Medicine Marrow Pg 71-80 (Hematology)
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Medicine Marrow Pg 71-80 (Hematology)

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Questions and Answers

What does a Mentzer index greater than 13 indicate?

  • Thalassemia trait
  • Iron deficiency (correct)
  • Anemia of chronic disease
  • Normal iron balance

    • In iron deficiency anemia, the serum ferritin level is typically higher than normal.

    False

    What term describes the larger variation in red blood cell size observed in iron deficiency anemia?

    Anisocytosis

    The __________ index is calculated using the formula mcv/RBC to differentiate between iron deficiency and thalassemia.

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

    Match the following indices/parameters with their typical values in iron deficiency anemia:

    <p>S. ferritin = ↓ S. iron = ↓ TIBC = ↑ Serum soluble transferrin receptors = ↑</p> Signup and view all the answers

    What is the primary function of the transferrin-iron complex in the bone marrow?

    <p>To transport iron for heme synthesis</p> Signup and view all the answers

    A normal serum ferritin level indicates high iron stores.

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

    What is the most sensitive indicator for iron deficiency?

    <p>Increased soluble transferrin receptor level</p> Signup and view all the answers

    The earliest indicator for iron deficiency is low _____ level.

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

    Match the following iron indices with their normal ranges:

    <p>S. Ferritin = 30 - 300 ng/ml S. Iron = 50 - 150 µg/dL TIBC = 300 - 360 µg/dL TSAT = 33%</p> Signup and view all the answers

    What characterizes Stage I (Pre-latent) of Iron Deficiency Anemia?

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

    In Stage II (Latent), both serum ferritin and bone marrow iron levels are normal.

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

    What is the characteristic serum ferritin level in Stage III (Iron Deficiency Anemia)?

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

    In Stage I (Pre-latent), there is a __________ iron balance.

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

    Match the stages of Iron Deficiency Anemia with their features:

    <p>Stage I (Pre-latent) = Negative iron balance Stage II (Latent) = Absent Stage III (Iron Deficiency Anemia) = Decreased serum ferritin</p> Signup and view all the answers

    Which of the following conditions is associated with a mutation in the HFE gene?

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

    In Sideroblastic Anemia, ALA synthase deficiency is commonly inherited in an X-linked manner.

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

    What substance plays a role in recycling iron from macrophages, enterocytes, and hepatocytes?

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

    In iron deficiency anemia, the serum iron level is typically __________.

    <p>very low</p> Signup and view all the answers

    Match the following conditions with their associated causes:

    <p>Sideroblastic Anemia = Lead exposure Copper deficiency = Acquired Sideroblastic Anemia Myelodysplastic syndrome = Acquired Sideroblastic Anemia Hemochromatosis = HFE gene mutation</p> Signup and view all the answers

    What is the primary function of hepcidin in the body?

    <p>Regulates iron absorption and release</p> Signup and view all the answers

    In anemia of chronic disease, hepcidin levels are typically decreased due to inflammation.

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

    What is the recommended dosage of iron sucrose for treating stage 3 iron deficiency?

    <p>200mg thrice daily</p> Signup and view all the answers

    Name one condition that can present as microcytic hypochromic anemia.

    <p>Thalassemia trait (minor)</p> Signup and view all the answers

    A deficiency of __________ in circulation is a consequence of increased hepcidin in anemia of chronic disease.

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

    Erythroid hyperplasia is associated with effective erythropoiesis.

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

    Match the following conditions with their blood picture description:

    <p>Iron deficiency anemia = Microcytic hypochromic Anemia of chronic disease = Normocytic normochromic (or microcytic hypochromic in severe cases) Sideroblastic anemia = Dimorphic (microcytic, macrocytic) Thalassemia trait = Microcytic hypochromic</p> Signup and view all the answers

    What follow-up evaluation should start to rise after 5-7 days following treatment?

    <p>Reticulocyte count</p> Signup and view all the answers

    Oral iron supplementation is typically resumed for __________ months after correction of iron deficiency.

    <p>6-12</p> Signup and view all the answers

    Match the type of iron treatment with the appropriate usage stage:

    <p>Parenteral Iron = Stage 3 iron deficiency Oral Iron = Latent stage of iron deficiency Iron sucrose = Given thrice daily Ferric carboxymaltose = 1g infusion</p> Signup and view all the answers

    What does a normal Red Cell Distribution Width (RDW) indicate?

    <p>Red blood cells are uniform in size</p> Signup and view all the answers

    The Mentzer index is used to primarily diagnose iron deficiency anemia.

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

    What parameter is typically assessed alongside red blood cell indices to evaluate iron deficiency anemia?

    <p>Serum ferritin</p> Signup and view all the answers

    The __________ index compares mean corpuscular volume to red blood cell count to differentiate between types of anemia.

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

    Match the following hematological indices with their significance:

    <p>RDW = Variation in red blood cell size Ferritin = Iron stores in the body MCV = Average volume of red blood cells Mentzer Index = Differentiation of anemia types</p> Signup and view all the answers

    Which of the following substances inhibits iron absorption?

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

    Heme iron is primarily found in plant sources such as spinach and potatoes.

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

    What is the primary substance that transports heme iron into the duodenum?

    <p>Heme carrier protein</p> Signup and view all the answers

    The storage form of iron in the body is __________.

    <p>Fe3+</p> Signup and view all the answers

    Match the following iron absorption components with their functions:

    <p>DMT1 = Transports ferrous iron across the luminal membrane Hepcidin = Regulates iron absorption Ceruloplasmin = Converts Fe3+ to Fe2+ Transferrin = Binds Fe3+ in the bloodstream</p> Signup and view all the answers

    What causes the presence of ring sideroblasts in sideroblastic anemia?

    <p>Iron surrounded by erythroblasts</p> Signup and view all the answers

    In thalassemia trait, serum iron indices typically show normal levels.

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

    What type of cellularity is typically observed in sideroblastic anemia?

    <p>Dimorphic (microcytic + macrocytic)</p> Signup and view all the answers

    In sideroblastic anemia, protoporphyrin is _______ and iron levels in the serum are _______.

    <p>absent, high</p> Signup and view all the answers

    Match the following components with their descriptions in regards to sideroblastic anemia:

    <p>ALA synthase = Deficiency leads to sideroblastic anemia Pappenheimer bodies = Found on electron microscopy TIBC = Typically low in sideroblastic anemia Transferrin saturation = High due to ineffective erythropoiesis</p> Signup and view all the answers

    Which clinical feature is associated with Plummer-Vinson syndrome?

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

    Koilonychia is a common clinical feature of iron deficiency.

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

    What therapy should be initiated after correcting iron stores in a patient with iron deficiency?

    <p>EPO therapy</p> Signup and view all the answers

    The formula for calculating the active space in iron deficit is ______.

    <p>3 x (Desired Hb - Patient's Hb) x Body weight + 1000 mg</p> Signup and view all the answers

    Match the following clinical features with their descriptions:

    <p>Angular cheilitis = Cracks at the corners of the mouth Median rhomboid glossitis = Red, smooth area at the mid-line of the tongue Atrophic glossitis = Loss of papillae on the tongue Oral candidiasis = Fungal infection in the mouth</p> Signup and view all the answers

    Study Notes

    Iron Metabolism

    • Iron Deficiency is a state where iron stores are low.
    • Iron Deficiency Anemia is a state where iron deficiency progresses to anemia.

    Iron Metabolism

    • Iron is absorbed in the duodenum.
    • Iron absorption is influenced by dietary factors.
    • Substances inhibiting iron absorption include phytates, tannins, and oxalates.
    • Heme iron (Fe2+) is absorbed through the heme carrier protein (HCP).
    • Non-heme iron (Fe3+) is absorbed through divalent metal transporter (DMT1).
    • Iron is stored as ferritin in the duodenum, bone marrow, and macrophages.
    • Iron is transported in the blood bound to transferrin.
    • Iron is taken up by erythroid marrow cells for heme synthesis.

    Iron Deficiency

    • Pre-latent Stage: Negative iron balance, low bone marrow iron, and low serum ferritin.
    • Latent Stage: Absent iron balance, low bone marrow iron, and low serum ferritin.
    • Iron Deficiency Anemia: Iron deficiency leading to anemia.
    • Earliest Indicator: Low ferritin level.
    • Most sensitive indicator: Increased soluble transferrin receptor level.
    • Most specific indicator: Soluble transferrin receptor (STFR).
    • Log ferritin is also an indicator.

    Iron Indices

    • Ferritin: 30- 300 ng/ml.
    • Iron: 50 - 150 µg/dL.
    • TIBC: 300 - 360 µg/dL.
    • TSAT: 33%.

    Iron Deficiency Anemia

    • Blood indices:
      • Decreased Serum iron: due to reduced absorption and circulation.
      • Increased TIBC: due to compensatory production of Transferrin and Soluble Transferrin Receptor (STFR).
      • Decreased TSAT: due to decreased serum iron and increased TIBC.
    • Peripherals:
      • Anisocytosis (variation in cell size)
      • Poikilocytosis (variation in cell shape)
      • Hypochromia (deficiency in hemoglobin content)
      • Pencil cells
      • Tear drop cells
    • Indices:
      • MCV: Normal to low
      • MCH: Normal to low
      • MCHC: Normal to low
      • RDW: > 14.5% (N: 11.5-14.5)
      • Mentzer index: > 13: Iron deficiency; < 13: Thalassemia trait.

    Iron Deficiency Anemia Treatment

    • Iron Supplementation: Stage 3 iron deficiency anemia
      • Parenteral Iron: Iron sucrose, iron isomaltose, ferric carboxymaltose.
      • Given intravenously or as an infusion.
    • Oral Iron: Latent stage of iron deficiency
      • 200 mg ferrous sulphate TID.
      • Continue for 6-12 months after correction.
    • Side effects of Iron Supplementation: Gastric intolerance.

    Iron Deficiency Anemia Follow Up

    • Reticulocyte count:
      • Rises after 5-7 days.
      • Peaks at 10-14 days.
    • Hb:
      • Starts to rise in 2 weeks.
      • Increase by 1 g/dL per month.

    Anemia of Chronic Disease

    • Pathophysiology: Inflammation causes increased hepcidin production, leading to iron trapping in stores and deficient iron in circulation.
    • Hepcidin: A peptide hormone produced in the liver that regulates iron absorption.
    • Factors influencing hepcidin Production:
      • Hypoxia: Inhibits hepcidin production, increases iron absorption.
      • HFE gene (chromosome 6), hemojuvelin, Tfra: Stimulates hepcidin production, decreases iron absorption, prevents iron overload.
    • Blood Picture:
      • Normocytic normochromic in mild cases.
      • Microcytic hypochromic in severe cases.
      • Dimorphic (microcytic, macrocytic) in some cases (e.g., rheumatoid arthritis).
    • Treatment Goals:
      • Address underlying inflammatory conditions.
      • Consider iron supplementation if needed.

    Sideroblastic Anemia

    • Causes:
      • Inherited: ALA synthase deficiency (X-linked).
      • Acquired: Myelodysplastic syndrome, copper deficiency, alcoholism, drugs, lead toxicity, etc.
    • Pathophysiology:
      • Inherited ALA synthase deficiency: Defective heme synthesis due to a malfunctioning ALA synthase enzyme.
      • Acquired sideroblastic anemia: Various factors can interfere with heme synthesis.
    • Blood Indices:
      • Increased Serum ferritin
      • Increased Serum iron
      • Decreased TIBC
      • Increased Transferrin saturation.
    • Treatment Goals:
      • Address the underlying cause.
      • Iron supplementation may be helpful in some cases.

    Thalassemia Trait (Minor)

    • Genetics: One normal beta chain and one defective beta chain.
    • Clinical Features
      • No ineffective erythropoiesis.
      • Normal iron indices.
      • Microcytic hypochromic anemia.

    Hematology (Bone Marrow)

    • Prussian blue stain: Absence of iron stores in iron deficiency.
    • Erythroid hyperplasia: Occurs due to ineffective erythropoiesis.

    Clinical Features of Iron Deficiency

    • Epithelial Lesions:
      • Oral Cavity: Angular cheilitis, oral candidiasis, median rhomboid glossitis, atrophic glossitis.
      • Hypopharynx: Plummer Vinson syndrome (dysphagia, esophageal webs), koilonychia, achlorhydria, atrophic gastritis, hyperactivity syndromes, growth and development defects, pica (pagophagia), mild splenomegaly, hair loss, worsening of congestive cardiac failure, neuropsychiatric manifestations.

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    Description

    This quiz covers essential concepts of iron metabolism, including absorption, storage, and transport mechanisms. It also details the stages of iron deficiency and its progression to anemia. Test your knowledge on how dietary factors influence iron levels and the role of various proteins in iron transport.

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