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201 Cards in this Set

  • Front
  • Back

Why should you evaluate red cell morphology?

Can be an indicator of anemia or other disease states

What is the cause of hypochromia?

Decrease in hemoglobin concentration

What is the cause of polychromatophilia?

Residual RNA in red blood cells

What is anisocytosis?

Variation in size

What is poikilocytosis?

Variation in shape

What causes poikilocytosis?

Change in red cell membrane


Abnormal metabolic state


Abnormal hemoglobin


Abnormal microenvironment


RBC age

In what condition will you see ovalocytes?

Megaloblastic anemias

In what condition will you see elliptocytes?

Iron deficiency anemia


Megaloblastic anemia


Hereditary elliptocytosis

What causes red cells to become elliptocytes?

Cytoskeleton defects

In what conditions will you see spherocytes?

Hereditary spherocytosis


Immune hemolytic anemia

In what conditions will you see target cells?

Liver disease


Abnormal hemoglobin

What causes red cells to become stomatocytes?

Membrane defect due to increased sodium permeability

In what conditions will you see stomatocytes?

Hereditary stomatocytosis


Liver disease


Alcoholism


Electrolyte imbalance

What causes red cells to become acanthocytes?

Due to change in plasma lipids

In what conditions will you see acanthocytes?

Alcoholism


Malabsorption


Lipid metabolism disorders

In what conditions will you see echinocytes?

Uremia


Acute blood loss


Stomach cancer

What causes red cells to become schistocytes?

Physical damage to RBC membrane

In what conditions will teardrop cells be seen?

Pernicious anemia


Beta thalassemia


Tuberculosis

What causes red cells to become teardrop cells?

Presence of an inclusion



Inability to regain shape after squeezing through small spaces

What causes red cells to become pyropoikilocytosis?

Thermal damage


Heat sensitivity


Abnormal spectrin in membrane

In what conditions will you see pyropoikilocytes?

Pyropoikilocytosis

What causes red cells to become sickle cells?

Abnormal hemoglobin S

In what conditions will you see sickle cells?

Sickle cell anemia


Hgb SC disease


Hgb S thalassemia

What causes basophilic stippling in red cells?

Ribosomal aggregates

In what conditions will basophilic stippling be seen?

Lead poisoning


Alcoholism


Megaloblastic anemia

What causes Howell-Jolly bodies in red cells?

Nuclear remnants of DNA

What is the most common inclusion showing accelerated erythopoiesis?

Basophilic stippling

In what conditions will HJ bodies be seen?

Megaloblastic anemia


Hemolytic anemia


Splenectomy

What causes Pappenheimer bodies in red cells?

Iron deposits

In what conditions will Pappenheimer bodies be seen?

Sideroblastic anemia


Hemoglobinopathies


Splenectomy

What is the confirmatory test for the presence of Pappenheimer bodies?

Prussian blue stain

What stain is used to identify Heinz bodies?

Supravital stain

What causes Heinz bodies to form in red cells?

Denatured hemoglobin

In what conditions are Heinz bodies seen?

G6PD deficiency


Toxic poisoning

What causes Cabot rings to be formed in red cells?

Remnants of microtubules or mitotic spindle

In what conditions will Cabot rings be seen?

Megaloblastic anemia


Lead poisoning

What causes rouleaux?

High levels of plasma proteins or abnormal proteins

What type of stain is used to identify hemoglobin H?

Supravital stain

What is anemia?

Inability of blood to apply tissues with oxygen

What does hematemasis mean?

Vomiting blood

What does melena mean?

Black stool

What does hematochezia mean?

Red blood in feces

What does hemoptysis mean?

Coughing up blood

What does epistaxis mean?

Nosebleed

What are the non specific signs of anemia?

Fatigue


Nausea


Constipation


Diarrhea


Dyspnea


Severe pallor


Smooth tongue

What is the most common cause of anemia?

Iron deficiency

What is the primary function of iron?

Oxygen transport

What are the sites of iron in the body?

Hemoglobin


Bone marrow


Liver


Spleen

How much of iron is formed in hemoglobin?

2/3

What is the function of transferrin?

To bind and transport iron

What is the function of hemosiderin?

Store iron during RBC degradation

Where is hemosiderin found?

Macrophages

What is the most frequently requested test to aid in the diagnosis of IDA?

Iron assay

What is the principle of the iron assay?

Measures iron bound to transferrin

What is the principle of the TIBC assay?

Measures the amount of iron that could be bound to transferrin

What test is the indicator of a patient's iron storage levels?

Ferritin

What is the first test to become abnormal when iron stores are depleted?

Ferritin

What are the clinical manifestations of IDA?

Weakness


Koilonychia


Cheilitis


Glossitis


Pica

What are sideroblastic anemias?

Anemias of abnormal iron metabolism

What causes sideroblastic anemias?

Defective iron reutilization


Improper incorporation of iron

What conditions may result from defective iron utilization?

Hemosiderosis


Hemochromatosis


Hereditary sideroblastic anemia

What is hemosiderosis?

Accumulation of excess iron in macrophages

What are megaloblastic anemias?

Group of dissidents characterized by defective nuclear maturation due to impaired DNA synthesis

What are the clinical manifestations of megaloblastic anemias?

Weakness


Fatigue


Congestive heart failure


Jaundice


Pallor

What is the major cause of vitamin B12 deficiency?

Malabsorption of vitamin B12

What is the most common cause of folate deficiency?

Decreased dietary intake

Where does optimal absorption of iron occur?

Small intestine

Excess iron is stored in what forms?

As ferritin or hemosiderin

What is the principle of the transferrin saturation percentage?

Measurement of the maximum amount of iron that is bound in serum

Transferrin saturation below what percentage is indicative of iron deficiency?

<16%

What are the CBC results of IDA?

Decreased H&H


MCV 55-74 fL


MCH 14-26 pg


MCHC 22-31 g/dL


Increases RDW

How does the PBS of IDA appear?

Microcytic


Hypochromic

What is hemochromatosis?

Accumulation of excess iron in tissues due to increase absorption

Where does the excess iron deposit in hemosiderosis?

RES

Where does the excess iron deposit in hemochromatosis?

Liver


Pancreas


Spleen


Heart

What condition is associated with bronze diabetes?

Hemochromatosis

What is the treatment for IDA?

Iron supplements


Intravenous iron


Transfusion for severe cases

What is the treatment for sideroblastic anemias?

Therapeutic phlebotomy


Chelating agents


Discontinuation of medications

What is the cause of hereditary sideroblastic anemia?

Detective heme synthesis

What are porphyrias?

Accumulation of porphyrin due to defective heme synthesis

What is the result of porphyria?

Decreased hemoglobin production

What type of porphyria is characterized by accumulation in the liver?

Hepatic porphyria

What type of porphyria is characterized by accumulation in the bone marrow?

Erythropoietic porphyria

What effects does erythropoietic porphyria have?

Photosensitivity

What effects does hepatic porphyria have?

Neurological disorders

What are the iron studies results in IDA?

Iron: Decreased


Ferritin: Decreased


TIBC: Increased


Saturation: Decreased

What are the iron studies results in sideroblastic anemia?

Iron: Increased


Ferritin: Increased


TIBC: Normal/Decreased


Saturation: Increased

The average adult has a total body iron content of?

3500 mg

What percent of ingested iron is absorbed?

5-10%

The hypochromic anemias represent a related group of disorder with?

Quantitative defect in hemoglobin synthesis

Older patients often develop IDA because of?

GI bleed

Microcytosis of red cells is reflected by?

Decreased MCV

What is a common feature of sideroblastic anemias?

Ringed sideroblasts

What is the most sensitive assay to screen for hemochromatosis?

Transferrin saturation

When does iron deficiency occur?

Inadequate iron intake


Excess iron loss


Increase need for iron

What is the minimum daily requirement for iron?

1 mg/day

What characteristic cells are present in megaloblastic anemias?

Megaloblasts


Macro-ovalocytes

What biochemical finding causes the megaloblastic morphology?

Decrease in thymidine triphosphate synthesis

How does thymidine deficiency affect nuclear development?

Interferes with maturation, DNA replication, and cell division

What are the primary causes for the lack of thymidine?

Vitamin B12 deficiency


Folate deficiency

What is the CBC findings of megaloblastic anemias?

RBC: Decreased


HGB: Normal/decreased


MCV: 100-160 fL


MCH: Increased


MCHC: Normal

How will the PBS appear in megaloblastic anemia?

Macrocytic


Normochromic

What inclusions are seen in megaloblastic anemia?

HJ bodies


Basophilic stippling


Cabot rings


Nucleated RBC's


Hypersegmented neutrophils

How long does the vitamin B12 stores last?

3-6 years

Vitamin B12 is absorbed at which site in the body?

Ileum


Small intestine

What is the function of vitamin B12?

Cofactor of enzymatic reactions in DNA synthesis

What two proteins are required for vitamin B12 transport?

Intrinsic factor


Transcobalamin II

What tissue secretes intrinsic factor?

Parietal cells of the stomach

What is the main transport protein of vitamin B12 to the tissues?

Transcobalamin II

What is the function of intrinsic factor?

Binds to vitamin B12 to be absorbed by the intestines

What is the most common form of vitamin B12 malabsorption?

Pernicious anemia

What is pernicious anemia?

Defective cobalamin absorption due to lack of intrinsic factor

What causes pernicious anemia?

Gastric parietal cell atrophy

What are the clinical manifestations of pernicious anemia?

Glossitis


Loss of appetite


Megaloblastic madness

Folate is absorbed at what site in the body?

Small intestine

What is the function of folate?

Cofactor of enzymatic reaction in DNA synthesis

What is the purpose of the Schilling test?

Differentiates between vitamin B12 deficiency and pernicious anemia

What is the pathophysiology of megaloblastic anemias?

Defective DNA synthesis and abnormal nuclear maturation

What is aplastic anemia?

Bone marrow failure and loss of cellularity leading to pancytopenia

What is the most common cause of aplastic anemia?

Idiopathic or unknown

What is characteristic of drug induced aplastic anemia?

Toxicity is not related to dosage

How does higher doses of radiation contribute to aplastic anemia?

Complete loss of all hematopoietic cells

How does lower doses of radiation contribute to aplastic anemia?

Reversible pancytopenia

How does ionizing radiation affect bone marrow?

Disrupts chemical bonds by forming free radicals that causes breaks to DNA

What types of viral infections are associated with aplastic anemia?

Hepatitis


EBV


Cytomegalovirus


HIV-1

How can infections contribute to aplastic anemia?

Immune destruction of bone marrow

What are the clinical manifestations of aplastic anemia?

Fatigue


Dyspnea


Pallor


Infection

What are the hematological findings of aplastic anemia?

CBC: Pancytopenia


PBS: N/N


RETIC: Decreased


DIFF: Lymphocytosis


BM: Hypocellular

What is the congenital form of aplastic anemia?

Fanconi's anemia

What is the congenital form of pure red cell aplasia?

Diamond-Blackfan anemia

What is pure red cell aplasia?

Loss of bone marrow red cell precursors

What are the hematological findings of pure red cell aplasia?

PBS: N/N


RETIC: Decreased


DIFF: Normal WBC/PLT


BM: Absent RBC precursors


EPO: Increased

What are hemolytic anemias?

Conditions of increased RBC destruction with inadequate BM response

What tests reflect Increased RBC destruction?

Increased indirect bilirubin


Decreased haptoglobin

What test reflects Increased RBC production?

Increased retic

How does the Coomb's test reflect hemolysis?

Used to detect autoimmune hemolytic anemia

Which hemolytic anemias are due to membrane defects?

Hereditary spherocytosis


Hereditary elliptocytosis


Hereditary pyropoikilocytosis

What causes hereditary spherocytosis?

Spectrin deficiency

What are the clinical manifestations of hereditary spherocytosis?

Anemia


Jaundice


Splenomegaly


Skeletal abnormalities

What are the lab findings of hereditary spherocytosis?

Increased indirect bilirubin


Decreased haptoglobin


Decreased hemoglobin


PBS: N/N


MCHC: Increased

What specialized testing is used to help diagnosis hereditary spherocytosis?

Osmotic fragility test

What causes hereditary elliptocytosis?

Altered spectrin or protein 4.1 deficiency

What causes hereditary pyropoikilocytosis?

Quantitative decrease and structural abnormality of spectrin

What test is used to detect hereditary pyropoikilocytosis?

Thermal sensitivity

What causes hereditary stomatocytosis?

Alterations in permeability of the RBC membrane to cations

What effect does stomatocytosis have on the red cells?

Sodium and water moves into the cell while potassium moves out

What are the lab findings of stomatocytosis?

MCV: Increased


MCHC: Decreased


Increased osmotic fragility

What are hemoglobinopathies?

Condition of abnormal hemoglobin synthesis

Genes for beta, delta, and gamma globin chains are located on which chromosome?

Chromosome 11

Genes for alpha and zeta globin chains are located on which chromosome?

Chromosome 16

The majority of hemoglobinopathies involve which chain?

Beta chain substitutions

Hemoglobin S results from what beta chain substitution?

Valine replaces glutamic acid

Hemoglobin C results from what beta chain variant?

Lysine replaces glutamic acid

What are the normal hemoglobins?

Hgb A


Hgb A2


Hgb F

What assembly constitutes Hgb A?

2 alpha chains


2 beta chains

What assembly constitutes Hgb A2?

2 alpha chains


2 delta chains

What assembly constitutes Hgb F?

2 alpha chains


2 gamma chains

What cause red cells to sickle?

Decrease in oxygen, pH or dehydration

What is sickle cell disease?

Genetic disorder characterized by the production of Hgb S

What test are performed to diagnose sickle cell?

CBC/PBS


Retic


Sickle solubility test


Hgb electrophoresis

What screening test is used to detect Hgb S?

Sickle solubility test

Which hemoglobin will yield positive results for the solubility test?

Hgb SS


Hgb AS


Hgb Barts


Hgb S thalassemia

How do you differentiate Hgb A2/c on electrophoresis?

Use an acidic agar

What is the only clinically significant alpha chain variant for hemoglobin?

Hgb G - Philadelphia

Hgb G-Philadelphia results from what alpha chain variant?

Lysine replaces asparagine

What are unstable hemoglobins?

Variants in which substitutions or deletions have weakened the binding forces that maintain the molecular structure

What is the cause of thalassemia?

Decreased synthesis of globin chains

What are thalassemias?

Anemias of abnormal globin synthesis

What is beta thalassemia major?

Absence of beta chain production

What is beta thalassemia minor?

Reduced amount of beta chains

Beta thalassemia has increased levels of which hemoglobin?

Hgb F


Hgb A2

What causes Hgb Barts/alpha thalassemia?

Absence of alpha chains

What causes Hgb H/alpha thalassemia?

Only one functioning alpha gene

What assembly constitutes Hgb Barts?

4 gamma chains

What assembly constitutes Hgb H?

4 beta chains

What causes alpha thalassemia minor?

Defects in 2 of 4 alpha globin chains

What causes the silent carrier alpha thalassemia?

One defective alpha globin chain of four

What effect does Hgb Barts have?

Incapable of oxygen transport to tissues

What is the most common RBC enzymopathy associated with hemolysis?

G6PD deficiency

What is G6PD?

Enzyme necessary for completion of hexose monophosphate shunt

What is paroxysmal nocturnal hemoglobinuria?

Acquired hemolytic anemia associated with cellular membrane abnormalities

What causes PNH?

Lack of certain surface proteins that leads to increased sensitivity to lysis by complement

How does sleep induce hemolysis in PNH?

CO2 retention and decrease in pH leads to increased complement activation

What is the screening test for PNH?

Sucrose hemolysis test

What is the confirmatory test for PNH?

Ham's test

What is the principle of the sucrose hemolysis test?

Sucrose promotes complement binds to RBC leading to hemolysis

What is the principle of Ham's test?

Acidic pH causes complement activation and hemolysis of RBC

What is the most common cause of death for PNH?

Thromboembolism

What is the most commonly accepted method for measuring hemoglobin?

Conversion of hemoglobin to cyanmethemoglobin followed by spectrophotometry

What is the purpose of the MCV?

Used as an estimation of the average size of red cells

What is the purpose of the MCH?

Measure of hemoglobin content

What is the function of spectrin?

Major role in cell membrane integrity

Zeta globin chain mature to what form?

Alpha chains

Epsilon globin chains mature to what form?

Beta


Delta


Gamma

Where does the initial and final phases of heme synthesis occur?

Mitochondria

Where does the intermediate phase of heme synthesis occur?

Cytoplasm

What percentage of adult hemoglobin is Hgb A?

96-97%

What percentage of adult hemoglobin is Hgb A2?

2-3%

What percentage of adult hemoglobin is Hgb F?

<1%

What are the nonfunctional forms of hemoglobin?

Methemoglobin


Sulfhemoglobin


Carboxyhemoglobin

What effect does methemoglobin have?

Cyanosis