HEMATOLOGY Flashcards
(149 cards)
1
Q
Blood cell production, maturation, and death occur in organs of the
A
reticuloendothelial system (RES)
2
Q
reticuloendo-thelial system (RES) includes:
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bone marrow, spleen, liver, thymus, lymph nodes
3
Q
Mesoblastic (yolk sac) phase begins at
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19 days gestation
4
Q
First cell to be produced
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Primitive nucleated erythroblast
5
Q
Embryonic hemoglobins includes:
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Portland
Gower I
Gower II
6
Q
Begins at 6 weeks gestation with production of mainly red blood cells, but also granulocytes, monocytes, and megakaryocytes.
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Hepatic (Liver) Phase
7
Q
Begins around the fifth month of gestation, with the bone marrow producing mainly granulocytes
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Myeloid/Medullary phase
8
Q
A normal ratio of M:E (myeloid:erythroid)
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3:1or 4:1
9
Q
Alpha- and gamma-globin chain production predominates at birth, forming
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Hgb F
10
Q
Myeloid/Medullary Phase hemoglobin present:
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Hgb F
Hgb A(1)
Hgb A.2
11
Q
In newborn, —- of bone marrow is active red marrow
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80-90%
12
Q
Young adult (age 20): — of bone marrow is active
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60%
13
Q
In young adult, hematopoiesis is confined to the proximal ends of large flat bone namely:
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bones
pelvis
sternum
14
Q
Older adult (age 55): percentage of active bone marrow
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40%
15
Q
Normocellular— Marrow percentage of hematopoietic cells
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30-70%
16
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Marrow has >70% hematopoietic cells.
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Hypercellular/hyperplastic
17
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Marrow has <30% hematopoietic cells
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Hypocellular/hypoplastic
18
Q
Marrow has few or no hematopoietic cells.
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Aplastic
19
Q
Differentiate into either B or T lymphocytes in response to cytokines/lymphokines/interleukins/ CSFs/growth factors
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Lymphoid
20
Q
Gives rise to the multipotential progenitor CFU-GEMM
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Myeloid
21
Q
Committed Progenitor Cell: CFU MEG
Growth Factors/ Interleukins: Thrombopoietin, GM-CSF
Mature cells:
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Thrombocytes
22
Q
Committed Progenitor Cell: CFU GM, CFU M
Growth Factors/ Interleukins: GM CSF, M-CSF, IL3
Mature cells:
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Monocytes
23
Q
Committed Progenitor Cell: CFU GM, CFU G
Growth Factors/ Interleukins: GM CSF, G CSF, IL3
Mature cells:
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neutrophils
24
Q
Committed Progenitor Cell: BFU E, CFU E
Growth Factors/ Interleukins: Erythropoietin, GM-CSF, IL-3
Mature cells:
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Erythrocytes
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Committed Progenitor Cell: CFU-Eo
Growth Factors/ Interleukins: GM CSF, IL-3, IL-5
Mature cells:
Eosinophils
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Committed Progenitor Cell: CFU-Ba
Growth Factors/ Interleukins: IL3, IL4
Mature cells:
BASOPHILS
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Primary lymphoid tissue
Bone Marrow
Thymus
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Secondary lymphoid tissue
lymph nodes
spleen
gut-associated tissue (Peyer's patches)
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WBC reference range (SI units)
4.0-11.0 x 10^9/L
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include neutrophils, eosinophils, and basophils
granulocytes
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the first to reach the tissues and phagocytize (destroy) bacteria
neutrophils
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lymphocyte that provide cellular immunity
T lymphocytes
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develop into plasma cells in the tissue and produce antibodies needed for humoral immunity
B lymphocytes
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destroy tumor cells and cells infected with viruses
NK lymphocytes
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modulate the allergic response caused by basophil degranulation
Eosinophil
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mediate immediate hypersensitivity reactions (type I, anaphylactic)
Basophils
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Blood cell production within the bone marrow
Medullary hematopoiesis
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Blood cell production outside the bone marrow
Extramedullary hematopoiesis
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Erythropoietin is produced mainly by the
kidneys
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Earliest RBC, size up to 20 |xm, with an N:C ratio of 8:1
1-3 nucleoli, nucleus has dark areas of DNA
Chromatin is fine and uniform, and stains intensely
Deep blue cytoplasm with no granules
Pronormoblast (rubriblast)
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Size up to 16 jjim with an N:C ratio of 6:1
Centrally located nucleus with 0-1 nucleoli
Chromatin is coarsening.
Cytoplasm is less blue but intensely basophilic (RNA).
Basophilic normoblast (prorubricyte)
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Size up to 12 (Jim with an N:C ratio of 4:1
Eccentric nucleus with no nucleoli
Chromatin shows significant clumping.
Begins to produce hemoglobin, resulting in gray-blue cytoplasm
Polychromatophilic normoblast (rubricyte)
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Size up to 10 fxm with an N:C ratio of 0.5:1
Eccentric nucleus with small, fully condensed (pyknotic) nucleus; no nucleoli
Pale blue to salmon cytoplasm
Hemoglobin synthesis decreases
Orthochromic normoblast (metarubricyte)
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Size up to 10 um
contains no nucleus but has mitochondria and ribosomes
Last stage to synthesize hemoglobin
Last stage in bone marrow before release to the blood
Reticulocyte
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Reticulocyte reference ranges are ------ for adults
0.5-1.5%
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Size range is 6-8 (Jim.
Round, biconcave discocyte
Salmon with central pallor (clearing in the center)
Mature erythrocyte
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Erythrocytes reference range for female:
4.0-5.4 X 10^12/L
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Erythrocytes reference range for male:
4.6-6.0 X 10^12/L
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Usually orthochromic normoblasts (metarubricyte) but can appear in any erythrocytic stage of maturation
Associated with thalassemia major, sickle cell anemia, and other hemolytic anemias, erythroleukemia, and myeloproliferative disorders
Nucleated RBCs (nRBCs, nucRBCs)
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Small, round DNA fragments (0.5-1.0 µm in diameter) usually one per cell, but can be multiple
d.Seen in sickle cell anemia, beta-thalassemia major, and other severe hemolytic anemias, megaloblastic anemia, alcoholism, post-splenectomy
Howell-Jolly bodies
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Multiple, tiny, fine, or coarse inclusions (ribosomal RNA remnants) evenly dispersed throughout the cell; "blueberry bagel" appearance
Seen in thalassemias, megaloblastic anemias, sideroblastic anemia, lead poisoning, and alcoholism
Basophilic stippling
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Small, irregular, dark-staining iron granules usually clumped together at periphery of the cell
Seen in sideroblastic anemia, hemoglobinopathies, thalassemia, megaloblastic anemia, myelodysplastic syndrome (RARS)
Pappenheimer bodies
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Thin, red-violet, single to multiple ringlike structures that may appear in loop or figure-eight shapes
Seen in megaloblastic anemia, myelodysplastic syndromes, lead poisoning
Cabot rings
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Condensed, intracellular, rod-shaped crystal
Seen in hemoglobin C or SC disease, but not in trait
Hemoglobin C crystals
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1-2 blunt, fingerlike projections extending from the cell membrane
Seen in hemoglobin SC disease
Washington monument
Hemoglobin SC crystals
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Multiple inclusions ranging in size from 0.3 to 2.0 µm
Invisible with Wright's stain; must use a supravital stain to visualize
Seen in G6PD deficiency, beta-thalassemia major, Hgb H disease, unstable hemoglobinopathies, drug-induced anemias
Heinz bodies
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Most common in the US; prevalent in infants, children, pregnancy, elderly with poor diets, and chronic blood loss
Iron deficiency anemia
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Laboratory: Microcytic/hypochromic anemia
low serum iron, ferritin, hemoglobin/hematocrit, and reticulocyte count
high RDW and TIBC
smear shows ovalocytes/pencil forms
Iron Deficiency Anemia
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Clinical symptoms: Fatigue, dizziness, pica, stomatitis, glossitis, koilonychia
Iron Deficiency Anemia
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Anemia due to inability to use available iron for hemoglobin production
Anemia of Chronic Disease
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Impaired release of storage iron associated with increased hepcidin levels
Anemia of Chronic Disease
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Laboratory:
Normocytic/normochromic or slightly microcytic/hypochromic anemia
increased ESR
normal to increased ferritin
low serum iron and TIBC
Anemia of Chronic disease
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Associated with persistent infections, chronic inflammatory disorders
Anemia of Chronic disease
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Caused by blocks in protoporphyrin pathway resulting in defective hemoglobin synthesis and iron overload
Sideroblastic anemia
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Excess iron accumulates in mitochondrial region of immature erythrocyte in bone marrow
Excess iron accumulates in mature erythrocyte in circulation
Pappenheimer bodies
Sideroblastic anemia
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Laboratory:
Microcytic/hypochromic anemia
increased ferritin and serum iron
decreased TIBC
Sideroblastic anemia
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Multiple blocks in protoporphyrin pathway affect heme synthesis
Seen mostly in children exposed to lead-based pain
Symptoms: Abdominal pain, muscle weakness, gum lead line
Lead poisoning
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Laboratory finding:
Normocytic/normochromic anemia with characteristic coarse basophilic stippling
Lead poisoning
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Group of inherited disorders with block in protoporphyrin pathway
Photosensitivity, abdominal pain, CNS disorders
Porphyrias
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Defective DNA synthesis causing abnormal nuclear maturation
Megaloblastic anemias
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Caused by vitamin B12 or folic acid deficiency
Megaloblastic anemia
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Laboratory: Pancytopenia, macrocytic/normochromic anemia with various inclusions
Megaloblastic anemia
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Anemia related to bone marrow failure causing pancytopenia
Aplastic anemia
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Laboratory:
Decreased hemoglobin/hematocrit reticulocytes;
normocytic/normochromic anemia
Aplastic anemia
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Hypoproliferative anemia caused by bone marrow replacement
Myelophthisic
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Laboratory:
Normocytic/normochromic anemia; leukoerythroblastic blood picture
Myelophthisic anemia
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Sudden loss of blood due to trauma or severe injury
Acute blood loss anemia
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Symptoms:
Hypovolemia, rapid pulse, low blood pressure, pallor
Blood loss anemia
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Initially normal reticulocyte count, hemoglobin/hematocrit; later drop in hemoglobin/hematocrit and RBC; reticulocytosis in 3-5 days
Acute blood less anemia
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Gradual, long-term loss of blood, often from gastrointestinal bleeding
Chronic blood loss anemia
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Initially normocytic/normochromic anemia; over time causes decrease in hemoglobin/hematocrit; gradual loss of iron causes microcytic/hypochromic anemia
Chronic blood loss
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Most common membrane defect; autosomal dominant; characterized by splenomegaly, variable degree of anemia, spherocytes on peripheral blood smear
Hereditary Spherocytosis
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Laboratory:
increased osmotic fragility
increased serum bilirubin
Hereditary spherocytosis
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Autosomal dominant; most persons asymptomatic; >25% ovalocytes on peripheral blood smear
Hereditary elliptocytosis
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Membrane defect caused by polarization of cholesterol
Hereditary elliptocytosis (ovalocytosis)
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Autosomal dominant; variable degree of anemia; up to 50% stomatocytes on blood smear
Hereditary stomatocytosis
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Membrane defect due to abnormal permeability to sodium and potassium
Hereditary stomatocytosis
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Autosomal recessive; mild anemia associated with steatorrhea, neurological and retinal abnormalities; 50-100% acanthocytes
Hereditary acanthocytosis (abetalipoproteinemia)
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Increased cholesterol:lecithin ratio in membrane
Hereditary acanthocytosis
90
Sex-linked enzyme defect; common deficiency in hexose monophosphate shunt
G6PD deficiency
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Autosomal recessive; common enzyme deficiency in Embden-Meyerhof pathway
Pyruvate kinase (PK) deficiency
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Lack of ATP causes impairment of cation pump and decreased erythrocyte deformability
Pyruvate kinase (PK) deficiency
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Acquired membrane defect with increased sensitivity for complement binding
Paroxysmal nocturnal hemoglobinuria (PNH)
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Characterized by: Pancytopenia, chronic intravascular hemolysis, hemoglobinuria, hemosiderinuria, low leukocyte alkaline phosphatase (LAP) score
Paroxysmal nocturnal hemoglobinuria (PNH)
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All cause normocytic/normochromic anemia due to defects extrinsic to the RBC, acquired disorders causing accelerated destruction with reticulocytosis
Hemolytic anemias due to extrinsic/immune defects
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RBCs coated with IgG and/or complement, leading to membrane loss and spherocytes
Warm autoimmune hemolytic anemia (WAIHA)
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60% idiopathic; others secondary to diseases altering immune response.
Warm autoimmune hemolytic anemia (WAIHA)
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Laboratory: Spherocytes, increased osmotic fragility, bilirubin, reticulocyte count; positive direct antiglobulin test (DAT)
Warm autoimmune hemolytic anemia (WAIHA)
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RBCs coated with IgM and complement at temperatures below 37°C
Cold autoimmune hemolytic anemia (CAIHA)
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Can be idiopathic or secondary to infections
Cold autoimmune hemolytic anemia (CAIHA)
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Laboratory: Seasonal symptoms, RBC clumping, increased MCHC, bilirubin, reticulocyte count; positive DAT
Cold autoimmune hemolytic anemia (CAIHA)
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IgG biphasic Donath-Landsteiner antibody fixes complement to RBCs in cold, leading to lysis when warmed
Paroxysmal cold hemoglobinuria (PCH)
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Can be idiopathic or secondary to viral infections
Paroxysmal cold hemoglobinuria (PCH)
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Laboratory: Variable anemia, increased bilirubin and plasma hemoglobin, decreased haptoglobin; positive DAT, Donath-Landsteiner test
Paroxysmal cold hemoglobinuria (PCH)
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Recipient antibodies to donor RBC antigens result in destruction
Hemolytic transfusion reaction
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ABO incompatibility causes immediate reaction with intravascular hemolysis.
Hemolytic transfusion reaction
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Laboratory: Positive DAT, increased plasma hemoglobin.
Hemolytic transfusion reaction
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Rh or ABO incompatibility causing severe anemia, positive DAT, high bilirubin.
Hemolytic disease of the newborn (HDN)
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Rh incompatibility treated with Rh immunoglobulin; ABO incompatibility leads to mild anemia, weakly positive DAT
Hemolytic disease of the newborn (HDN)
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Skin puncture collection site for under 1 year of age
lateral or medial plantar surface of the heel
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Collection site for skin puncture of children older than 1 year of age and in adults
Palmar surface of the distal portion of the third or fourth finger
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Capillary blood contains more glucose than venous blood
amount:
10-20 mg/dL
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Capillary blood contains more leukocytes than venous blood
Amount
up to 1000/uL
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Capillary blood has lower RBC count and hemoglobin value than venous blood
amount
5%
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Hematopoietically inactive bones .
Made up of adipocytes
The remaining 50% of the space occupied
Yellow Marrow
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Largest structure of the lymphoid system
Reticuloendothelial organ
Spleen
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Graveyard of old cells
Spleen
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Reference range for MCV
80-100 fL
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Increased in megaloblastic anemia, hemolytic anemia with reticulocytosis, liver disease, and normal newborn.
MCV
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Decreased in iron deficiency anemia, thalassemia, sideroblastic anemia, and lead poisoning
MCV
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Reference range for MCG
26-34 picograms
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indicator of the average weight of hemoglobin in individual RBCs.
MCH
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Increased in macrocytic anemia
MCH
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Indicator of the average/mean volume of erythrocytes (RBCs).
MCV
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Decreased in microcytic, hypochromic anemia
MCH
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Reference range for MCHC
32-37 g/dL
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Measure of the average concentration of hemoglobin in grams per deciliter
MCHC
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Normochromic RBCs
32-37 g/dL MCHC
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Hypochromic RBCs:
Lesser than (<) 32 g/dL
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RBC indices seen in iron deficiency and thalassemia.
Hypochromic RBCs
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Reference range for RDW
11.5-14.5%
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Increased proportional to the degree of anisocytosis (variation in size); coefficient of variation of the mean corpuscular volume
RDW
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Seen post-transfusion, post-treatment (e.g., iron, B12, or folic acid therapy), idiopathic sideroblastic anemia, in the presence of two concurrent deficiencies (iron and folic acid deficiencies)
High RDW
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Reference range for platelets
150-450 X 10^9/L (conventional units 150,000-450,000/^L)
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Most commonly used routine peripheral blood smear stain
Wright's stain
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basic dye, which stains acidic cellular components (DNA and RNA) blue
Methylene blue
137
an acidic dye, which stains basic components- red-orange
Eosin
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used in the staining process to fix the cells to the slide
Methanol fixative
139
phosphate buffer pH range
pH 6.4- 6.8
140
Contains potassium ferrocyanide, HCl, and a safranin counterstain
Prussian blue stain
141
Used to visualize iron granules in RBCs (siderotic iron granules), histiocytes, and urine epithelial cells
Prussian blue stain
142
Used to stain specific cellular components
Supravital monochrome stain
143
used to precipitate RNA in reticulocytes; measure of bone marrow erythropoiesis
New methylene blue
144
a counterstain is used to visualize Heinz bodies; clinical disorders associated with Heinz bodies include G6PD deficiency and other unstable hemoglobin disorders
Neutral red with brilliant cresyl green
145
Earliest RBC, size up to 20 um, with an N:C ratio of 8:1
Pronormoblast (nutriblast)
146
Chromatin is coarsening.in RBC maturation
Basophilic normoblast (prorubricyte)
147
RBC maturation stage:
Begins to produce hemoglobin, resulting in gray-blue cytoplasm
Polychromatophilic
148
Eccentric nucleus with small, fully condensed (pyknotic) nucleus; no nucleoli
Pale blue to salmon cytoplasm
Orthochromic normoblast (metarubricyte)
149
RBC maturation stage:
contains no nucleus but has mitochondria and ribosomes
Reticulocyte
