Podcast
Questions and Answers
What type of proteins primarily make up the hexagonal lattice in red blood cells?
What type of proteins primarily make up the hexagonal lattice in red blood cells?
What is the approximate distance of globular ankyrin structures from the distal ends of spectrin filaments?
What is the approximate distance of globular ankyrin structures from the distal ends of spectrin filaments?
Which component is found in cross-linking junctional complexes along with protein 4.1?
Which component is found in cross-linking junctional complexes along with protein 4.1?
What condition is associated with hereditary spherocytosis based on membrane protein properties?
What condition is associated with hereditary spherocytosis based on membrane protein properties?
Signup and view all the answers
What staining method is used to separate proteins via SDS–PAGE?
What staining method is used to separate proteins via SDS–PAGE?
Signup and view all the answers
What is the inheritance pattern for Hereditary Stomatocytosis in most patients with severe hemolysis?
What is the inheritance pattern for Hereditary Stomatocytosis in most patients with severe hemolysis?
Signup and view all the answers
What primarily causes the swelling of erythrocytes in Hereditary Stomatocytosis?
What primarily causes the swelling of erythrocytes in Hereditary Stomatocytosis?
Signup and view all the answers
Which cells are characteristic of a blood smear from a patient with Hereditary Xerocytosis?
Which cells are characteristic of a blood smear from a patient with Hereditary Xerocytosis?
Signup and view all the answers
What is the main cause of cell dehydration in Hereditary Xerocytosis?
What is the main cause of cell dehydration in Hereditary Xerocytosis?
Signup and view all the answers
Which abnormality is associated with Hereditary Stomatocytosis?
Which abnormality is associated with Hereditary Stomatocytosis?
Signup and view all the answers
What is the classification of hereditary defects affecting the red cell membrane primarily based on?
What is the classification of hereditary defects affecting the red cell membrane primarily based on?
Signup and view all the answers
Which type of hereditary defect is characterized by spherocytes?
Which type of hereditary defect is characterized by spherocytes?
Signup and view all the answers
The defect in which kind of interaction contributes to spherocyte formation?
The defect in which kind of interaction contributes to spherocyte formation?
Signup and view all the answers
Which of the following disorders is included in morphologically related disorders to hereditary elliptocytosis?
Which of the following disorders is included in morphologically related disorders to hereditary elliptocytosis?
Signup and view all the answers
Hereditary acanthocytosis results from defects primarily affecting which aspect of red cell morphology?
Hereditary acanthocytosis results from defects primarily affecting which aspect of red cell morphology?
Signup and view all the answers
Defects in spectrin dimer self-association primarily lead to which type of red cell alteration?
Defects in spectrin dimer self-association primarily lead to which type of red cell alteration?
Signup and view all the answers
Which hereditary defect is characterized by a defect in the horizontal interaction of the red cell membrane?
Which hereditary defect is characterized by a defect in the horizontal interaction of the red cell membrane?
Signup and view all the answers
Hereditary xerocytosis is primarily associated with which type of morphological change?
Hereditary xerocytosis is primarily associated with which type of morphological change?
Signup and view all the answers
What red cell morphology is commonly associated with hereditary spherocytosis?
What red cell morphology is commonly associated with hereditary spherocytosis?
Signup and view all the answers
Which condition is associated with the presence of target cells?
Which condition is associated with the presence of target cells?
Signup and view all the answers
What red blood cell morphology is associated with acute hepatic necrosis?
What red blood cell morphology is associated with acute hepatic necrosis?
Signup and view all the answers
In which condition do you expect to see bizarre poikilocytes?
In which condition do you expect to see bizarre poikilocytes?
Signup and view all the answers
Which morphology is typically seen in megaloblastic anemia?
Which morphology is typically seen in megaloblastic anemia?
Signup and view all the answers
What red blood cell morphology is associated with uremia?
What red blood cell morphology is associated with uremia?
Signup and view all the answers
Which red cell morphology indicates hereditary elliptocytosis?
Which red cell morphology indicates hereditary elliptocytosis?
Signup and view all the answers
What shape do red blood cells take due to the biconcave disc structure?
What shape do red blood cells take due to the biconcave disc structure?
Signup and view all the answers
What condition is associated with intracytoplasmic parasites?
What condition is associated with intracytoplasmic parasites?
Signup and view all the answers
Which of the following conditions is related to a defect in the erythropoietic process?
Which of the following conditions is related to a defect in the erythropoietic process?
Signup and view all the answers
What condition is most consistent with severe hemolytic episodes after exposure to anti-malarial drugs in a patient with congenital non spherocytic hemolytic anemia?
What condition is most consistent with severe hemolytic episodes after exposure to anti-malarial drugs in a patient with congenital non spherocytic hemolytic anemia?
Signup and view all the answers
Hereditary pyropoikilocytosis (HP) is characterized by which of the following features?
Hereditary pyropoikilocytosis (HP) is characterized by which of the following features?
Signup and view all the answers
A patient with congenital non spherocytic hemolytic anemia presents with red cell inclusions. What does this indicate about hemoglobin status?
A patient with congenital non spherocytic hemolytic anemia presents with red cell inclusions. What does this indicate about hemoglobin status?
Signup and view all the answers
Which specific symptom did the African-American patient experience after taking primaquine?
Which specific symptom did the African-American patient experience after taking primaquine?
Signup and view all the answers
What is a common result of G-6-PD deficiency when exposed to oxidative drugs?
What is a common result of G-6-PD deficiency when exposed to oxidative drugs?
Signup and view all the answers
In patients presenting with hereditary pyropoikilocytosis, what would you expect to see under microscopic examination?
In patients presenting with hereditary pyropoikilocytosis, what would you expect to see under microscopic examination?
Signup and view all the answers
Which of the following conditions does NOT typically cause red cell inclusions due to hemoglobin denaturation?
Which of the following conditions does NOT typically cause red cell inclusions due to hemoglobin denaturation?
Signup and view all the answers
What is the primary mechanism leading to hemolysis in G-6-PD deficiency?
What is the primary mechanism leading to hemolysis in G-6-PD deficiency?
Signup and view all the answers
What type of anemia is characterized by the premature destruction of red cells?
What type of anemia is characterized by the premature destruction of red cells?
Signup and view all the answers
Which of the following is NOT a classification of hemolytic anemia?
Which of the following is NOT a classification of hemolytic anemia?
Signup and view all the answers
What is one of the clinical findings associated with hemolytic anemia?
What is one of the clinical findings associated with hemolytic anemia?
Signup and view all the answers
Which type of hemolytic anemia is related to hereditary defects?
Which type of hemolytic anemia is related to hereditary defects?
Signup and view all the answers
Which laboratory test reflects increased red cell destruction in hemolytic anemia?
Which laboratory test reflects increased red cell destruction in hemolytic anemia?
Signup and view all the answers
What is a characteristic feature of hereditary spherocytosis?
What is a characteristic feature of hereditary spherocytosis?
Signup and view all the answers
Which condition is classified as an extracorpuscular defect in hemolytic anemia?
Which condition is classified as an extracorpuscular defect in hemolytic anemia?
Signup and view all the answers
What is one reason the bone marrow can increase output in response to hemolytic anemia?
What is one reason the bone marrow can increase output in response to hemolytic anemia?
Signup and view all the answers
Which of the following conditions leads to increased levels of methemalbumin in the blood?
Which of the following conditions leads to increased levels of methemalbumin in the blood?
Signup and view all the answers
What happens to serum unconjugated bilirubin levels in hemolytic anemia?
What happens to serum unconjugated bilirubin levels in hemolytic anemia?
Signup and view all the answers
Study Notes
Hemolytic Anemia
- Hemolytic anemia (HA) is a heterogeneous group of normocytic normochromic anemias characterized by premature destruction of red blood cells (RBCs) or a shortened RBC life span.
- Bone Marrow (BM) can increase its output 6-8 fold to compensate for the anemia; which corresponds to an RBC life span of 15-20 days.
Hemolytic Anemia Classification
-
Intracorpuscular Defects:
- Hereditary defects:
- Defects in RBC membrane.
- Enzyme defects.
- Hemoglobinopathies.
- Thalassemia syndrome
- Acquired defects: Paroxysmal nocturnal hemoglobinuria (PNH)
- Hereditary defects:
-
Extracorpuscular Defects:
- Immune hemolytic anemia.
- Infections.
- Exposure to chemicals and toxins.
- Exposure to physical agents.
- Microangiopathic and macroangiopathic hemolytic anemias.
- Splenic sequestration (hypersplenism)
- General systemic disorders (in which hemolysis is not the dominant feature of the anemia.)
Diagnosis of Hemolytic Anemia (HA)
- Establish the presence of hemolysis:
- Tests reflecting increased RBC destruction:
- Serum un-conjugated (indirect) bilirubin
- Hemoglobinemia
- Hemoglobinuria (dark urine)
- Hemosiderinuria (iron storage protein in a spun deposit of urine)
- Methemalbumin in blood (Schumm’s test)
- Methemoglobinemia
- Serum Hemopexin
- Serum Haptoglobin
- Urine urobilinogen & fecal stercobilinogen
- Tests reflecting increased RBC destruction:
- Tests reflecting increased RBC production
- Reticulocyte count
- Reticulocyte production index (RPI)
- Bone Marrow (BM) erythroid hyperplasia, M:E ratio from 3-4:1 is reduced to 1:1 or even reversed.
- RPI >2.5-3.0 is indicative of HA
Degradation of Hb After Intra- & Extravascular Hemolysis
- When present in large quantities, met-albumin and hemopexin-heme complex imparts a brownish color to plasma.
- Results in hemoglobinuria, hemosiderinuria and urobilinogenuria.
Other important aspects
-
Red cell membrane structure is determined by biconcave disc shape which is a result of Excess SA:Volume ratio; cell volume is 90 µm³ while SA is ~140 µm³.
-
Red cell membrane skeleton network is composed of proteins like spectrin, actin, ankyrin, band 4.1, adducin, etc.
-
Hereditary defects of RBC membrane:
- Hereditary spherocytosis (HS)
- Hereditary elliptocytosis (HE)
- Hereditary pyropoikilocytosis (HPP)
- Hereditary stomatocytosis
- Hereditary xerocytosis
- Hereditary acanthocytosis
-
Hereditary Enzyme deficiencies:
- Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD)
- Pyruvate Kinase (PK) Deficiency
- Methemoglobin Reductase Deficiency
Assessment Questions
- Assessment questions are provided.
Studying That Suits You
Use AI to generate personalized quizzes and flashcards to suit your learning preferences.
Related Documents
Description
Test your knowledge on the proteins that make up the hexagonal lattice in red blood cells. This quiz covers important concepts such as spectrin filaments, ankyrin structures, and the role of protein 4.1 in cross-linking junctions. Discover the association between membrane protein properties and hereditary spherocytosis, as well as techniques like SDS–PAGE used in protein analysis.
