Disease State: Erytrocytes- Anemia- Microcytic

What is a correct characteristic of Alpha Thalassemia?

Decreased or absent alpha chain production results in excess gamma chain production during and shortly after birth, followed by excess beta chains later.

What are the expected results of the laboratory markers in iron deficiency anemia?

Decreased serum iron, increased transferrin, decreased ferritin, decreased percent saturation, and increased total iron binding capacity.

All of the following are characteristic findings in patients with iron deficiency anemia EXCEPT?

Decreased total iron-binding capacity (TIBC)

If a patient has three inactive alpha globin genes, which of the four alpha (a) thalassemia classifications would he/she have?

HB h disease

Which abnormal hemoglobin may be formed in alpha thalassemia?

Hb Bart's

Which hemoglobin will be increased in delta-beta thalassemia?

Hb F

On this alkaline electrophoresis gel, which patient most likely has alpha thalassemia? Lanes 5 and 6 represent controls.

Patient 3

In which disorder may a mild anemia be present even with an increased red blood cell (RBC) count and normal adult hemoglobin electrophoresis?

Alpha thalassemia minor

Which alpha thalassemia shows no anemia or evidence of disease, although a slight decrease in the MCV and MCHC may be seen?

Alpha thalassemia silent carrier

A person with beta thalassemia intermedia might exhibit what clinical symptoms?

Patients with thalassemia intermedia vary in clinical symptoms based on genetic inheritance patterns.

Which genotype is associated with alpha thalassemia minor?

-a/-a

How is beta thalassemia defined?

A decrease in the rate of production of beta chains due to a partial or total deletion of loci from chromosome 11 that code for the beta chain.

The most common forms of hereditary sideroblastic anemia (SA) are due to which of the following?

A defective X-linked recessive gene.

Which of the following microcytic anemia's is the most common anemia other than Iron Deficiency Anemia (IDA) and accounts for more than one-third of anemia in hospitalized patients without blood loss?

Anemia of chronic disease/inflammation

What characteristics are most often associated with alpha thalassemia intermedia?

Anemia, splenomegaly

Anemia of chronic inflammation, also known as anemia of chronic disease, can be caused by all of the following mechanisms EXCEPT:

Decreased ferritin

Which of the following sets of results is indicative of stage 3 iron deficiency (iron deficiency anemia)?

Decreased ferritin, increased sTfR, increased TIBC

This drawing depicts which alpha chain genotype? GAMMA ALPHA 2 GAMMA

Alpha thalassemia intermedia (Hemoglobin H Disease)

Newborns who will develop Hemoglobin H disease may demonstrate up to 30% of which type of hemoglobin?

Bart's

Which of the following hemoglobins is replaced by hemoglobin H (HbH) after birth in individuals with HbH disease?

Bart's

Hemoglobin H (HbH) bodies are tetramers of which globin chain?

Beta

The diagram above represents which of the following beta thalassemias? E GAMMA Y ALPHA BETA E GAMMA Y ALPHA

Beta thalassemia intermedia

Codocytes (target cells) are an expected finding in all of the following phenotypes of beta thalassemia, EXCEPT?

Beta thalassemia minima

Which beta thalassemia shows no anemia and may not be detected using hemoglobin electrophoresis?

Beta thalassemia minima

A patient has an increased RBC count with a decreased Hb and normal Hct. The MCV is microcytic and the RDW is within normal limits. Many codocytes are present on the peripheral smear. Serum iron is 165 µg/dL (normal = 60 -150 µg/dL), and the TIBC is 230 µg/dL (normal = 250 - 400 µg/dL). Consider also the findings on alkaline hemoglobin electrophoresis, which show an increased level of hemoglobin A2 of 5% (normal values are less than 3.5%). What is this patient's most likely diagnosis?

Beta thalassemia minor

The genetic analysis of a patient suspected of having beta thalassemia resulted in the pattern shown. This drawing most likely depicts which beta chain genotype E GAMMA Y GAMMA A s b E GAMMA Y GAMMA A s half off b

Beta thalassemia minor

The decreased serum iron levels seen in anemia of chronic disease (also referred to as anemia of chronic infection or inflammation) is caused by which of the following?

Decreased iron released from macrophages

Which chromosome demonstrates a partial or full gene loci deletion in various forms of beta thalassemia?

Chromosome 11

This image is from a patient with alpha thalassemia. Though not diagnostic for this condition, what morphology is present which would lead you to consider a quantitative disorder of hemoglobin synthesis?

Codocytes

Beta thalassemia major, is also known as:

Cooley's anemia

Which anemia is formed due to the inability to incorporate iron into the protoporphyrin ring?

Sideroblastic Anemia

Which of the following genotypes cause beta thalassemia minor?

ß0/ß

Which disorder exhibits a genetic code that is incompatible with life?

Alpha thalassemia major

A mother brings in her 6-year-old son to the clinic with concerns of learning difficulties from his school and also states he seems very tired all the time. The family has been staying in an old, run down motel recently as they try to find housing with no other significant family history. Testing was ordered to include a CBC. The CBC shows a microcytic anemia but otherwise normal results. Which of the following would be present upon manual differential review?

Basophilic stippling

Cooley's anemia is another name for which form of beta thalassemia?

Beta thalassemia major

Nucleated RBCs are most likely to be seen in the peripheral blood of which type of beta thalassemia?

Beta thalassemia major

Skeletal deformations are most commonly present in which of the following beta thalassemias?

Beta thalassemia major

These test results are most consistent with which of the following types of beta thalassemia? TestPatient Result Red blood cell (RBC) count 5.6 x 1012/L4.1 - 5.1 x 1012/L Hemoglobin (Hb)11.2 g/dL12.3 - 15.3 g/dL Hematocrit (HCT)34.5%35.9 - 44.6% RDW14.0<14.5

Beta thalassemia minor

Which of the following describes a beta thalassemia genotype ß+/ß?

Beta thalassemia minor

A 5-year-old African American child with hepato-splenomegaly and skeletal abnormalities has the following laboratory results: WBC = 8.8 X 109/L RBC = 4.70 X 1012/L HGB = 6.2 g/dL 40 NRBC/100 WBC Many target cells Marked microcytosis, hypochromasia, anisocytosis & poikilocytosis were present Serum Iron = 200 µg/dL (elevated) Sickle Solubility = negative Hemoglobin F = elevated

Beta-thalassemia major

A hemoglobin F concentration of 100% may be seen in which beta thalassemia?

Delta-beta thalassemia major (dß0/dß0)

The hemoglobin electrophoresis pattern for patient #2 shows an increase in Hb F and a decrease in both Hb A and Hb A2. These results correlate best with which of these thalassemias?

Delta-beta thalassemia minor

Which of the following combination of globin chains comprise Hemoglobin H?

Four beta chains

With the exception of beta thalassemia minima, which hemoglobin is decreased in beta thalassemia?

Hb A

An alpha thalassemia patient is diagnosed as having three of their four genes deleted which code for alpha hemoglobin chains. Which one of the following types of hemoglobin is abnormal and would be found in such a patient?

Hb H

This image depicts a brilliant cresyl blue stained blood smear. What inclusion bodies are shown in the erythrocyte indicated by the arrow (B)?

Hb H inclusions

In which disorder do neonates demonstrate the presence of Bart's hemoglobin that changes to beta chain tetramers in adults?

Hemoglobin H disease

The anemia seen in anemia of chronic disease (also known as anemia of chronic infection) is characterized by low serum iron levels. Which of the following contributes to the low serum iron levels seen in persons with anemia of chronic disease?

Hepcidin

A peripheral smear with red blood cells photographed in a typical field was submitted for review. All of the following conditions are most likely associated with the red blood cell population found here, EXCEPT:

Hereditary Hemochromatosis

Which genetic description will NOT contain Bart's hemoglobin at birth ?

Heterozygous alpha thalassemia-2

The bone marrow in alpha thalassemia major usually demonstrates:

Hyperplasia

Hemoglobin H bodies can directly alter which of the following?

Integrity of the RBC membrane

All of the following are usually increased in alpha thalassemia EXCEPT?

Iron binding capacity

Which of these conditions could result in a microcytic anemia?

Iron deficiency

A patient is admitted to the emergency room with severe lethargy, glossitis, and muscle dysfunction. After the physician orders a complete blood count with differential, the hematology technologist observes cells matching the image to the right. Which condition is most consistent with the clinical and laboratory findings?

Iron deficiency anemia

All of the following are causes of hemolytic anemia, EXCEPT?

Iron deficiency anemia

In which of the following conditions would you NOT expect to find Howell-Jolly bodies?

Iron deficiency anemia

The abnormal RBCs seen in this illustration are indicative of:

Iron deficiency anemia

What is the condition most likely associated with the peripheral blood picture shown in the image on the right?

Iron deficiency anemia

Considering the red cell distribution width (RDW) in alpha thalassemia, how would you expect the red blood cell histogram to look?

Shifted to the left

A patient presents with the following measured parameters on the complete blood count: ParameterValue White blood cells (WBC)5.6 x 109/L4.0-11.0 x 109/L Red blood cells (RBC)3.6 x 1012/L4.2-5.9 x 1012/L Hemoglobin9.2 g/dL12-16 g/dL Hematocrit27%37-48% MCV75 fL80-100 fL RDW-CV17%11.0-15.0%

Microcytic, heterogenous anemia

A patient is admitted to the emergency room with lethargy and pallor. The CBC results are as follows: RBC = 4.1 x 1012/L Hemoglobin = 7.9 g/dL Hematocrit = 29% How would you classify this anemia?

Microcytic, hypochromic

Which one of the following statements about iron deficiency anemia (IDA) is false:

On average, 25% to 35% of ingested iron is absorbed each day.

Which of the following red cell inclusions are often found in peripheral blood smears of patients with sideroblastic anemia?

Pappenheimer bodies

All of the following are decreased in beta thalassemia major EXCEPT?

Red cell distribution width (RDW)

Note the iron-stained bone marrow preparation in the image to the right. The cells as shown can be associated with which type of anemia?

Sideroblastic anemia

Which of the following alpha thalassemia syndromes has the genotype -a/aa ?

Silent carrier

This image is from a patient with beta thalassemia. Though not diagnostic for this condition, what abnormal RBC morphology is present that would lead you to consider thalassemia?

Spherocytes

All of the following are associated with RBC macrocytosis EXCEPT?

Thalassemia

What are the usual results of the mean corpuscular volume (MCV) and the red cell distribution width (RDW) in alpha thalassemia?

The MCV is decreased but the RDW is usually increased

Alpha thalassemia is defined as:

The decrease in the rate of production of alpha chains due to a partial or total deletion of the genetic code