G6PD Deficiency and Aplastic Anemia Quiz
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Heinz bodies are ______ precipitates attached to the cytomembrane.

globin

Episodic hemolysis can be ______ or intravascular.

extravascular

In extravascular hemolysis, fragments of RBC membrane with attached Heinz bodies are plucked out by splenic ______.

macrophages

G6PD deficiency anemia can cause episodes of acute hemolysis ______ days after oxidant exposure.

<p>2-3</p> Signup and view all the answers

G6PD deficiency anemia does not typically show features of chronic hemolysis, such as ______ or cholelithiasis.

<p>splenomegaly</p> Signup and view all the answers

Laboratory findings in G6PD deficiency anemia include ______ cells, spherocytes, and Heinz bodies.

<p>bite</p> Signup and view all the answers

Heinz bodies can be identified using ______ stains.

<p>supravital</p> Signup and view all the answers

Immune hemolytic anemia (IHA) is also known as ______ hemolytic anemia.

<p>autoimmune</p> Signup and view all the answers

Aplastic anemia (AA) is characterized by a ______ of all blood cell lines.

<p>decrease</p> Signup and view all the answers

In aplastic anemia, the reticulocyte count is typically ______ 1%.

<p>less than</p> Signup and view all the answers

The severity of aplastic anemia is assessed based on the ______ of cytopenia.

<p>degree</p> Signup and view all the answers

A ______ bone marrow aspiration is a hallmark feature of aplastic anemia.

<p>hypocellular</p> Signup and view all the answers

A ______ can cause aplastic anemia by potentially producing antibodies against erythropoietin.

<p>thymoma</p> Signup and view all the answers

Pure red cell aplasia (PRCA) primarily affects the production of ______.

<p>red blood cells</p> Signup and view all the answers

In PRCA, the bone marrow shows a ______ in erythroid precursors.

<p>reduction</p> Signup and view all the answers

Parvovirus B19 infection can trigger a ______ crisis in patients with existing hemolytic anemia.

<p>aplastic</p> Signup and view all the answers

Hemolytic anemias are characterized by increased destruction of ______.

<p>RBCs</p> Signup and view all the answers

Anemias with ______ hemolysis occur outside of the blood vessels.

<p>extravascular</p> Signup and view all the answers

Anemia resulting from trauma to RBCs is known as ______.

<p>traumatic hemolysis</p> Signup and view all the answers

Sickle cell anemia is classified under ______ anemias.

<p>hemolytic</p> Signup and view all the answers

G6PD deficiency can lead to ______ hemolytic anemia.

<p>immune</p> Signup and view all the answers

Hemolytic anemias result in a decrease in ______ levels in the blood.

<p>Hb</p> Signup and view all the answers

Paroxysmal nocturnal hemoglobinuria is associated with ______ hemolysis.

<p>intravascular</p> Signup and view all the answers

Thalassemia is an example of ______ anemia.

<p>microcytic</p> Signup and view all the answers

Folate deficiency can lead to a type of ______ anemia.

<p>macrocytic</p> Signup and view all the answers

Anemia of chronic kidney disease is associated with decreased ______ production.

<p>erythropoietin</p> Signup and view all the answers

Spherocytosis is characterized by small RBCs without central ______.

<p>pallor</p> Signup and view all the answers

In severe cases of IHA, there may be nucleated ______ and Howell-Jolly bodies.

<p>RBCs</p> Signup and view all the answers

In cold antibody IHA, spontaneous RBC ______ can occur in the peripheral blood smear.

<p>agglutination</p> Signup and view all the answers

Unconjugated hyperbilirubinemia is often seen in ______ hemolytic anemias.

<p>immune</p> Signup and view all the answers

Polychromasia/reticulocytosis indicates an increased number of ______ in the blood.

<p>reticulocytes</p> Signup and view all the answers

In warm antibody IHA, blood films may show a high frequency of ______.

<p>microspherocytes</p> Signup and view all the answers

Erythroid hyperplasia in the bone marrow is a sign of ______ anemia.

<p>severe</p> Signup and view all the answers

The presence of Howell-Jolly bodies indicates ______ in red blood cell maturation.

<p>abnormalities</p> Signup and view all the answers

Anemia due to blood loss can be classified as either anemia due to ______ blood loss or chronic blood loss.

<p>acute</p> Signup and view all the answers

Chronic bleeding, such as from a peptic ulcer or colon cancer, can lead to depletion of ______ stores.

<p>iron</p> Signup and view all the answers

The clinical effects of anemia due to acute blood loss depend on the rate of ______.

<p>hemorrhage</p> Signup and view all the answers

If a patient survives acute blood loss, there will be a shift of water from the interstitial fluid compartment leading to ______.

<p>hemodilution</p> Signup and view all the answers

In cases of massive blood loss, cardiovascular collapse can lead to ______ shock.

<p>hypovolemic</p> Signup and view all the answers

Activation of erythropoietin (EPO) synthesis is a response to reduced oxygen-carrying capacity and leads to erythroid ______.

<p>hyperplasia</p> Signup and view all the answers

Reticulocytosis, an increase in reticulocytes, can be seen ______ to ______ days after acute blood loss.

<p>5-7</p> Signup and view all the answers

Both anemia due to acute blood loss and chronic blood loss can lead to ______-deficiency anemia if iron stores are depleted.

<p>iron</p> Signup and view all the answers

Megaloblastic anemia is characterized by ______ due to the deficiency of Vit B12 or folate.

<p>macrocytic anemia</p> Signup and view all the answers

The methionine synthase reaction plays a crucial role in the intersection of Vit B12 and ______ metabolism.

<p>folate</p> Signup and view all the answers

In megaloblastic anemia, there is a delay in nuclear division and maturation, resulting in ______ where cytoplasm maturation is preserved.

<p>nuclear-cytoplasmic asynchrony</p> Signup and view all the answers

The primary cause of megaloblastic anemia is a deficiency in either ______ or folate.

<p>Vitamin B12</p> Signup and view all the answers

Cobalamin is another name for ______, which is essential for many bodily functions.

<p>Vitamin B12</p> Signup and view all the answers

Ineffective erythropoiesis in megaloblastic anemia leads to the formation of large erythroblasts called ______.

<p>megaloblasts</p> Signup and view all the answers

The regenerative process of tetrahydrofolate (THF) is facilitated by cobalamin during the transfer of a ______ group.

<p>methyl</p> Signup and view all the answers

Anemia caused by a deficiency in Vitamin B12 or folate can lead to an increased mean corpuscular volume (MCV) greater than ______ fL.

<p>100</p> Signup and view all the answers

Flashcards

Heinz bodies

Precipitates of denatured hemoglobin attached to the red blood cell membrane. They are formed in response to oxidative stress.

Hemolytic anemia

A condition in which red blood cells are prematurely destroyed, leading to anemia.

Extravascular hemolysis

The process by which red blood cells are destroyed outside of blood vessels, primarily by the spleen.

Intravascular hemolysis

The process by which red blood cells are destroyed within blood vessels.

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G6PD deficiency anemia

A genetic deficiency of the enzyme glucose-6-phosphate dehydrogenase (G6PD), which leads to an inability to protect red blood cells from oxidative damage.

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Immune hemolytic anemia (IHA)

A type of hemolytic anemia caused by antibodies that bind to red blood cells, leading to their destruction.

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Warm antibody IHA

A type of IHA where antibodies bind to red blood cells at body temperature (37˚C).

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Cold agglutinin IHA

A type of IHA where antibodies bind to red blood cells at lower temperatures (0-4˚C).

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What is anemia?

Anemia is a condition where the blood doesn't have enough red blood cells.

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What is hemolytic anemia?

Hemolytic anemia is a type of anemia where red blood cells break down prematurely.

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How is hemolytic anemia categorized?

Hemolytic anemia is categorized based on where the red blood cells break down (inside or outside the blood vessels).

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What is extravascular hemolysis?

Extravascular hemolysis occurs when red blood cells break down outside the blood vessels.

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What is intravascular hemolysis?

Intravascular hemolysis occurs when red blood cells break down inside the blood vessels.

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What is sickle cell anemia?

Sickle cell anemia is a type of hemolytic anemia that involves abnormal hemoglobin.

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What is hereditary spherocytosis?

Hereditary spherocytosis is a type of hemolytic anemia caused by a genetic defect in red blood cells.

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What is G6PD deficiency?

Glucose-6-phosphate dehydrogenase deficiency (G6PD deficiency) is a type of hemolytic anemia.

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What is immune hemolytic anemia?

Immune hemolytic anemia is caused by the immune system attacking the body's red blood cells.

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What is paroxysmal nocturnal hemoglobinuria?

Paroxysmal nocturnal hemoglobinuria is a type of hemolytic anemia that affects red blood cells at night.

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Spherocytes

Spherocytes are small, densely packed red blood cells without a central pallor (lighter area). They are indicative of immune hemolytic anemia (IHA), but not exclusive to it.

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Polychromasia & Reticulocytosis

Polychromasia is the presence of red blood cells with varying colors. Reticulocytosis is an increase in the number of reticulocytes, immature red blood cells. Both are seen in IHA due to accelerated red blood cell production.

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Acute blood loss anemia

Anemia caused by a rapid loss of blood, usually from trauma or surgery.

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Chronic blood loss anemia

Anemia resulting from a gradual and ongoing loss of blood, often due to conditions like ulcers or cancer.

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Hypovolemia

The mechanism by which acute blood loss anemia develops, involving a rapid reduction in blood volume.

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Fluid shift

The process by which the body compensates for acute blood loss by shifting fluid from tissues into the bloodstream.

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Erythropoietin (EPO)

The hormone that stimulates red blood cell production in response to low oxygen levels.

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Reticulocytosis

An increased number of immature red blood cells in the blood, indicating an attempt to increase red blood cell production.

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Hypovolemic shock

A critical condition caused by massive blood loss, leading to a drop in blood pressure and inadequate organ perfusion.

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Iron recapture

The process by which the body reclaims iron from the breakdown of red blood cells during acute blood loss.

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Megaloblastic anemia

Anemia characterized by large, immature red blood cells (megaloblasts) due to impaired DNA synthesis.

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Vitamin B12 Deficiency Anemia

A type of megaloblastic anemia caused by a deficiency of vitamin B12, essential for DNA synthesis.

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Folate Deficiency Anemia

A type of megaloblastic anemia caused by a deficiency of folate, essential for DNA synthesis.

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Macrocytic anemia

Anemia with red blood cells larger than normal (MCV > 100 fL).

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Ineffective erythropoiesis

The process of red blood cell formation, which is ineffective in megaloblastic anemias due to impaired DNA synthesis.

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Nuclear-cytoplasmic asynchrony

A clinical feature of megaloblastic anemias where the nucleus of red blood cell precursors lags behind the cytoplasm in maturation.

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Megaloblasts

Immature red blood cells that are larger than normal, seen in megaloblastic anemias.

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Macrocytes

Mature red blood cells that are larger than normal, found in megaloblastic anemias.

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Aplastic Anemia (AA)

A rare condition characterized by failure of the bone marrow to produce red blood cells due to various factors, including viral infections, autoimmune diseases, and exposure to toxins. This results in a decline in red blood cell production, leading to anemia.

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AA: Peripheral Blood Findings

Patients with AA often present with morphologically normal red blood cells (normocytic normochromic anemia), indicating a low red blood cell production rate. The reticulocyte count, an indicator of red blood cell production, is usually below 1%, reflecting the impaired red blood cell production.

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AA: White Blood Cell Changes

AA is marked by decreased numbers of all types of white blood cells, particularly neutrophils, leading to neutropenia or agranulocytosis. The number of monocytes is also low, and there is a relative increase in lymphocytes. Platelet counts are also deficient, indicating a problem with platelet production.

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Pure Red Cell Aplasia (PRCA)

A severe condition that affects the production of red blood cells specifically, while the production of other blood cells remains normal.

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PRCA: Bone Marrow Aspiration & Biopsy

A common finding in PRCA where the bone marrow fails to produce red blood cells, but the production of other blood cell types remains normal.

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Parvovirus B19 and PRCA

PRCA and Parvovirus B19 infections are linked, especially in individuals with preexisting hemolytic anemia.

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Thymomas and PRCA

A type of tumor that can be associated with the production of autoantibodies to erythropoietin, a hormone essential for red blood cell production. This can contribute to the development of PRCA by interfering with red blood cell development.

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Aplastic Anemia: Severity Assessment

The severity of aplastic anemia is determined by the degree of reduction in all blood cell types.

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Study Notes

Anemias (Except Hemoglobinopathies)

  •  The document outlines various types of anemia, excluding hemoglobin disorders.
  •  It categorizes anemias into distinct parts: hemolytic anemias (excluding sickle cell and HbC diseases, and thalassemias), anemias due to reduced erythropoiesis (excluding megaloblastic anemias), macrocytic (megaloblastic) anemias, and anemias due to blood loss.

Part 1: Hemolytic Anemias (Except Sickle Cell and HbC Diseases, and Thalassemias)

  •  This section focuses on anemias caused by the destruction of red blood cells.
  • Detailed study of hereditary spherocytosis (HS), G6PD-deficiency anemia, immune hemolytic anemias (IHA), malaria-related anemia, and hemolytic anemia due to trauma to RBCs are included.

Learning Objectives (Page 2)

  •  Objectives for each type of hemolytic anemia, requiring students to explain etiology, pathogenesis, laboratory diagnosis, and correlate them with associated clinical features.

Hereditary Spherocytosis (HS) (Page 2)

  •  A hereditary condition common in Northern Europe.
  •  Caused by a defect in the red blood cell (RBC) membrane skeleton.
  •  Outcome: Loss of membrane fragments leads to spherocytes.

HS: Clinical Manifestations (Page 3)

  •  Anemia
  •  Splenomegaly (0.5-1.0 kg)
  •  Intermittent jaundice, often linked to viral infections.
  •  Aplastic crisis related to Parvovirus B19 infection.
  •  Gallstones
  •  Leg ulcers
  •  Mild-to-severe variability in presentation.

HS: Lab Findings (CBC) (Page 4)

  •  RBC count: normal to reduced.
  •  Normocytic RBCs (MCV 80-100 μm³)
  •  Hb: normal to reduced.
  •  HCT: normal to reduced.
  •  MCH: normal to reduced.
  •  MCHC: increased.
  •  Reticulocyte count: increased (>3%).

HS: Additional Lab Findings (Page 4)

  •  Peripheral blood smear (PBS) shows spherocytes
  •  Polychromasia/reticulocytosis
  •  Bone marrow analysis: erythroid hyperplasia with normoblastic reaction
  •  Serum bilirubin: unconjugated hyperbilirubinemia confirmed by osmotic fragility test.

HS: Treatment and Treatment Side-Effects (Page 5)

  •  Splenectomy is a treatment option, eliminating splenic sequestration and reducing phagocytosis of spherocytes, minimizing the impact on anemia and other symptoms.
  •  Side effects include reduced immunity to encapsulated microorganisms.

G6PD Deficiency Anemia (Page 6)

  •  A recessive, X-linked disorder.
  •  Common in Africa, the Mediterranean, and African Americans.
  •  Potentially protective against malaria.

G6PD-Deficiency Anemia: Clinicolaboratory Correlates (Page 6)

  •  Episodes of acute hemolysis (2–3 days later) manifest as anemia, hemoglobinemia, and hemoglobinuria.
  •  No features of chronic hemolysis (splenomegaly, gallstones).
  •  Laboratory findings include bite cells, spherocytes, and Heinz bodies.

Immune Hemolytic Anemia (IHA) (Page 7)

  •  Also known as autoimmune hemolytic anemia.
  •  Warm antibody type (80%): IgG binds RBCs at 37°C.
  •  Cold agglutinin type (20%): IgM binds RBCs at cooler temperatures.

Warm Antibody IHA (Pages 7-8)

  •  Predisposing conditions in about 50% of cases, often linked to autoimmune conditions (SLE) or medications.
  •  Antibody production targeting RBCs.
  •  RBC destruction by splenic macrophages.

Cold Agglutinin IHA (Page 8)

  •  Predisposing factors include infections (mycoplasma pneumoniae, Epstein-Barr virus, cytomegalovirus) or lymphoid neoplasms.
  •  IgM antibodies bind to RBCs at lower temperatures (0–4°C) resulting in agglutination.
  •  Complement activation leads to RBC destruction predominately via extravascular processes.

Anemia in Malaria (Page 9)

  •  Anemia due to malaria is caused by infection by parasites (e.g., Plasmodium falciparum).
  •  Mechanisms involve extravascular hemolysis where infected RBCs are targeted by the immune system leading to their removal.
  •  Reduced erythropoiesis can also occur (anemia of inflammation) as a result of the inflammatory response.

Paroxysmal Nocturnal Hemoglobinuria (PNH) (Page 10)

  •  A rare, acquired genetic disorder affecting the PIGA gene.
  •  Characterized by intravascular hemolysis (persistent or paroxysmal).
  •  Defects in GPI-anchored proteins (CD55 and CD59), making RBCs, and other blood cells vulnerable to complement-mediated damage.

PNH: Hematologic Lab Findings(Page 10)

  •  CBC may reveal normal to reduced RBC counts, with normal to elevated MCV values.
  •  Reticulocyte counts are typically increased, exceeding 3%.
  •  Peripheral blood smear may show typical features of normocytic or macrocytic picture (without major abnormalities).
  •  Bone marrow may show erythroid hyperplasia.

PNH: Flow Cytometry (Page 11)

  •  The gold standard test for PNH.
  •  Diagnostic feature: a distinctive bimodal distribution of CD59- and CD55-negative cells.

PNH: Serum and Urine Findings (Page 11)

  •  Tests for intravascular hemolysis reveal hemoglobinemia and low or absent haptoglobin.
  •  Serum assays include elevated LDH (lactate dehydrogenase) for hemolytic markers.

PNH: Clinical Findings (Page 11)

  •  Patients exhibit pallor, dyspnea, palpitations, and jaundice (chronic hemolysis).
  •  Episodes of hemolysis (paroxysmal characteristic) may occur due to minor acidosis conditions.

Hemolytic Anemia Resulting from Trauma to RBCs (Page 12)

  •  Resulting from mechanical or traumatic damage.
  •  Leads to intravascular hemolysis and fragmented red blood cells (schistocytes).
  •  Possible causes: aortic stenosis, prosthetic valves, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, or disseminated intravascular coagulation.
  •  Characteristic lab findings include the presence of schistocytes (helmet cells).

Part 2: Anemias Due to Diminished Erythropoiesis (Except Megaloblastic)

  •  Anemia resulting from decreased red blood cell production due to damaged or impaired bone marrow function
  • Covers Aplastic anemia
  • Including pure red cell aplasia, myelophthisic anemia, and anemia of chronic kidney disease
  • Covers iron-deficiency anemia and inflammation-related anemia
  • Covers sideroblastic anemia

Aplastic Anemia (AA) (Page 13)

  •  Life-threatening bone marrow (BM) failure characterized by BM hypoplasia due to suppression of multipotent myeloid cells
  •  Clinical symptoms include pallor, fatigue, fever, cellulitis, and pneumonia
  •  Laboratory findings often show pancytopenia (anemia, neutropenia, thrombocytopenia), low reticulocyte count, hypocellular bone marrow.

Part 3: Macrocytic (Megaloblastic) Anemias

  •  Anemia arising from impaired DNA synthesis due to vitamin B12 or folate deficiency
  •  Includes Vitamin B12 deficiency and folate deficiency
  •  Clinical features associated with these include anemia, neurological issues, and gastrointestinal disturbances

Part 4: Anemias Due to Blood Loss

  •  This section focuses on anemias due to acute and chronic blood loss.
  •  Acute blood loss anemias are often characterized by rapid blood loss, hemoconcentration, and hemolysis.
  • Chronic blood loss anemias develop gradually, affecting the iron stores and impacting erythropoiesis.

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Anemias (Except Hemoglobinopathies) PDF

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Test your knowledge on G6PD deficiency and aplastic anemia with this quiz. Explore critical concepts like hemolysis, lab findings, and key features of these conditions. Perfect for medical students and healthcare professionals.

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