Anemias in Children — MCQs
A 29-year-old woman was found to have a hemoglobin level of 7.8 mg/dl, with a reticulocyte count of 0.8%. The peripheral blood smear showed microcytic hypochromic anemia. On high-performance liquid chromatography (HPLC), hemoglobin A2 and hemoglobin F were 2.4% and 1.3%, respectively. The serum iron and total iron-binding capacity (TIBC) were 15 mg/dl and 420 micrograms per deciliter, respectively. What is the most likely cause of anemia in this patient?
Which of the following statements is true regarding anemia of prematurity?
All of the following are characteristic features of treatment of iron deficiency anemia with oral iron supplements, except which of the following?
A child presenting with the following appearance is at risk of developing?
Which is NOT a feature of microangiopathic hemolytic anemia?
Which of the following is not a characteristic of Fanconi's anemia?
Normal reticulocyte count at birth is
Common age of presentation of Henoch-Schönlein purpura is?
Which of the following does not require a lumbar puncture in children?
A 5-year-old male child presents with episodic anaemia and jaundice since birth. He is least likely to have which of the following conditions?
Anemias in Children Indian Medical PG Practice Questions and MCQs
Question 1: A 29-year-old woman was found to have a hemoglobin level of 7.8 mg/dl, with a reticulocyte count of 0.8%. The peripheral blood smear showed microcytic hypochromic anemia. On high-performance liquid chromatography (HPLC), hemoglobin A2 and hemoglobin F were 2.4% and 1.3%, respectively. The serum iron and total iron-binding capacity (TIBC) were 15 mg/dl and 420 micrograms per deciliter, respectively. What is the most likely cause of anemia in this patient?
- A. Iron deficiency anemia (Correct Answer)
- B. Beta thalassemia minor
- C. Sideroblastic anemia
- D. Anemia due to chronic infection
Explanation: ### Iron deficiency anemia - The **low serum iron** level (15 mg/dl) and **high TIBC** (420 micrograms/dl) are classic indicators of iron deficiency. - Microcytic hypochromic anemia with a low reticulocyte count further supports this diagnosis, indicating insufficient **iron** for erythropoiesis [1]. [2] ### Beta thalassemia minor - Typically presents with **normal serum iron** levels, which is not consistent with the low iron levels seen here. - HPLC would show elevated hemoglobin A2, which is **not observed** in this patient's results. ### Sideroblastic anemia - Characterized by the presence of **ringed sideroblasts** in the bone marrow and often has normal or increased serum iron levels. - The iron levels reported here do not align with what is expected in sideroblastic anemia. ### Anemia due to chronic infection - Often associated with normocytic or mild microcytic anemia but does not typically lead to such **low serum iron** levels with high TIBC [1]. - This type typically presents with **inflammatory markers** and altered iron metabolism different from the findings in this case.
Question 2: Which of the following statements is true regarding anemia of prematurity?
- A. Low reticulocyte response (Correct Answer)
- B. Hemoglobin level <10 gm/dL
- C. 10 ml/kg packed cell transfusion
- D. Microcytic hypochromic type
Explanation: ***Low reticulocyte response*** - Anemia of prematurity results from several factors, including a **blunted erythropoietin response** to anemia, **shortened red blood cell lifespan**, and **rapid growth with increased blood volume requirements**. - The combination of these factors leads to **insufficient red blood cell production** by the bone marrow, reflected by a **low reticulocyte count** despite anemia. - This low reticulocyte response is a **key diagnostic feature** distinguishing it from hemolytic anemias. *Hemoglobin level <10 gm/dL* - While premature infants with anemia of prematurity develop low hemoglobin, a specific cutoff of **<10 gm/dL is not universally definitive** for diagnosis. - Hemoglobin nadirs vary based on **gestational age** (more premature = lower nadir) and occur at different postnatal ages. - Transfusion thresholds are determined by **clinical stability and symptoms**, not just a single Hb value. *10 ml/kg packed cell transfusion* - This describes a **treatment intervention**, not a characteristic of the disease itself. - Transfusion volume is typically **10-15 ml/kg** when indicated, but the decision to transfuse depends on gestational age, postnatal age, clinical stability, and symptoms like apnea or bradycardia. - This is **not a defining feature** of anemia of prematurity. *Microcytic hypochromic type* - Anemia of prematurity is typically **normocytic, normochromic**, not microcytic hypochromic. - **Microcytic hypochromic** anemia suggests **iron deficiency**, which is a different condition. - The red cells in anemia of prematurity have **normal size (MCV) and normal hemoglobin content** per cell.
Question 3: All of the following are characteristic features of treatment of iron deficiency anemia with oral iron supplements, except which of the following?
- A. Bioavailability is enhanced with vitamin C
- B. The proportion of iron absorbed reduces as hemoglobin improves
- C. The reticulocyte count should begin to increase within 7-10 days and peak at 2-4 weeks; this suggests good response to treatment
- D. The treatment should be discontinued immediately once hemoglobin normalizes to prevent side effects of iron (Correct Answer)
Explanation: ***The treatment should be discontinued immediately once hemoglobin normalizes to prevent side effects of iron*** - Treatment of **iron deficiency anemia** with oral iron supplements should continue for at least **3-6 months** after hemoglobin normalizes to replenish **iron stores**. - Premature cessation can lead to a rapid **recurrence of anemia** due to depleted iron reserves, despite normal hemoglobin levels. *Bioavailability is enhanced with vitamin C* - **Ascorbic acid (vitamin C)** creates an acidic environment in the stomach and reduces ferric iron (Fe3+) to ferrous iron (Fe2+), which is more readily absorbed. - This enhancement of **ferrous iron absorption** is a common practice to improve the efficacy of oral iron supplements. *The proportion of iron absorbed reduces as hemoglobin improves* - The body's **iron absorption mechanism** is tightly regulated by **hepcidin**, a hormone that increases when iron stores are sufficient. - As hemoglobin levels improve and iron stores are replenished, hepcidin levels rise, leading to a **decrease in iron absorption** to prevent iron overload. *The reticulocyte count should begin to increase in two weeks and peak in 4 weeks this suggests good response to treatment* - An increase in **reticulocyte count** by approximately **7-10 days** and peaking around **2-4 weeks** after starting iron therapy indicates that the bone marrow is effectively responding to the increased iron availability by producing new red blood cells. - This **reticulocytosis** is an early and reliable sign of a positive treatment response before a significant rise in hemoglobin is observed.
Question 4: A child presenting with the following appearance is at risk of developing?
- A. Immunodeficiency
- B. Lymphoproliferative disorder
- C. Gingivostomatitis
- D. Pure red cell aplasia (Correct Answer)
Explanation: ***Pure red cell aplasia*** - The image shows a "slapped cheek" rash, which is characteristic of **erythema infectiosum** (fifth disease) caused by **Parvovirus B19**. - Parvovirus B19 has a tropism for **erythroid progenitor cells** in the bone marrow, leading to a temporary cessation of red blood cell production, especially critical in individuals with underlying hemolytic anemias. *Immunodeficiency* - While viral infections can be more severe in immunodeficient individuals, the presented rash is specifically characteristic of **Parvovirus B19 infection**, not a general sign of immunodeficiency. - Immunodeficiency would typically involve recurrent, severe, or unusual infections, which are not directly indicated by the rash alone. *Lymphoproliferative disorder* - Lymphoproliferative disorders involve abnormal proliferation of lymphocytes and typically do not present with a "slapped cheek" rash. - Clinical signs would more likely include **lymphadenopathy**, hepatosplenomegaly, and cytopenias, not the classic facial rash seen here. *Gingivostomatitis* - **Gingivostomatitis** is an inflammation of the gums and oral mucosa, often caused by herpes simplex virus, presenting with **sores and ulcers in the mouth**. - The rash seen in the image is on the **cheeks and body**, not primarily oral, and is a classic presentation of erythema infectiosum.
Question 5: Which is NOT a feature of microangiopathic hemolytic anemia?
- A. Spherocytes (Correct Answer)
- B. High LDH
- C. Schistocytes
- D. Low haptoglobin
Explanation: ***Spherocytes*** - **Spherocytes** are typically found in conditions like **hereditary spherocytosis** or **autoimmune hemolytic anemia**, where red blood cells are damaged or improperly formed, leading to a spherical shape [1]. - In **microangiopathic hemolytic anemia (MAHA)**, red cells are fragmented by shear stress from damaged small blood vessels, resulting in **schistocytes**, not spherocytes. *High LDH* - **Lactate dehydrogenase (LDH)** is an intracellular enzyme released when red blood cells are destroyed, making **elevated LDH** a common finding in hemolytic anemias, including MAHA. - Its high levels reflect increased red cell turnover and destruction in the microvasculature. *Schistocytes* - **Schistocytes**, or fragmented red blood cells, are the **hallmark** of microangiopathic hemolytic anemia. - They are formed when red blood cells pass through damaged small blood vessels containing fibrin strands, leading to their shearing and destruction. *Low haptoglobin* - **Haptoglobin** is a plasma protein that binds free hemoglobin released during red blood cell destruction. - In hemolytic anemias like MAHA, there is increased hemoglobin release, which saturates and depletes haptoglobin, leading to **low or undetectable levels**. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 602-603.
Question 6: Which of the following is not a characteristic of Fanconi's anemia?
- A. Skeletal anomalies
- B. Pancytopenia
- C. Chromosome fragility
- D. Bone marrow failure in infancy (Correct Answer)
Explanation: ***Bone marrow failure in infancy*** - Fanconi anemia patients are typically **asymptomatic at birth** with normal blood counts. - **Progressive bone marrow failure** develops gradually, with median age of onset around **7 years** (range 5-10 years). - While subtle hematologic changes (macrocytosis, elevated HbF) may appear earlier, clinically significant **pancytopenia does not occur in infancy**. - This delayed hematologic presentation distinguishes Fanconi anemia from other congenital bone marrow failure syndromes. *Pancytopenia* - **Pancytopenia** is the hallmark hematologic feature of Fanconi anemia, but develops in **mid-childhood**, not infancy. - Results from progressive bone marrow failure affecting all three cell lines: **red blood cells, white blood cells, and platelets**. - Thrombocytopenia is often the first manifestation, followed by anemia and neutropenia. *Skeletal anomalies* - **Skeletal anomalies** are common congenital malformations present in approximately **60-75%** of patients. - Include **radial ray defects** (absent or hypoplastic thumbs, absent radius), **short stature**, and other limb abnormalities. - These are present from birth and often lead to early clinical suspicion. *Chromosome fragility* - **Chromosome fragility** is the **diagnostic hallmark** of Fanconi anemia due to defective DNA repair mechanisms. - Diagnostic test uses **diepoxybutane (DEB)** or **mitomycin C (MMC)** to induce DNA crosslinks, revealing increased chromosomal breaks and rearrangements. - This test is positive regardless of age or hematologic status.
Question 7: Normal reticulocyte count at birth is
- A. 2-6% (Correct Answer)
- B. 1-2%
- C. 6-10%
- D. 30-40%
Explanation: ***2-6%*** - At birth, the normal **reticulocyte count** is elevated primarily due to the physiological stress of **adapting to extrauterine life** and increased erythropoiesis. - This higher range reflects the body's need for a rapid turnover of red blood cells to meet oxygen demands after birth. *1-2%* - This range is considered a **normal reticulocyte count** for **older children and adults**, indicating a steady state of red blood cell production. - It does not account for the **physiological erythropoietic surge** observed in healthy neonates. *6-10%* - While higher than adult levels, a range of **6-10%** in a neonate would still be considered unusually high and might suggest **pathological hemolysis** or significant **blood loss**. - Without other supporting clinical signs, this level is typically higher than the **physiological norm**. *30-40%* - A reticulocyte count of **30-40%** at birth is **extremely elevated** and is highly indicative of a severe underlying condition such as **hemolytic disease of the newborn** or significant **neonatal hemorrhage**. - This level is far beyond the **normal physiological response** and requires urgent investigation.
Question 8: Common age of presentation of Henoch-Schönlein purpura is?
- A. < 10 years (Correct Answer)
- B. 10-15 years
- C. 15-20 years
- D. 20-30 years
Explanation: ***< 10 years*** - **Henoch-Schönlein purpura (HSP)** is predominantly a disease of **childhood**, with the majority of cases occurring in children under the age of 10. - The peak incidence is typically between **2 and 6 years of age**. *10-15 years* - While some cases of HSP can occur in this age group, it is **less common** than in younger children. - The incidence **decreases significantly** as age progresses beyond early childhood. *15-20 years* - HSP is **rare** in adolescents and young adults, though it can occur at any age. - When it does occur in older individuals, the disease presentation may be **more severe**. *20-30 years* - HSP is **uncommon** in this age range. - In adults, HSP is often associated with a higher risk of **renal involvement** and more chronic courses.
Question 9: Which of the following does not require a lumbar puncture in children?
- A. HL (Correct Answer)
- B. AML
- C. NHL
- D. ALL
Explanation: ***HL*** - While central nervous system (CNS) involvement is possible in Hodgkin lymphoma (HL), it is **rare** and does not routinely warrant a **lumbar puncture** for initial staging or surveillance in asymptomatic children. - HL primarily affects **lymph nodes** and the **spleen**, with CNS spread being an uncommon complication that typically presents with specific neurological symptoms. *AML* - **Acute myeloid leukemia (AML)** has a significant risk of **CNS involvement**, requiring a **lumbar puncture** for diagnostic staging and administration of intrathecal chemotherapy. - CNS prophylaxis and treatment are crucial in AML to prevent and manage **leptomeningeal disease**. *NHL* - **Non-Hodgkin lymphoma (NHL)**, particularly aggressive subtypes like Burkitt lymphoma or lymphoblastic lymphoma, has a **high propensity for CNS spread**. - A **lumbar puncture** is essential for staging to detect CNS involvement and guide the need for intrathecal chemotherapy or radiation. *ALL* - **Acute lymphoblastic leukemia (ALL)** carries a well-documented **high risk of CNS infiltration**, necessitating routine **lumbar punctures** at diagnosis for CNS staging and throughout treatment for intrathecal chemotherapy. - CNS prophylaxis is a cornerstone of ALL treatment to prevent **leptomeningeal relapse**.
Question 10: A 5-year-old male child presents with episodic anaemia and jaundice since birth. He is least likely to have which of the following conditions?
- A. Hereditary spherocytosis
- B. PNH (Correct Answer)
- C. G–6–PD deficiency
- D. Sickle cell anemia
Explanation: ***PNH*** - **Paroxysmal nocturnal hemoglobinuria (PNH)** is a rare, acquired clonal disorder that can present with anemia and jaundice; however, it is most commonly diagnosed in **adulthood** (median age 30-40 years). - While it can occur in children, presentation **since birth** is highly atypical for this acquired condition. *Hereditary spherocytosis* - This is an **inherited hemolytic anemia** characterized by defective red blood cell membrane proteins, leading to spherocytes that are prematurely destroyed. - It commonly presents with **episodic anemia and jaundice beginning in childhood**, often detected at birth or in early infancy. *Sickle cell anemia* - A **hereditary hemoglobinopathy** where red blood cells become sickle-shaped under low oxygen conditions, leading to chronic hemolysis, anemia, and vaso-occlusive crises. - Symptoms including **anemia and jaundice typically manifest in infancy** after the decline of fetal hemoglobin. *G–6–PD deficiency* - This is an **inherited enzyme deficiency** that makes red blood cells susceptible to oxidative damage, causing hemolytic anemia upon exposure to certain triggers (e.g., fava beans, certain drugs, infections). - Presentation with **episodic anemia and jaundice is common in childhood**, occurring when the child encounters oxidative stressors.
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