Hematology Quiz: Splenomegaly & Hemolytic Anemia

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Questions and Answers

What is the most specific morphologic finding associated with hemolytic anemia?

Elliptocytosis

Acanthocytosis

Spherocytosis (correct)

Target cells

What is the treatment for conditions characterized by splenomegaly and hemolytic anemia?

Blood transfusion

Iron supplementation

Hormonal therapy

Splenectomy (correct)

Which condition is associated with increased osmotic fragility observed in the Pink test?

Thalassemia

Iron deficiency anemia

Sickle cell anemia

Hereditary spherocytosis (correct)

What is a key consequence of Glucose-6-Phosphate Dehydrogenase deficiency?

Oxidative injury leading to hemolysis Signup and view all the answers

Which of the following factors can increase oxidative stress and exacerbate G6PD deficiency?

Infections Signup and view all the answers

What is the significance of Heinz bodies in G6PD deficiency?

They can lead to membrane damage and hemolysis. Signup and view all the answers

Which vaccine is recommended for patients prone to infections due to splenectomy?

Pneumococcal vaccine Signup and view all the answers

What metabolic impact does a deficiency in G6PD cause?

Decreased NADPH production Signup and view all the answers

What is the chief feature of megaloblastic anemias?

Impaired DNA synthesis Signup and view all the answers

Which clinical feature is NOT associated with α-Thalassemia trait?

Severe anemia Signup and view all the answers

What does the presence of hypersegmented neutrophils indicate in megaloblastic anemia?

Diagnosis of megaloblastic anemia Signup and view all the answers

What is a common peripheral blood finding in megaloblastic anemias?

Macrocytic oval-shaped red cells Signup and view all the answers

Which condition is characterized as lethal in utero?

Hydrops fetalis Signup and view all the answers

What type of red cell variation is characteristic in megaloblastic anemias?

Anisocytosis and poikilocytosis Signup and view all the answers

What is the MCHC level in megaloblastic anemia?

Not elevated Signup and view all the answers

What genetic pattern is associated with the Silent carrier of α-Thalassemia?

−/α α/α Signup and view all the answers

What happens to HbS molecules when they become deoxygenated?

They undergo aggregation and polymerization. Signup and view all the answers

What is a vaso-occlusive crisis primarily caused by?

Blocking of small vessels by sickle cells. Signup and view all the answers

Which condition develops due to the irreversible sickling of red blood cells?

Extravascular hemolysis. Signup and view all the answers

What clinical feature is commonly associated with sickle cell anemia?

Severe hemolytic anemia. Signup and view all the answers

How does autosplenectomy affect the body?

It increases susceptibility to infections. Signup and view all the answers

What is one of the emergencies that can occur in sickle cell disease?

Acute chest syndrome. Signup and view all the answers

What is a common risk associated with the lack of a functional spleen?

Increased incidence of Salmonella osteomyelitis. Signup and view all the answers

Which symptom is associated with leg pain in sickle cell disease?

Vascular occlusion. Signup and view all the answers

What is iron primarily bound to for transportation after absorption?

Transferrin Signup and view all the answers

Which population is at the highest risk of iron deficiency due to increased requirement?

Pregnant women Signup and view all the answers

What dietary component can inhibit the absorption of inorganic iron?

Tannates Signup and view all the answers

Which condition is associated with impaired absorption of iron?

Fat malabsorption Signup and view all the answers

What is the most common cause of iron deficiency in the Western world?

Chronic blood loss Signup and view all the answers

How much iron must be absorbed daily to maintain a normal balance?

1 mg Signup and view all the answers

What factor enhances the absorption of iron from the diet?

Citric acid Signup and view all the answers

Which of the following is NOT a source of iron losses at equilibrium?

Nasal mucosa Signup and view all the answers

What is the primary cause of pernicious anemia?

Autoimmune gastritis affecting intrinsic factor production Signup and view all the answers

Which condition is associated with increased levels of serum bilirubin and LDH enzyme?

Ineffective erythropoiesis Signup and view all the answers

Which of the following is NOT a cause of folic acid deficiency?

Decreased intrinsic factor production Signup and view all the answers

What is a risk factor for atherosclerosis associated with vitamin B12 deficiency?

Increased levels of homocysteine Signup and view all the answers

What is a clinical difference between folic acid deficiency anemia and vitamin B12 deficiency anemia?

Folic acid deficiency does NOT cause neurological abnormalities Signup and view all the answers

What confirms the diagnosis of vitamin B12 deficiency anemia after treatment?

Outpouring of reticulocytes and rise in hematocrit Signup and view all the answers

What is the most common nutritional disorder in the world?

Iron deficiency anemia Signup and view all the answers

Which of the following is NOT associated with malabsorption of vitamin B12?

Decreased dietary intake of iron Signup and view all the answers

Study Notes

Splenomegaly and Hemolytic Anemia

Characterized by splenomegaly, mild to moderate hemolytic anemia, and increased bilirubin levels, leading to jaundice and pigment gallstones.
Chronic hemolysis raises the risk for acute red-cell aplasia, particularly from parvovirus B19.
Laboratory findings include increased osmotic fragility (Pink test) and elevated mean corpuscular hemoglobin concentration (MCHC).
Splenectomy is the recommended treatment; vaccinations for encapsulated organisms (e.g., pneumococcus, H. influenzae) are essential.

Morphology in Disorders

Spherocytosis is a notable morphologic feature, indicative but not exclusive to membrane loss conditions, such as autoimmune hemolytic anemia.

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Affects hexose monophosphate shunt and glutathione metabolism, resulting in red cell vulnerability to oxidative stress.
G6PD deficiency is a recessive X-linked condition, leading to diminished NADPH and reduced glutathione, causing oxidative injury and intravascular hemolysis.
Common stressors include fava beans, infections (viral hepatitis, pneumonia, typhoid), and specific drugs (antimalarials, sulphonamides).
Heinz bodies form due to oxidative stress, leading to hemolysis, as macrophages clear these inclusions in the spleen.

Sickle Cell Anemia

HbS becomes insoluble under low oxygen conditions, causing red cell sickling and potential vaso-occlusive crises.
Initially reversible sickling can progress to irreversible changes, leading to extensive hemolysis and complications.
Clinical manifestations include severe hemolytic anemia, erythroid hyperplasia, increased bilirubin levels, and gallstones.
Sickle cells cause vessel obstruction, leading to pain crises, autosplenectomy, and increased susceptibility to infections, particularly with encapsulated organisms.
Risk of complications includes Salmonella osteomyelitis, leg ulcers, and acute chest syndrome.

Thalassemias

Genetic classification includes α-thalassemia, with silent carriers and various severity levels, including hydrops fetalis, which is lethal without transfusions.

Megaloblastic Anemias

Caused by impaired DNA synthesis due to vitamin B12 or folate deficiencies, leading to delayed mitosis and macrocytic red blood cells.
Peripheral blood findings include hypersegmented neutrophils and low reticulocyte counts.
Elevated serum bilirubin and LDH levels may indicate hemolytic anemia features.

Vitamin B12 Deficiency (Pernicious Anemia)

An autoimmune condition impairs intrinsic factor production, essential for vitamin B12 absorption.
Additional causes of deficiency include achlorhydria and gastric surgery.
Diagnosis confirmed through reticulocyte response post vitamin B12 treatment; associated with increased gastric cancer risk.

Folic Acid Deficiency

Megaloblastic anemia caused by folate deficiency, without neurological involvement unlike vitamin B12 deficiency.
Major causes of folic acid deficiency include inadequate intake, increased requirements, and impaired utilization.

Iron Deficiency Anemia

The most prevalent nutritional deficiency worldwide; primarily caused by inadequate hemoglobin synthesis.
Iron metabolism involves absorption from the gut, transport via transferrin, and recycling post erythrocyte lifespan.
Causes include dietary lack, impaired absorption, increased needs, and chronic blood loss.
Heme iron is more efficiently absorbed than inorganic iron; absorption influenced significantly by dietary components.

Diagnostic Considerations

In adult men and postmenopausal women, iron deficiency is often attributed to gastrointestinal bleeding until proven otherwise.

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Description

This quiz covers crucial aspects of hematology related to splenomegaly and mild to moderate hemolytic anemia. Participants will learn about laboratory testing, manifestations of increased bilirubin, and conditions associated with parvovirus B19. Engage with questions focusing on osmotic fragility and MCHC measurements.