Giant Platelet Disorders Quiz

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Which disorder is characterized by abnormal neutrophil inclusions?

Bernard–Soulier syndrome

May–Hegglin anomaly (correct)

Mediterranean macrothrombocytopenia

Hereditary macrothrombocytopenia with hearing loss

Which treatment is the main option for people with bleeding symptoms due to giant platelet disorders?

DDAVP

Splenectomy

Platelet transfusion (correct)

None of the above

Which platelet disorder is associated with alpha granules defect?

Montreal platelet syndrome

Gray platelet syndrome (correct)

Sebastian syndrome

Bernard–Soulier syndrome

Which of the following is a common symptom of giant platelet disorders?

Frequent nosebleeds Signup and view all the answers

What is the main cause of abnormal bleeding in individuals with giant platelet disorders?

Inadequate platelet adhesion to blood vessel walls Signup and view all the answers

Which of the following is NOT a genetically inherited giant platelet disorder?

Immune thrombocytopenic purpura (ITP) Signup and view all the answers

How are giant platelet disorders typically diagnosed?

Through platelet aggregation studies and flow cytometry Signup and view all the answers

What is the characteristic feature of giant platelet disorders in terms of platelet count and mean platelet volume (MPV)?

Low platelet count and high MPV Signup and view all the answers

Which platelet disorder is caused by glycoprotein abnormalities?

Bernard–Soulier syndrome Signup and view all the answers

Which platelet disorder is caused by calpain defect?

Montreal platelet syndrome Signup and view all the answers

Which platelet disorder is associated with abnormal neutrophil inclusions?

May–Hegglin anomaly Signup and view all the answers

Which platelet disorder is associated with systemic manifestations?

Hereditary macrothrombocytopenia with hearing loss Signup and view all the answers

Which zone of the platelet contains clotting mediators such as factor V, factor VIII, fibrinogen, fibronectin, platelet-derived growth factor, and chemotactic agents?

Organelle zone Signup and view all the answers

What is the hormone that regulates megakaryocyte and platelet production?

Thrombopoietin Signup and view all the answers

What is the approximate average life span of circulating platelets?

8-9 days Signup and view all the answers

Which granule contains ADP or ATP and is involved in platelet secretion upon activation?

δ granules Signup and view all the answers

Platelets are fragments of cytoplasm derived from the megakaryocytes of the bone marrow or lung, which then enter the circulation. What is the function of platelets?

To initiate blood clotting Signup and view all the answers

What is the process of stopping bleeding at the site of interrupted endothelium called?

Hemostasis Signup and view all the answers

What is the main function of platelets in hemostasis?

To form a platelet plug Signup and view all the answers

Which of the following is a key step in the process of coagulation?

Deposition of fibrin Signup and view all the answers

What initiates the process of coagulation after an injury to a blood vessel?

Exposure of subendothelial tissue factor Signup and view all the answers

What is the function of secondary hemostasis in coagulation?

Deposition of fibrin strands Signup and view all the answers

Which of the following is the first stage of hemostasis?

Vasoconstriction Signup and view all the answers

What triggers the extrinsic pathway of the coagulation cascade?

Tissue factor Signup and view all the answers

What is the final stage of hemostasis called?

Tertiary hemostasis Signup and view all the answers

Which pathway is responsible for the initiation of blood coagulation?

Tissue factor pathway Signup and view all the answers

What is the role of tissue factor (TF) pathway in the coagulation cascade?

To generate a thrombin burst Signup and view all the answers

What is the final common pathway of the coagulation cascade?

Factor X, thrombin, and fibrin Signup and view all the answers

What is the most common cause of pernicious anemia?

Autoimmune attack on the cells that create intrinsic factor Signup and view all the answers

How is pernicious anemia diagnosed?

All of the above Signup and view all the answers

Which disorder is considered an end stage of autoimmune atrophic gastritis?

Pernicious anemia Signup and view all the answers

What is the role of Helicobacter pylori infection in pernicious anemia (PA)?

It causes gastric autoimmunity Signup and view all the answers

What can cause impaired B12 absorption following gastric surgery?

Gastric removal (gastrectomy) Signup and view all the answers

Which disease is NOT associated with microangiopathic hemolytic anemia?

Malignant hypertension Signup and view all the answers

What is the common feature of red blood cells in microangiopathic hemolytic anemia?

They are destroyed by the reticuloendothelial system Signup and view all the answers

In which condition do immune complexes aggregate with platelets, forming intravascular thrombi?

Systemic lupus erythematosus Signup and view all the answers

Which of the following diseases is NOT associated with microangiopathic hemolytic anemia?

Polyarteritis nodosa Signup and view all the answers

What is the main cause of microangiopathic hemolytic anemia?

Formation of a fibrin mesh Signup and view all the answers

Which enzyme is responsible for the conversion of protoporphyrinogen IX to protoporphyrin IX?

Protoporphyrinogen oxidase Signup and view all the answers

What is the name of the molecule formed when protoporphyrin IX is complexed with an iron(II) cation?

Heme Signup and view all the answers

Which of the following is NOT a heme-containing protein?

Chlorophyll Signup and view all the answers

What is the precursor molecule of protoporphyrin IX in the biosynthesis pathway?

Uroporphyrinogen III Signup and view all the answers

What is the oxygen saturation?

The amount of oxygen bound to hemoglobin Signup and view all the answers

What is the P50 in the context of the oxyhemoglobin dissociation curve?

The partial pressure of oxygen at which hemoglobin is 50% saturated Signup and view all the answers

Which molecule binds to the iron of the heme prosthetic group in hemoglobin?

Oxygen Signup and view all the answers

What causes a change in the shape of hemoglobin, increasing its ability to bind carbon dioxide and decreasing its ability to bind oxygen?

Presence of carbon dioxide Signup and view all the answers

What is the relationship between oxygen saturation (SO2) and partial pressure of oxygen in the blood (PO2) called?

Oxyhemoglobin dissociation curve Signup and view all the answers

Which factor causes a shift to the right in the oxyhemoglobin dissociation curve?

Decreased affinity for oxygen Signup and view all the answers

Which condition is associated with a left shift of the oxyhemoglobin dissociation curve?

Decreased 2,3-bisphosphoglycerate (2,3-BPG) concentration Signup and view all the answers

Which of the following is NOT a factor that can cause a right shift of the oxyhemoglobin dissociation curve?

Decreased carbon dioxide concentration Signup and view all the answers

What is the effect of a decrease in pH on the oxyhemoglobin dissociation curve?

Shift to the right Signup and view all the answers

Which transcriptional factor is responsible for the commitment of granulocytes from GMPs to monocytes?

PU.1 Signup and view all the answers

Which cytokine enhances the production of GM-CSF and G-CSF?

IL-3 Signup and view all the answers

Which factor is the master regulator of steady state granulopoiesis?

C/EBPα Signup and view all the answers

Which transcriptional factor is the main regulator of emergency granulopoiesis?

C/EBPβ Signup and view all the answers

Which program of granulopoiesis is activated after a bacterial infection?

Emergency granulopoiesis Signup and view all the answers

Which type of white blood cell is referred to as a polymorphonuclear leukocyte (PMN)?

Neutrophil Signup and view all the answers

Where does granulopoiesis take place?

Bone marrow Signup and view all the answers

Which of the following is NOT a mature granulocyte produced by granulopoiesis?

Monocyte Signup and view all the answers

What is the first unipotent cell in granulopoiesis?

Myeloblast Signup and view all the answers

What are the two parts of granulopoiesis?

Granulocyte lineage determination and Committed granulopoiesis Signup and view all the answers

Which region has the highest prevalence of sickle cell disease cases?

Sub-Saharan Africa Signup and view all the answers

What is the most common complication of sickle cell disease?

Vaso-occlusive crisis Signup and view all the answers

At what age do problems in sickle cell disease typically begin?

Around 5 to 6 months of age Signup and view all the answers

What is the average life expectancy for individuals with sickle cell disease in the developed world?

40 to 60 years Signup and view all the answers

Which of the following is a common complication of sickle cell anaemia?

Increased risk of severe bacterial infections Signup and view all the answers

What is one of the earliest clinical manifestations of sickle cell disease?

Dactylitis Signup and view all the answers

What is the main cause of microangiopathic hemolytic anemia in sickle cell disease?

Intravascular sickling of the bowel Signup and view all the answers

Which organ is affected in sickle cell disease, leading to an increased risk of severe bacterial infections?

Spleen Signup and view all the answers

Which of the following is a common complication of sickle cell disease?