vitamin B12 and EPO
Lifespan of 120 days
Cannot reproduce
No synthetic activities
Contains no organelles
Removed in reticulo-endothelial system
Haemoglobin
o Each red cell contains 640x106 molecules of Hb
o Hb haem attached to globin chains
o Haem made in mitochondria, contains Fe2+ broken down to
bilirubin and excreted by the liver
o Globin 4 polypeptide chains
HbA 22 98%
Defects in globin thalassaemia, sickle cell disease
Degraded down to amino acids and returned to body pool
Erythropoiesis
o Under influence of erythropoietin hormone produced in
kidneys
o Stages of development in bone marrow
Cell becomes smaller
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Nucleus matures chromatin condensation
Cytoplasm gains haemoglobin
Nucleus extruded to produce mature RBC
Anuclear biconcave disc with flexible membrane
o 200 billion red blood cells made each day
Requirements for erythropoiesis
o Erythropoietin (EPO)
Regulates red blood cell production
90% produced in kidneys
Stimulus to EPO production is renal O2 tension anaemia,
low atmosphere O2, defective cardiac/pulmonary function
o Iron
Integral part of haemoglobin
From diet 5-10% absorbed in upper small bowel
Transported to marrow bound to transferrin
Excess stored in macrophages as ferratin
Require 1-2mg/day
Varies by age and gender
o Vitamin B12
Required for nuclear maturation
From diet animal products
Absorbed in ileum requires instrinsic factor
Large stores last years
o Folate
From fruit, vegetables
Absorbed in upper small bowel
Small stores 3 months
Deficiencies result in macrocytic anaemia
Red cell metabolism
o Glucose is metabolized in RBCs by anaerobic glycolysis
o Embden-Meyerhof pathway
o Reduced RBC lifespan/increased rate of RBC destruction
haemolysis
Leukocytes:
Eosinophils
o 1-5% of leukocytes
o 2-lobed nucleus with large orange granules
o Circulation time 3-8 hours
o Involved in allergy, parasitic infection
Basophils
o <1% of white cells
o Deep purple-black granules overlie nucleus
o Function unknown
Monocytes
o 2-8% of WBC
o 20-40hrs in blood
o Mature to macrophage in tissues
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Document Summary

What is haematology: science/medicine of blood, disorders of the blood/bone marrow, blood transfusion. Blood: cells, water, proteins and electrolytes, cells erythrocytes, leukocytes, thrombocytes, water plasma, average volume 5l. Blood transfusions: blood products, packed red blood cells, platelets, fresh frozen plasma, immunoglobulins, blood donation, collection of blood, separation and blood grouping, screening for infectious agents. Indications: acute blood loss, symptomatic, bone marrow failure, thrombocytopenia, immune deficiencies, risks, wrong blood group, volume overload, fever, immune reaction, infection. Infectious mononucleosis (ebv: lymphocytes reactive to viral infection, altered lymphocyte morphology, monocytosis, uncommon, causes, chronic infection, tuberculosis, malaria, eosinophilia. Increased in allergic conditions asthma, eczema, hay- fever: parasitic infections, drug reactions, basophilia, very rare almost always malignant, chronic myeloid leukaemia. Immunophenotyping antigen expression by flow cytometry used to characterize: many genetic abnormalities, treatment chemotherapy, transplantation, chronic leukaemia, malignant proliferation of mature lymphoid or myeloid cells, chronic lymphocytic leukaemia.

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