Hematology Exam 3

What RBC inclusion is composed of DNA and seen in post-splenectomy, megaloblastic anemias, or hemolytic anemias?

Howell-Jolly body

What RBC inclusion is composed of RNA and is associated with decreased RBC survival & erythroid hyperplastic marrow?

Reticulocyte/polychromasia

What RBC inclusion is composed of iron and is seen in sideroblastic anemia or thalassemia?

Pappenheimer body

What RBC inclusion is composed of precipitated hemoglobin and seen in G6PD deficiency using supravital stain?

Heinz body

What RBC inclusion is crystalized hemoglobin C and seen in Hemoglobin C disease?

C crystal

What is a ringed sideroblast composed of?

Iron

What RBC inclusion is composed of RNA aggregates and is seen in thalassemia or lead poisoning?

Basophilic stippling

What is a nucleated red blood cell composed of?

DNA

What is the formula for calculating hematocrit?

RBC x MCV/10

What affects the MCV?

Agglutination

Will an increased WBC count affect the RBC count?

Yes

Does lipemia affect the hemoglobin or hematocrit?

Hemoglobin

What is the formula for calculating the MCV?

Hct x 10/RBC

What is the formula for calculating the MCH?

Hgb x 10/RBC

What is the formula for calculating the MCHC?

Hgb x 100/Hct

What is the reference range for MCV?

80-100 fL

What is the reference range for MCH?

28-32 pg

What is the reference range for MCHC?

32-36% g/dl

What is the reference range for RDW?

11.5-14.5%

What is a macrocytic anemia we learned about?

Megaloblastic anemia

What is defective in megaloblastic anemia?

Defective DNA synthesis from B12 or folate; poor diet

What will the bone marrow show in megaloblastic anemia?

Asynchrony, karryohexis, giant metamyelocytes, & bands

What are the laboratory results for megaloblastic anemia?

Hypersegmented neutrophils, increased DBili, Bili, and LD, decreased retic, pancytopenia

How is megaloblastic anemia treated?

Vitamin therapy

What tapeworm is associated with megaloblastic anemia?

Diphyllobothrium latum

What are causes of folate deficient?

Alcoholism, poverty, pregnancy,

What do you lack in pernicious anemia?

Intrinsic factor

What is the function of intrinsic factor?

Aids in B12 absorption

What test confirms pernicious anemia?

Schilling test

What are macrocytic cells associated with other than megaloblastic anemia?

Liver disease, alcoholism, reticulocytosis,

What are causes of iron deficiency anemia?

Chronic blood loss, poor diet, malabsorption,

What are the laboratory results for iron deficiency anemia?

Decreased Fe, Ferritin, increased TIBC, increased retic only after therapy

What are the laboratory findings for anemia of chronic disease?

Decreased iron, TIBC, increased ferritin

What is the sequence of development in IDA?

1. Stores depleted
2. Iron deficient erythropoiesis
3. Hgb/hct depletion
4. Symptoms appear

What is the treatment for IDA?

Treat underlying cause, iron supplements (based on severity: oral, transfusion, injection)

What is hemosiderosis?

Increased iron in reticuloendothelial cells

What is hemochromatosis?

More severe than hemosiderosis, excess iron is stored in the liver

What is the treatment for hemochromatosis?

Phlebotomy or chelation therapy to deplete iron stores

What are some microcytic anemias?

Iron deficiency anemia, hemochromatosis, hemosiderosis thalassemia, anemia of chronic disease, lead poisoning, sideroblastic anemia

What RBC inclusion is seen in lead poisoning?

Coarse basophilic stippling

What happens in lead poisoning?

Lead blocks heme production at several spots resulting in low heme production and low hemoglobin

What is defective in sideroblastic anemia?

Abnormal heme metabolism is present

What is defective in beta thalassemia?

Beta chains are reduced or absent, alpha chains are increased

What is the most common descent for beta thalassemia?

Mediterranean, South East Asians, and African American

What are the laboratory results for beta thalassemia?

Target cells, basophilic stippling, increased A2 & F in hemoglobin electrophoresis

What is a 4 gene deletion called in alpha thalassemia?

Hydrops fetalis (alpha thalassemia major)

What is a 3 gene deletion in alpha thalassemia?

Hgb H (4 beta chains)

What is a 2 gene deletion in alpha thalassemia?

Alpha thalassemia minor

What is a single gene deletion in alpha thalassemia?

Silent carrier

What hemoglobins are decreased in alpha thalassemia?

Hgb A, A2, & F

What is it called to have 4 gamma chains present?

Hgb Barts

What are the laboratory results for alpha thalassemia?

Target cells, basophilic stippling

What is the most common hemolytic anemia?

Malaria

What is defective in hereditary spherocytosis?

Inherited defect in spectrin

What are the laboratory results for hereditary spherocytosis?

Spherocytes, polychromasia, increased LD and Bili, increased osmotic fragility with hemolysis starting at 0.65%

Where does hemolysis start in a normal osmotic fragility?

0.45%

What is paroxysmal nocturnal hemoglbinuria?

Clonal stem cell disorder with cells sensitive to complement lysis

What are the laboratory results for PNH?

Red urine, + urine hemosiderin, + sucrose, + Ham's test

What is the older screening test for PNH?

Sugar water test

What is the older confirmatory test for Paroxysmal Nocturnal Hemoglobinuria

Ham's test

What test distinguishes hereditary spherocytosis from warm autoimmune hemolytic anemia?

DAT

What are the elements that must be present for hemolysis to occur in the positive tube of the Ham's test?

Acid, complement, patient RBC lacking the DAF and HRF Proteins

What is the current, most accurate testing methodology for PNH?

Flow cytometry with CD55 & 59

What happens when taking oxidant drugs with G6PD deficiency?

Oxidant drugs cause hemoglobin to denature and precipitate

What are the laboratory results for G6PD deficiency?

Heinz Bodies seen with supravital stain, polychromasia, increased retic, anisocytosis

Why must G6PD testing be done after the hemolytic crisis has passed?

During hemolytic crisis the BM is producing new cells at an accelerated rate. Retics have more of the enzyme than mature cells

What is seen on a peripheral smear in warm autoimmune hemolytic anemia?

Spherocytes & polychromasia

Which anemia occurs after an infection with infectious mono or mycoplasma?

Cold autoimmune hemolytic anemia

What is seen on a peripheral smear in cold autoimmune hemolytic anemia?

Agglutination

What happens in pyruvate kinase deficiency?

Reduced ATP production results in alterations of the RBC membrane leading to cell dehydration (echinocytes)

What is the defect in sickle cell anemia?

Valine is substituted for glutamic acid on the sixth position of the Beta chain

What test is used in sickle cell but cannot differentiate from disease and trait?

Sickle solubility

What is the confirmatory test for sickle cell?

Hemoglobin electrophoresis

What is seen on peripheral smear in hemoglobin C disease?

Hgb C crystals, target cells

What is increased in hemochromatosis?

Iron absorption

What anemia is found in mononucleosis?

Cold agglutinin

What percent of transferrin is bound to iron?

30%

How is intravascular distinguished from extravascular hemolysis?

Urine hemosiderin

What other tests are utilized in the detection of hemolytic anemia?

Bilirubin, haptoglobin, urine hemosiderin

What is the retic value for anemias?

increased in hemolytic anemias, decreased in nutritional deficiency until needs are met then increased, normal or decreased in aplastic anemia

Which of the anemias are life threatening?

Alpha thalassemia major

How can IDA and thalassemia be differentiated?

Hemoglobin electrophoresis

How can Hgb A with Hgb S be confirmed?

Hemoglobin electrophoresis using citrate agar

How can IDA and anemia of chronic disease be differentiated?

Ferritin & TIBC

What will the bone marrow of anemia indicate?

Erythroid hyperplasia 1:1

What is the purpose of the schilling test?

Detects malabsorption of B12 found in pernicious anemia

What is the breakdown product of hemoglobin?

Urobilinogen

What is a product of heme synthesis?

One of the porphyrins: protoporphyrin, uroporphyrin, or coproporphyrin

Which thalassemia is more serious?

Alpha thalassemia major

What microscopic power is used to see BM megakaryocytes?

10x

What do karryohexis and asynchrony indicate?

Defective DNA synthesis

Which chemistry test are increased in hemolytic anemia?

Bili & LD

Which group of anemias has the most polychromasia?

Hemolytic anemias

Ringed sideroblasts on an iron stain indicate what?

Sideroblastic anemia

In aplastic anemia, what is the bone marrow fat to cell ratio?

90:10

If the wrong type of blood is transfused, what type of hemolysis is found?

Intravascular

What is the M:E ratio for erythroid hyperplasia?

1:1

How is the M:E ratio calculated?

Myeloid cells divided by erythroid cells

What is a normal M:E ratio?

3:1

What is the M:E ratio in anemia?

1:1