Disease state- ertrocytic-anemia-Normocytic

The first time a cell containing HbSS is deoxygenated, it forms a sickle cell. Upon reoxygenation, which of the following is true for the sickle cell?

It will return to a normal biconcave shape.

Nitric oxide is associated with the prevention of vaso-occlusion by decreasing cellular adherence to endothelium. Which amino acid is decreased in patients with sickle cell disease and is needed as a substrate to produce nitric oxide?

L-arginine

Which of the following causes schistocyte formation in disseminated intravascular coagulation (DIC)?

Microthrombi disposition in small vessels

Which of the following is characterized by the bone marrow being invaded by fibrotic, granulomatous, or neoplastic cells?

Myelophthisic anemia

Which group of conditions increases the risk of HbS polymerization?

Acid pH, dehydration, increased level of 2,3-DPG

In most cases of Fanconi anemia, which of the following types of anemia develops?

Aplastic

What is the MOST likely explanation for a patient with sickle cell anemia whose hemoglobin has dropped to 4 g/dL and has a reticulocyte count of 0.1%?

Aplastic Crisis

Which of the following conditions can be associated with the red blood cell morphologies shown in this image?

Autoimmune hemolytic anemia

In HbSS blood, an increased amount of which of the following surface antigens on young sickle cells (reticulocytes) may allow platelets to form a bridge between the reticulocytes and endothelial cells, ultimately leading to vaso-occlusion?

CD36

Which of the following disorders is characterized by a stem cell mutation due to the PIGA gene that leads to clones of cells that bind abnormally large amounts of complement?

Paroxysmal nocturnal hemoglobinuria

Disorders affecting the thyroid, pituitary, and adrenal glands can all lead to anemia (mild to moderate). This is most often associated to which of the following?

Decrease in erythropoietin (EPO)

Which of the following laboratory test results indicates that a sickle cell patient may be in aplastic crisis?

Decreased reticulocyte count

Which of the following types of anemia can be described as an inherited bone marrow failure syndrome with patients developing dystrophic nails and white patches in the mouth?

Dyskeratosis congenita

All of the following are associated with HELLP syndrome EXCEPT:

Elevated lipid levels

Treatment of "sequestration crisis," often seen in infants and young children with sickle cell disease, involves which of the following?

Exchange transfusions, and/or splenectomy.

Which of the following assays is more sensitive and specific for the diagnosis of Paroxysmal Nocturnal Hemoglobinuria (PNH)?

Flow cytometry for GPI-anchored proteins

Which of the following could cause a sickling event?

Fluctuations in temperature

What is the MOST likely cause of a patient who developed a severe hemolytic episode after taking anti-malarial drugs, and red cell inclusions caused by denatured hemoglobin were seen on the differential smear?

G-6-PD deficiency

Which hemoglobin, when elevated, acts as a protection against sickling in patients with HbS?

Hemoglobin F

A completely sickled cell (drepanocyte) is most commonly seen in which of these conditions?

Homozygous HbSS

Which of the following is an FDA-approved therapeutic agent for treating sickle cell patients?

Hydroxyurea

Spherocytes are associated with which of the following conditions?

Immune hemolytic anemia

Which of the following viruses is MOST often reported as the cause of infection leading to aplastic crisis in a patient with sickle cell disease?

Parvovirus B19

Which of the following laboratory results would you find with disseminated intravascular coagulation (DIC) but NOT with thrombotic thrombocytopenic purpura (TTP) or hemolytic uremic syndrome (HUS)?

Prolonged PT and aPTT

What is the best description of the phenomenon seen in this illustration?

Rosette formation

Gastrointestinal infections of which of the following a organisms are associated with hemolytic uremic syndrome?

Shiga toxin producing E. coli

The abnormal cells indicated by the arrows are indicative of which of the following?

Sickle cell disease

All of the following are laboratory findings in cases of hemolytic uremic syndrome (HUS) EXCEPT:

Increased uric acid

How does hydroxyurea aid in the treatment of sickle cell disease?

Induces increased production of HbF.

A CBC performed on a 10-year-old African-American male who was brought to the emergency department with rapid, shallow breathing and fever provided the following results: Parameter Patient Result WBC28.0 x 109/L3.2- 9.8 x 109/L RBC3.88 x 1012/L4.50 - 5.70 x 1012/L Hemoglobin10.0 g/dL13.6 - 17.2 g/dL MCV82 fL80 - 110 fL RDW2311 - 14.5 Reticulocyte 8.0%0.5 - 1.5%

Sickle cells

Which of the following characteristics can be seen in the microcytes present in some hemolytic disorders?

Spherocytosis

Disseminated intravascular coagulation (DIC) is associated with all of the following clinical conditions EXCEPT:

Thrombocytosis

To produce hemoglobin S (HbS), glutamic acid that is normally present in the sixth position on the beta globin chain is substituted with which of the following?

Valine

Complaints of pain from a patient with sickle cell disease are most likely associated with which of the following conditions/events?

Vaso-occlusive crisis