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What role does iron play after being separated from transferrin?
What role does iron play after being separated from transferrin?
How is excess iron stored in cells?
How is excess iron stored in cells?
What is the average lifespan of a red blood cell (RBC)?
What is the average lifespan of a red blood cell (RBC)?
Which process primarily recycles iron in the body?
Which process primarily recycles iron in the body?
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What is the daily iron requirement for adult men?
What is the daily iron requirement for adult men?
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What happens if an individual's iron absorption is lower than the body’s requirements?
What happens if an individual's iron absorption is lower than the body’s requirements?
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What is the main source of additional iron needed by the body?
What is the main source of additional iron needed by the body?
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What mechanisms are involved in iron loss from the body?
What mechanisms are involved in iron loss from the body?
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What is the primary feature indicated by a leukoerythroblastic smear pattern in the diagnosis of myelofibrosis?
What is the primary feature indicated by a leukoerythroblastic smear pattern in the diagnosis of myelofibrosis?
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Which condition is NOT classified under the diseases causing secondary myelofibrosis?
Which condition is NOT classified under the diseases causing secondary myelofibrosis?
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What is a characteristic of the anemia observed in patients with myelofibrosis?
What is a characteristic of the anemia observed in patients with myelofibrosis?
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Which of the following processes occurs due to the proliferation of fibroblasts in the bone marrow?
Which of the following processes occurs due to the proliferation of fibroblasts in the bone marrow?
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What is the impact of myelofibrosis on hematopoietic progenitor cells?
What is the impact of myelofibrosis on hematopoietic progenitor cells?
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What is a common early symptom of iron deficiency before anemia develops?
What is a common early symptom of iron deficiency before anemia develops?
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Which group of diseases does NOT cause secondary myelofibrosis?
Which group of diseases does NOT cause secondary myelofibrosis?
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What is myeloid metaplasia?
What is myeloid metaplasia?
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Which level of hemoglobin indicates that an individual may start experiencing symptoms of anemia?
Which level of hemoglobin indicates that an individual may start experiencing symptoms of anemia?
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In the context of myelofibrosis, what does pancytopenia indicate?
In the context of myelofibrosis, what does pancytopenia indicate?
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Which of the following conditions is characterized by strictures in the esophagus due to iron deficiency?
Which of the following conditions is characterized by strictures in the esophagus due to iron deficiency?
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What does pica refer to in the context of iron deficiency?
What does pica refer to in the context of iron deficiency?
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Which symptoms are considered specific and more commonly associated with advanced stages of iron deficiency?
Which symptoms are considered specific and more commonly associated with advanced stages of iron deficiency?
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What is the function of cytochrome enzymes that can be affected by iron deficiency?
What is the function of cytochrome enzymes that can be affected by iron deficiency?
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What basic test is commonly used to diagnose iron deficiency anemia?
What basic test is commonly used to diagnose iron deficiency anemia?
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Which rare manifestation might be observed in an individual with iron deficiency?
Which rare manifestation might be observed in an individual with iron deficiency?
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What indicates a definitive diagnosis of iron deficiency anemia?
What indicates a definitive diagnosis of iron deficiency anemia?
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What does a Transferrin Saturation (TS) value below 20% indicate?
What does a Transferrin Saturation (TS) value below 20% indicate?
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Which of the following tests is NOT part of the iron profile used for diagnosing iron deficiency anemia?
Which of the following tests is NOT part of the iron profile used for diagnosing iron deficiency anemia?
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What is a characteristic finding in hypochromic microcytic anemia observed in blood smears?
What is a characteristic finding in hypochromic microcytic anemia observed in blood smears?
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What are the normal ferritin levels for adult males in ng/mL?
What are the normal ferritin levels for adult males in ng/mL?
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What happens to ferritin levels during the development of iron deficiency?
What happens to ferritin levels during the development of iron deficiency?
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Which type of anemia is characterized by small RBCs that are smaller than the nucleus of a lymphocyte?
Which type of anemia is characterized by small RBCs that are smaller than the nucleus of a lymphocyte?
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What does RDW indicate in the context of blood tests?
What does RDW indicate in the context of blood tests?
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What is the primary role of iron in mammals?
What is the primary role of iron in mammals?
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Which protein in blood plasma is primarily responsible for transporting iron?
Which protein in blood plasma is primarily responsible for transporting iron?
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What form of transferrin has the highest affinity for its receptors?
What form of transferrin has the highest affinity for its receptors?
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What is the average amount of iron lost by the human body daily?
What is the average amount of iron lost by the human body daily?
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What is the main stored form of iron within cells?
What is the main stored form of iron within cells?
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What happens to iron-bound transferrin upon interaction with transferrin-specific receptors?
What happens to iron-bound transferrin upon interaction with transferrin-specific receptors?
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Which type of cells have the highest concentration of transferrin receptors?
Which type of cells have the highest concentration of transferrin receptors?
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Why is free iron considered toxic to the body?
Why is free iron considered toxic to the body?
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What is a significant feature of patients with bone marrow fibrosis?
What is a significant feature of patients with bone marrow fibrosis?
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What condition frequently leads to increased WBC counts and can result in a leukemoid reaction?
What condition frequently leads to increased WBC counts and can result in a leukemoid reaction?
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Which red blood cell characteristics can indicate iron deficiency anemia?
Which red blood cell characteristics can indicate iron deficiency anemia?
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What percentage of young women and adolescents in the United States is estimated to suffer from iron deficiency?
What percentage of young women and adolescents in the United States is estimated to suffer from iron deficiency?
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What is the primary function of hemoglobin in red blood cells?
What is the primary function of hemoglobin in red blood cells?
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What should be investigated first when facing secondary myelofibrosis?
What should be investigated first when facing secondary myelofibrosis?
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Which of the following is a common cause of microcytic anemia?
Which of the following is a common cause of microcytic anemia?
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What can be observed in the peripheral blood smear of patients with myelofibrosis?
What can be observed in the peripheral blood smear of patients with myelofibrosis?
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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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Description
This quiz explores the causes and pathophysiology of myelophthisic anemias, focusing on both primary and secondary myelofibrosis. Learn about the various cancerous conditions, infectious diseases, and hematologic disorders that contribute to this condition, as well as the impact on bone marrow and blood cell production.