Myelophthisic Anemias Overview

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Questions and Answers

What role does iron play after being separated from transferrin?

  • It is primarily excreted from the body.
  • It is used for producing hemoglobin or enzyme synthesis. (correct)
  • It aids in cellular breathing.
  • It is converted to transferrin.

How is excess iron stored in cells?

  • By binding to hemoglobin.
  • By binding to apoferritin, which transforms into ferritin. (correct)
  • By being excreted into the blood.
  • By converting to transferrin.

What is the average lifespan of a red blood cell (RBC)?

  • 150 days
  • 90 days
  • 120 days (correct)
  • 60 days

Which process primarily recycles iron in the body?

<p>Phagocytosis of red blood cells (D)</p> Signup and view all the answers

What is the daily iron requirement for adult men?

<p>1.0 mg (D)</p> Signup and view all the answers

What happens if an individual's iron absorption is lower than the body’s requirements?

<p>Bone marrow becomes hypoproliferative, causing microcytic hypochromic anemia. (C)</p> Signup and view all the answers

What is the main source of additional iron needed by the body?

<p>Dietary sources. (A)</p> Signup and view all the answers

What mechanisms are involved in iron loss from the body?

<p>Menstrual cycles, shedding of epithelial cells, and mucosal loss. (B)</p> Signup and view all the answers

What is the primary feature indicated by a leukoerythroblastic smear pattern in the diagnosis of myelofibrosis?

<p>Presence of nucleated red blood cells (B)</p> Signup and view all the answers

Which condition is NOT classified under the diseases causing secondary myelofibrosis?

<p>Hemophilia (D)</p> Signup and view all the answers

What is a characteristic of the anemia observed in patients with myelofibrosis?

<p>Normocytic and normochromic (B)</p> Signup and view all the answers

Which of the following processes occurs due to the proliferation of fibroblasts in the bone marrow?

<p>Myeloid metaplasia (B)</p> Signup and view all the answers

What is the impact of myelofibrosis on hematopoietic progenitor cells?

<p>Ineffective production of healthy blood cells (C)</p> Signup and view all the answers

What is a common early symptom of iron deficiency before anemia develops?

<p>Weakness and early fatigue (A)</p> Signup and view all the answers

Which group of diseases does NOT cause secondary myelofibrosis?

<p>Alzheimer's disease (B)</p> Signup and view all the answers

What is myeloid metaplasia?

<p>The occurrence of hematopoietic cells at extramedullary sites (A)</p> Signup and view all the answers

Which level of hemoglobin indicates that an individual may start experiencing symptoms of anemia?

<p>10 mg per deciliter (C)</p> Signup and view all the answers

In the context of myelofibrosis, what does pancytopenia indicate?

<p>Reduction in red, white, and platelet counts (B)</p> Signup and view all the answers

Which of the following conditions is characterized by strictures in the esophagus due to iron deficiency?

<p>Plummer-Vinson syndrome (D)</p> Signup and view all the answers

What does pica refer to in the context of iron deficiency?

<p>An abnormal craving for unusual food items (B)</p> Signup and view all the answers

Which symptoms are considered specific and more commonly associated with advanced stages of iron deficiency?

<p>Glossitis and angular stomatitis (B)</p> Signup and view all the answers

What is the function of cytochrome enzymes that can be affected by iron deficiency?

<p>Aiding in energy production (A)</p> Signup and view all the answers

What basic test is commonly used to diagnose iron deficiency anemia?

<p>Complete blood count (CBC) (C)</p> Signup and view all the answers

Which rare manifestation might be observed in an individual with iron deficiency?

<p>Koilonychia (spoon-shaped nails) (D)</p> Signup and view all the answers

What indicates a definitive diagnosis of iron deficiency anemia?

<p>A series of blood iron tests known as the iron profile (D)</p> Signup and view all the answers

What does a Transferrin Saturation (TS) value below 20% indicate?

<p>Iron deficiency (B)</p> Signup and view all the answers

Which of the following tests is NOT part of the iron profile used for diagnosing iron deficiency anemia?

<p>Measurement of hemoglobin (A)</p> Signup and view all the answers

What is a characteristic finding in hypochromic microcytic anemia observed in blood smears?

<p>Increased variation in the size of RBCs (anisocytosis) (D)</p> Signup and view all the answers

What are the normal ferritin levels for adult males in ng/mL?

<p>50-100 (D)</p> Signup and view all the answers

What happens to ferritin levels during the development of iron deficiency?

<p>Ferritin levels drop below 50 ng/mL (C)</p> Signup and view all the answers

Which type of anemia is characterized by small RBCs that are smaller than the nucleus of a lymphocyte?

<p>Microcytic anemia (A)</p> Signup and view all the answers

What does RDW indicate in the context of blood tests?

<p>Red blood cell volume distribution (C)</p> Signup and view all the answers

What is the primary role of iron in mammals?

<p>Oxygen transport (C)</p> Signup and view all the answers

Which protein in blood plasma is primarily responsible for transporting iron?

<p>Transferrin (C)</p> Signup and view all the answers

What form of transferrin has the highest affinity for its receptors?

<p>Diferric transferrin (D)</p> Signup and view all the answers

What is the average amount of iron lost by the human body daily?

<p>1 to 2 mg (A)</p> Signup and view all the answers

What is the main stored form of iron within cells?

<p>Ferritin (D)</p> Signup and view all the answers

What happens to iron-bound transferrin upon interaction with transferrin-specific receptors?

<p>It is internalized by the cell (C)</p> Signup and view all the answers

Which type of cells have the highest concentration of transferrin receptors?

<p>Developing erythroblasts (C)</p> Signup and view all the answers

Why is free iron considered toxic to the body?

<p>It reacts with free radicals (D)</p> Signup and view all the answers

What is a significant feature of patients with bone marrow fibrosis?

<p>Bone marrow is often 'tap dry' during aspiration attempts. (B)</p> Signup and view all the answers

What condition frequently leads to increased WBC counts and can result in a leukemoid reaction?

<p>Infections and inflammatory processes (A)</p> Signup and view all the answers

Which red blood cell characteristics can indicate iron deficiency anemia?

<p>Teardrop-shaped red blood cells and hypochromia (B)</p> Signup and view all the answers

What percentage of young women and adolescents in the United States is estimated to suffer from iron deficiency?

<p>2% to 5% (A)</p> Signup and view all the answers

What is the primary function of hemoglobin in red blood cells?

<p>To carry oxygen (C)</p> Signup and view all the answers

What should be investigated first when facing secondary myelofibrosis?

<p>Treatable underlying diseases (D)</p> Signup and view all the answers

Which of the following is a common cause of microcytic anemia?

<p>Iron deficiency (C)</p> Signup and view all the answers

What can be observed in the peripheral blood smear of patients with myelofibrosis?

<p>Presence of myeloblasts and promyelocytes (B)</p> Signup and view all the answers

Flashcards

Myelofibrosis

Fibrosis in the bone marrow, often seen in blood smears as leukoerythroblastosis.

Primary Myelofibrosis

A condition where bone marrow fibrosis is the primary disease, meaning it's not caused by another underlying condition.

Secondary Myelofibrosis

Bone marrow fibrosis that occurs as a secondary response to other diseases. For example, cancer, infections, or other blood disorders.

Myeloid Metaplasia

The movement of blood cell production from the bone marrow to other locations like the spleen, liver, and lymph nodes.

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Ineffective Erythropoiesis

Production of blood cells that are unhealthy and have a short lifespan, leading to ineffective blood production.

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Leukoerythroblastosis

A blood smear pattern that shows the presence of immature white blood cells and red blood cells, indicating problems with bone marrow function.

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Normocytic Normochromic Anemia

A type of anemia where blood cells are normal in size and color, often seen in cases of secondary myelofibrosis.

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Leukoerythroblastic Smear Pattern

A blood smear showing abnormal red blood cell shapes, including teardrop-shaped cells and nucleated red blood cells.

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Leukemoid Reaction

An increase in white blood cell count, often exceeding 30,000-50,000 cells per microliter, typically seen in patients with certain conditions like bone marrow fibrosis, chronic inflammatory diseases, or certain infections. It indicates an overproduction of white blood cells.

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Immature Myeloid Cells in Peripheral Blood

Immature myeloid lineage cells, such as myeloblasts, promyelocytes, and myelocytes, appearing in the peripheral blood, suggesting immature cells are being released from the bone marrow, often indicative of a bone marrow disorder or stress.

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Tap Dry Bone Marrow

A condition where the bone marrow is so fibrotic (scarred), making it difficult to obtain a bone marrow sample during aspiration. This is a characteristic sign of bone marrow fibrosis.

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Leukoerythroblastic Pattern

Abnormal red blood cell shapes, including teardrop-shaped cells, found in peripheral blood, often seen in conditions like myelofibrosis, indicating bone marrow dysfunction.

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Iron Deficiency Anemia

The most common form of anemia globally, often affecting young women and adolescents. It occurs when your body doesn't have enough iron to produce adequate red blood cells.

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RBC Function: Oxygen Transport

The primary role of red blood cells (RBCs) is to carry oxygen throughout the body using hemoglobin.

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Hemoglobin Synthesis Requirements

Hemoglobin, the oxygen-carrying molecule within red blood cells, requires three crucial components: globin protein, porphyrin ring, and iron to function optimally. Deficiencies in any of these components can lead to abnormal red blood cell production, causing anemia.

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Global Prevalence of Iron Deficiency Anemia

The prevalence of iron deficiency anemia is significantly high globally, affecting over 500 million people, highlighting the importance of addressing iron deficiency in public health efforts.

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Iron Distribution in the Body

The amount of iron in the body is approximately 4 grams, with 2100 mg in hemoglobin, 300 mg in myoglobin, 1000 mg stored in the liver, and 600 mg in the macrophage system of the bone marrow and spleen.

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Why is Iron Important for Oxygen Transport?

Iron's ability to easily gain and lose electrons (changing between Fe²⁺ and Fe³⁺) makes it essential for oxygen transport. This process involves binding and releasing oxygen.

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How is Iron Transported in the Blood?

Iron is transported in the blood by transferrin, a protein that can bind two iron atoms. This binding is important for regulating iron levels in the body.

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Transferrin's Affinity for Receptors

Iron-bound transferrin has a higher affinity for its receptors, while apotransferrin (without iron) has a lower affinity. This ensures that iron is delivered to cells that need it the most.

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Iron Release from Transferrin

When iron-bound transferrin binds to its receptor, it's internalized by the cell. In an acidic endosome, iron is released from transferrin.

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Iron Deficiency in Developing Countries

Iron deficiency is more prevalent in developing countries due to food scarcity and gastrointestinal parasites. These factors contribute to reduced iron absorption and increased iron loss.

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Iron Storage and Toxicity

Free iron is toxic to the body, so it's stored and transported in a bound form, primarily with ferritin within cells.

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Iron Cycle Overview

The iron cycle involves the absorption of iron from food, its release from the reticuloendothelial system, and its binding to transferrin. This ensures iron is available for use by the body.

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Iron Cycle

The process where iron is taken up by cells, used for functions like hemoglobin synthesis, and then recycled back into the bloodstream.

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Transferrin

A protein that binds to iron in the bloodstream, transporting it to cells that need it.

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Transferrin Receptor

A receptor found on the surface of cells that binds to transferrin, allowing iron to enter the cell.

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Ferritin

The protein that stores excess iron inside cells, preventing harmful buildup.

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Red Blood Cell Phagocytosis

The process where old red blood cells are broken down by specialized cells in the body.

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Daily Iron Recycling

The amount of iron that is recycled back into the bloodstream each day from broken down red blood cells.

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Hypoproliferative Bone Marrow

A condition where the bone marrow produces fewer red blood cells than normal due to insufficient iron absorption.

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Microcytic Hypochromic Anemia

A type of anemia where the red blood cells are smaller than normal and have less color due to iron deficiency.

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Plummer-Vinson Syndrome

A rare symptom of iron deficiency anemia characterized by difficulty swallowing due to strictures and webs in the esophagus.

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Pica

The abnormal craving for non-food items like ice, dirt, or starch, a common symptom associated with iron deficiency.

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Glossitis

An inflammation of the tongue, often appearing red and losing its papillae. A symptom commonly seen in iron deficiency.

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Angular Stomatitis

Cracks at the corners of the mouth, often observed in advanced stages of iron deficiency.

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Koilonychia

Spoon-shaped nails, a rare manifestation of iron deficiency.

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Pale Sclerae

Pale sclera (whites of the eyes), a rare symptom seen in iron deficiency.

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Complete Blood Count (CBC) Test

A blood test that measures red blood cell count, hemoglobin, hematocrit, and other parameters to diagnose anemia.

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Microcytic Anemia

The presence of smaller than normal red blood cells (RBCs) in a blood sample.

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Hypochromic Anemia

A type of anemia where the red blood cells are paler than normal, indicating a reduced amount of hemoglobin.

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Serum Iron

A blood test that measures the amount of iron in the blood. It's used to assess iron deficiency.

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TIBC (Total Iron Binding Capacity)

A blood test that measures the total amount of iron that can be bound by transferrin, a protein that carries iron in the blood. It gives an idea of the blood's iron-carrying capacity.

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Anisocytosis

A blood smear analysis looking for variations in the size of red blood cells. This can indicate anemia.

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RDW (Red Blood Cell Distribution Width)

A blood test that measures the variation in the size of red blood cells. This can point towards iron deficiency anemia.

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Iron Profile

A panel of blood tests used to diagnose iron deficiency. This panel includes tests like serum iron, TIBC, and ferritin.

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Study Notes

Myelophthisic Anemias

  • Fibrosis in bone marrow can be primary (primary myelofibrosis or myeloid metaplasia) or secondary (myelophthisis or secondary myelofibrosis)
  • Secondary myelofibrosis can be caused by various factors.
    • Cancerous conditions: Metastatic cancers (breast, lung, prostate, neuroblastomas)
    • Infectious diseases: Mycobacterial infections (tuberculosis, atypical mycobacteria), fungal infections, HIV, sarcoidosis
    • Hematologic disorders: Multiple myeloma, lymphomas, hairy cell leukemia, chronic myeloid leukemia (CML)

Pathophysiology of Bone Marrow Fibrosis

  • Fibroblast proliferation: Myelofibrosis is caused by extensive fibroblast proliferation within the bone marrow, directly impacting hematopoiesis.
  • Extramedullary hematopoiesis: Hematopoietic cells spread to other organs (long bones, spleen, liver, lymph nodes). This process is known as myeloid metaplasia.
  • Ineffective erythropoiesis: Despite high proliferation, erythrocytes are inefficiently produced and have a reduced lifespan. This often leads to various cytopenias (e.g., pancytopenia).

Anemia in Myelofibrosis

  • Anemia is frequently a prominent feature of secondary fibrosis.
  • Anemia is generally normocytic and normochromic.
  • Peripheral blood smears show leukoerythroblastosis (nucleated red blood cells, teardrop-shaped red blood cells, and various abnormal RBC shapes).
  • White blood cell (WBC) counts can be elevated (leukemoid reaction, which can range from 30,000-50,000). Immature myeloid cells (myeloblasts, promyelocytes, myelocytes) may also be seen in the peripheral blood.
  • Platelet counts are sometimes significantly elevated and platelets display enlarged size.

Bone Marrow Biopsy

  • Bone marrow biopsies in cases of fibrosis are often "tap dry", meaning it is difficult to aspirate marrow due to extensive fibrosis.

Treatment

  • Treatment depends on the underlying cause of secondary myelofibrosis.
  • Treatable diseases (infections) should be targeted
  • Blood transfusions may be required to alleviate symptoms.

Iron Deficiency Anemia

  • RBCs are responsible for carrying oxygen, requiring hemoglobin, which needs iron.
  • Iron deficiency can lead to hypochromic microcytic anemia.
  • Symptoms include fatigue, pallor and weakness.
  • Diagnosed via blood tests that assess iron levels (e.g., serum iron, TIBC, ferritin).

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