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Hematology LEC Trans 6 - Notes
Medical Technology (MD)
Our Lady of Fatima University
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Red Blood Cell Abnormalities
CLINICAL HEMATOLOGY 1
Instructor: Prof. Antonio C. Pascua, Jr., RMT, MSMT
Date: November 4, 2021
LEC 6
TRANS 6
2021 – 2022
1 st Semester HEMA LEC
OLFU
RMT 2023
Outline At the end of the session, the student must be able to learn: I. Red Blood Cells II. Variation of Red Cell in Size A. Normocytic Anemia B. Microcytic Anemia C. Macrocytic III. Variation in Red Cell Color IV. Variation in Red Cell Shape A. Poikilocytes Secondary to Developmental Macrocytosis A. Oval Macrocyte B. Poikilocytes Secondary to Membrane Abnormalities A. Acanthocytes B. Echinocytes C. codocytes D. Spherocytes E. Stomatocytes F. Elliptocytes C. Poikilocytes Secondary to Trauma-Injury A. Schistocytes B. Dacryocyte D. Poikilocytes Secondary to Abnormal Hb Content A. Drepanocyte E. Other Poikilocytes A. Blister Cells B. Degmacyte F. Reporting of Poikilocytes V. Red Blood Cell Morphology and Related Condition
I. RED BLOOD CELLS
❖ Red Blood Cells ➢ Non-nucleated, biconcave disc-like cell ➢ 6 to 8 microns in diameter ➢ N/N (Normocytic/Normochromic) ➢ Central area of Pallor (1/3): extra surface area, when expose to hypotonic solution, before they are lysed, they will first expand (But when there is a continuous exposure to hypotonic solution, it will be lysed) ❖ Nutritional Requirements ➢ CHON and Amino Acids ➢ Vitamin B12, Folic Acid, Vitamin B6 and Fe2+ ➢ Riboflavin, Pantothenic Acid, Nicotinic Acid ❖ Morphologic Abnormalities ➢ Physical features of the red cell (size, shape, and color of RBC) ❖ Variations of RBCs ➢ Size: Normally, 6 to 8um diameter ▪ Anisocyte (Anisocytosis) - Increased variation in cell size (larger or smaller than their usual size) - Prominent in severe anemias Normocyte: normal in size (6-8um) Microcyte: smaller (<6um) Macrocyte: larger (>8um) ▪ Use MCV (Mean Cell/Corpuscular Volume) - Measuring the volume of RBC in order to measure its individual size - The volume of the cell is directly proportional to its size ➢ Hb Content ▪ Normally, 1/3 palor area ▪ Anisochromia - Red cells that vary in color ➢ Shape ▪ Normally, discoid shape ▪ Poikilocytes (Poikilocytosis) - Red cells that vary in shape in blood, side from discoid
II. VARIATION IN RED CELL SIZE
❖ Microcytic: lesser than 80fL ❖ Normocytic: normal MCV 80 to 100fL ❖ Macrocytic: more than 100fL ❖ Normocyte ➢ Not all normal sized red cells are normal, some normal looking RBCs are associated with disease ➢ There are diseases that can give normal size of RBC such as AHA (Aplastic Anemia, Hemolytic Anemia, Acute Bleeding)
A. Normocytic Anemia
❖ Normocytic Anemia ➢ The problem is number of circulating RBCs and not the quality of red cell ➢ Quantity over quality ➢ Less RBC, less RBC transporting oxygen ❖ Aplastic Anemia ➢ Bone marrow does not produce enough number of cells ❖ Hemolytic ➢ Marrow was able to produce normal red cell but factors in the blood are destroying RBC ▪ E. Plasmodium falciparum ❖ Acute Bleeding: Loss of blood, number of circulating RBC is down
B. Microcytic Anemia
❖ Microcytic Anemia ➢ <80fL smaller than expected size ➢ Caused by a problem in hemoglobin production ➢ Diseases Associated: TICS – (hypochromic) ❖ Thalassemia ➢ Problem with globin chains, for example, instead of 4, they only have 3 or 2 which causes insufficient hemoglobin in red cell membrane ❖ Iron Deficiency Anemia ➢ Ferrous sulfate (FeSO 4 ) is necessary in formation of heme ➢ Less iron will mean less heme, less hemoglobin resulting to microcytic RBC ❖ Chronic Anemia ➢ Any chronic disease such as inflammation, or damage to tissue ➢ Iron cannot enter cells because it is blocked by the diseases such as inflammation ❖ Sideroblastic Anemia ➢ Sideroblast (immature cell that contain iron deposits) ➢ Contains iron deposits inside but cannot be processed as it does not have protoporphyrin IX to form a heme
C. Macrocytic
❖ Macrocytic ➢ >80%fL ➢ Larger because red cells did not mature properly because of insufficient nutrients ➢ Observed when there are problems in liver or megaloblastic stage (Vitamin 12 and Folic Acid) ❖ Macrocytosis ➢ The result of a defect in either nuclear maturation or stimulated erythropoiesis ➢ True Macrocytes represent a nuclear maturation defect associated with a deficiency of either vitamin B 12 or folate ➢ result from a disruption of the regular mitotic division in the bone marrow, and this defect, the cells appear as mature, enlarged erythrocytes in the circulating blood ➢ The other type of macrocytosis is caused by increased erythropoietin stimulation, which increases the synthesis of hemoglobin in developing cells ➢ This disorder causes a premature release of reticulocytes into the blood circulation
➢ These cells not only will appear to be macrocytic but also may be basophilic or slightly hypochromic on a peripheral smear ❖ Microcytosis ➢ Associated with a decrease in hemoglobin synthesis ➢ The decrease in hemoglobin content may be produced by a deficiency of iron, an impaired globulin synthesis, or a mitochondrial abnormality affecting the synthesis of the heme unit of the hemoglobin molecule ➢ Disorders in which microcytosis may occur include malabsorption syndrome, iron deficiency anemia, and in the case of variant hemoglobin types, hemoglobinopathies
III. VARIATION IN RED CELL COLOR
❖ MCH or MCHC: identification in color ➢ MCH (Mean Cell/Corpuscular Hemoglobin) ▪ Average weight of hemoglobin ▪ Normal Value: 27 to 32 picogram ➢ MCHC (Mean Cell/Corpuscular Hemoglobin Concentration) ▪ Actual concentration of hemoglobin ▪ Normal Value: 32 to 36%, when this exceeds the normal value, there may be lysis or abnormal red cells in blood ❖ Normochromic ➢ Normal erythrocyte has a moderately pinkish-red appearance with a lighter-colored center when stained with a conventional blood stain ➢ Lighter Color ▪ Thinner portion of the cell does not normally exceed one third of the cell’s diameter and is referred to as the central pallor ➢ 1/3 pallor area ➢ Color of red cell is normal ➢ Normal hemoglobin content, normal size ➢ Normocytic – Normochromic ▪ Normal in size and normal in color ➢ Diseases Associated ▪ AHA: Normal appearance but the number of circulating red cells are low – anemia ❖ Hypochromic/ Hyperchromia ➢ >1/3 palor area, paler appearance ➢ Inadequate iron stores result in a decrease in hemoglobin synthesis ➢ Deficient hemoglobin content expresses itself as inadequate coloration or a lack of the typical red color associated with an erythrocyte on a peripheral smear ➢ Lesser hemoglobin resulting to smaller size RBC ➢ Clinically associated with iron deficiency anemia ➢ Microcytic – Hypochromic red cell ➢ Disease Associated are: TICS
Table 1: Hypochromasia Grading
Grade Description 1+ Central pallor is 1/2 of cell diameter 2+ Central pallor is 2/3 of cell diameter 3+ Central pallor is 3/4 of cell diameter 4+ Thin rim of hemoglobin
❖ Hyperchromic ➢ <1/3 palor are (or in most cases, no more palor area) ➢ Susceptibility of RBC to lysis due to lack of pallor area, but it does not mean that RBCs contain excess Hgb ➢ Spherocytes ▪ Abnormal RBC that don’t contain pallor area which makes them susceptible to lysis ❖ Polychromasia ➢ Variation in hemoglobin content showing a slight blue tinge (wright stain), gray-blue and larger than normal ➢ Residual RNA ➢ Reticulocytes: appearance may indicate excess production of RBCs in the bone marrow, especially if there is anemia ➢ Increase production in bone marrow
Table 2: Polychromasia Grading (Smear) Grade % Polychromatic Red Cell Slight 1% 1+ 3% 2+ 5% 3+ 10% 4+ >11%
IV. VARIATION IN RED CELL SHAPE
❖ Poikilocytosis ➢ The general term for mature erythrocytes that have a shape other than the normal round, biconcave appearance on a stained blood smear or variations ❖ Poikilocytes ➢ Assume many shapes but frequently resemble common objects such as eggs, pencils and teardrops ➢ Specific names have been given to many of these shapes
A. Poikilocytes Secondary to Developmental Macrocytosis
A. Oval Macrocyte
❖ Have an oval or egg-like appearance ❖ Cells are similar in appearance to elliptocytes, megalocytes are macrocytic and have a fuller and rounder appearance ❖ Elongated and bigger than normal ❖ Occur when MCV value reaches 125fL (normal value is 80 – 100 ) ❖ Bipolar arrangement in hemoglobin, reduction in membrane cholesterol due to megaloblastic state which is due to deficiency in vitamins ❖ Pencil Cells or Oat Cells ➢ Thinner variant in ovalocytes ❖ Markedly increased MCV (<100) ❖ Megaloblastic erythropoiesis ❖ No central area of pallor ❖ Megaloblastic Anemia ➢ Abnormally in development of red cell because of deficiency in B12 and folic acid which causes nuclear maturation defects
B. Poikilocytes Secondary to Membrane Abnormalities
❖ Changes with lipids, proteins and carbohydrates (protein deficiency or excess lipids)
A. Acanthocytes (Spur Cells or Thorn Cells)
❖ Have multiple thorny, spike-like projections that are irregularly distributed around the cellular membrane and may vary in size ➢ Have 3 to 12 long spikes caused by abnormal ratio of Lecithin and Sphingomyelin (lipids) ❖ Lacks pallor area due of abnormal spicules ❖ Mostly associated with Abetalipoproteinemia ➢ Rare hereditary disorder and spur cell anemia ➢ Lacks LDL (bad cholesterol) that helps transport cholesterol from liver to tissue resulting to less cholesterol distributed
E. Stomatocyte
❖ Mouth Cell or Bowl-Shaped Cell ❖ Have a slit-like opening that resembles a mouth ➢ the slit-like opening is on one side of the cell ❖ Result from increase sodium ion and decreased potassium ion concentrations within the cytoplasm of the erythrocyte ❖ Caused by osmotic exchanges due to cation imbalances in red cells ❖ Increased permeability to sodium causing improper or imbalance distribution of hemoglobin in RBCs ➢ K+: Inside, normally more permeable to K+ ➢ Na+: Outside ❖ Associated with Cases Such As: ➢ Alcoholism ➢ Cirrhosis ➢ Glutathione deficiency ➢ Infectious mononucleosis ➢ Lead poisoning ➢ Malignancies ➢ Thalassemia minor ➢ Hereditary stomatocytosis ➢ Obstructive liver disease or any liver problem ❖ Rh Null Conditions ➢ Has presence of stomatocytes ➢ Does not have D antigen resulting to increased permeability of membrane to sodium
F. Elliptocytes
❖ Elongated and narrow ❖ Rod or Cigar Shaped RBCs, defects are on cytoskeleton on RBCs ➢ Membrane defect in which the membrane is radically affected and suffers a loss of integrity ❖ Defect is on protein band 4 resulting to elongation of cell membrane making cells susceptible to lysis ❖ Can be Encountered in Cases of: ➢ Elliptocytosis ➢ Iron deficiency of anemia ➢ Thalassemia ➢ Anemias associated with malignancy ➢ Hemoglobin (Hb) C disease ➢ Hemolytic anemias ➢ Pernicious anemia ➢ Sickle cell trait
C. Poikilocytes Secondary to Trauma – Injury
A. Schistocyte (Schizocyte)
❖ Are fragments of erythrocytes that are small and irregularly shaped ❖ These cells are produced as the result of the breaking apart of an erythrocyte ❖ Half the size of a normal erythrocyte and may have a deeper red appearance ❖ Form due to fragmentation produced by damage RBC ➢ Form because of presence of blood vessel injury or clot ➢ When the RBCs come in to contact with the fragmented clots, they will be damaged ❖ Fragmentation may be caused by: ➢ Clot (fibrin) ➢ Prosthetic heart valves ➢ Altered blood vessels ❖ Keratocytes ➢ Schistocytes with horn-like projections ➢ Can be encountered in disease such as: ▪ Microangiopathichemolytic Anemia (MAHA) ▪ Burns ▪ Disseminated intravascular coagulopathy (DIC)
B. Dacryocyte (Tear-Drop Cells)
❖ Usually smaller than normal erythrocytes ❖ Resemble tears ❖ Due to squeezing and fragmentation of RBCs whenever they pass the spleen ❖ Disease Associated: ➢ Splenomegaly ➢ Thalassemia ➢ Myeloid Dysplasia: Marrow produces abnormal or immature cells ➢ Homozygous beta-thalassemia ➢ Myeloproliferative syndromes ➢ Pernicious anemia ➢ Severe anemias
D. Poikilocytes Secondary to Abnormal Hb Content
A. Drepanocyte (Sickle Cells or Menisocytes
❖ Leaf-like/crescent-shape ❖ One of the ends of the cell must be pointed ❖ The membrane is smooth and the cell stains uniformly throughout ❖ Result from the gelation of polymerized deoxygenated Hb S ❖ Polymerization of Hb S is influenced by both lowered oxygen levels and decreased blood pH ❖ The influx of sodium ions and other metabolic changes produce an extremely increased level of intracellular calcium ions ❖ Form because of polymerization of deoxygenated abnormal hemoglobin (particularly Hemoglobin S) ❖ Sickling or Sickle Like Cells ➢ Precipitated hemoglobin S ❖ Associated with Diseases such as: ➢ Sickle cell anemia ➢ Hemoglobin S diseases ▪ Due to abnormal amino acids in globins
E. Other Poikilocytes
A. Blister Cell (Helmet Cells)
❖ Are usually the larger scooped out part of the cell that remains after the rupturing of a blister cell and are formed as a result of the physical process of fragmentation ❖ These cell fragments are formed in the spleen and intravascular fibrin clots ❖ Precursor cells before turning to Schistocytes, they will first be Blister cell ❖ Are erythrocytes containing one or more vacuoles that resemble a blister on the skin ❖ Has a significantly thinned area at the periphery or outer border of the cell membrane ❖ The vacuoles may rupture. If rupturing does occur, distorted cells (keratocytes) and cell fragment (schistocytes) are produced ❖ Alterations are found where there is damage to the membrane ❖ Increased numbers can be seen as the result of pulmonary emboli in sickle cell anemia and microangiopathic hemolytic anemia ❖ Associated with Microangiopathic hemolytic anemia (MAHA)
B. Degmacyte (Bite cells)
❖ Bitten peace of donut, removal of denatured hemoglobin by macrophages in spleen ❖ If hemoglobin is denatured, the inclusion bodies, Heinz bodies will be removed by macrophages in spleen. It will leave bite marks (permanent damage in red cells) ❖ Diseases Associated: ➢ G6PD ➢ Drug Induced Anemia ➢ Thalassemia
F. Reporting of Poikilocytes
❖ Inclusion ➢ Unwanted particular matters inside cytoplasm
Table 3: Reporting of Poikilocytes Slight <5% Moderate 5 to 15% Marked >15% Particular Variations Occasional <1% Few 1 to 5% Frequent 5 to 10% Many >10%
Hematology LEC Trans 6 - Notes
Course: Medical Technology (MD)
University: Our Lady of Fatima University
