Hematology Practice Questions

Q: Common age of presentation of Henoch-Schönlein purpura is?

< 10 years. ***< 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.

Q: According to the WHO criteria, anaemia in infants of 6 months of age is defined as haemoglobin less than:

110 gm/litre. ***110 gm/litre*** - According to the **World Health Organization (WHO)** criteria, anaemia in infants aged **6-59 months** is defined as a haemoglobin concentration of less than 110 g/L (11.0 g/dL). - This threshold is used for **population-based screening** and **public health interventions** to identify and manage anaemia in young children. *100 gm/litre* - A haemoglobin level of less than 100 g/L (10.0 g/dL) in infants typically indicates **moderate to severe anaemia**, but the WHO threshold for defining anaemia in this age group is higher. - This value would represent a more pronounced degree of anaemia, often warranting immediate investigation and treatment. *105 gm/litre* - While 105 g/L is close to the threshold, the **WHO standard** for defining anaemia in infants aged 6-59 months specifically sets the cut-off at 110 g/L. - Using 105 g/L might underestimate the prevalence of anaemia or delay interventions for children who are considered anaemic by the official criteria. *115 gm/litre* - A haemoglobin level of 115 g/L (11.5 g/dL) in an infant of 6 months is generally considered **within the normal range** and does not meet the WHO criterion for anaemia. - This value would typically indicate a healthy haemoglobin status, and therefore, would not prompt a diagnosis of anaemia.

Q: 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?

PNH. ***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.

Q: Which of the following statements is true of hereditary spherocytosis?

About 50% of affected infants have moderately severe neonatal jaundice.. ***About 50% of affected infants have moderately severe neonatal jaundice*** - **Moderately severe neonatal jaundice** is indeed common in hereditary spherocytosis, affecting about 50% of infants. - The **increased breakdown of red blood cells** leads to elevated bilirubin levels, causing jaundice. *Infra vascular hemolysis is a common feature* - Hereditary spherocytosis mainly involves **extravascular hemolysis** rather than intravascular hemolysis [2][3]. - The destruction occurs in the **spleen** [2][4], which is not suitable for the classification of infra vascular hemolysis. *The disorder is usually due to autosomal recessive inheritance* - Hereditary spherocytosis is typically inherited in an **autosomal dominant** manner, not recessive. - Genetic mutations often affect proteins that maintain the **red blood cell membrane** [1], leading to spherocyte formation [4]. *Diagnosis can be made in neonatal period easily by examination of a blood film* - Diagnosis in the neonatal period often requires further testing beyond just a **blood film** [2], making it not straightforward. - Specific tests, such as the **osmotic fragility test** [2], are needed for definitive diagnosis rather than relying solely on blood films. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 640-641. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 597-598. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 639-640. [4] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 602-603.

Q: A 5-year-old child presents with non-blanching macules and papules on the skin. Skin biopsy reveals a perivascular IgA deposition. Which of the following is the most likely diagnosis?

Henoch-Schönlein purpura. ***Henoch-Schönlein purpura*** - This condition commonly presents in children with **non-blanching macules and papules** (palpable purpura), often on the lower extremities and buttocks. - Skin biopsy showing **perivascular IgA deposition** is the hallmark diagnostic finding for Henoch-Schönlein purpura. *Wegener's granulomatosis* - This is a **systemic vasculitis** (now called Granulomatosis with Polyangiitis) primarily affecting the respiratory tract and kidneys. - It is typically associated with **antineutrophil cytoplasmic antibodies (ANCA)**, specifically c-ANCA, and does not feature IgA deposition. *Kawasaki disease* - Characterized by **fever, rash, conjunctivitis, oral mucosal changes**, and **cervical lymphadenopathy** in young children. - While it is a form of vasculitis, it does not typically present with IgA deposition on skin biopsy and has a distinct clinical presentation. *Drug-induced vasculitis* - This condition is caused by an adverse reaction to a medication and can present with various skin lesions, including purpura. - Although it can cause vasculitis, the specific finding of **IgA deposition** is more characteristic of Henoch-Schönlein purpura than general drug-induced vasculitis.

Q: Which of the following is NOT a characteristic of Fanconi anemia?

Hematologic abnormalities in infancy. ***Hematologic abnormalities in infancy*** - Fanconi anemia typically does NOT present with hematologic abnormalities in infancy - this is why this option is correct for a "NOT" question. - The **median age of diagnosis** is between 6 and 8 years of age, when hematologic abnormalities become clinically apparent. - The characteristic **progressive bone marrow failure** with pancytopenia usually manifests in early-to-mid childhood, not in infancy. - While congenital physical abnormalities may be present at birth, the hematologic manifestations develop later. *Pancytopenia* - **Pancytopenia** is a hallmark of Fanconi anemia, as the disease causes progressive bone marrow failure. - This includes **anemia**, **leukopenia**, and **thrombocytopenia**, which typically develop in childhood and worsen over time. *Skeletal anomalies* - Various **skeletal abnormalities** are common in Fanconi anemia and present at birth. - These include malformations of the thumb (absent, hypoplastic, or supernumerary), radial aplasia, short stature, and abnormalities of the hips, spine, or kidneys. *Chromosome fragility* - **Chromosome fragility** is the diagnostic hallmark of Fanconi anemia. - Cells show increased chromosomal breakage, especially when exposed to DNA cross-linking agents (diagnostic test). - This chromosomal instability underlies the increased risk of myeloid malignancies and solid tumors.

Q: A 7-year-old boy presents with a palpable, non-blanching rash that started 3 days prior and involves the lower limbs and buttocks, following a previous viral upper respiratory tract infection. His blood pressure is normal, and kidney function tests are normal. What is the diagnosis?

Henoch-Schonlein purpura. ***Henoch-Schönlein purpura (IgA vasculitis)*** - The classic presentation of a **palpable, non-blanching purpuric rash** on the lower limbs and buttocks, especially after a viral upper respiratory infection in a child, is highly characteristic of Henoch-Schönlein purpura (HSP), now termed **IgA vasculitis**. - Although HSP can involve the kidneys, the **normal kidney function tests** and blood pressure here are consistent with early or mild disease, or prior to the development of renal manifestations which can occur later. - HSP is a **small vessel vasculitis** characterized by IgA immune complex deposition affecting skin, joints, GI tract, and kidneys. *Meningococcemia* - While meningococcemia can cause a **non-blanching rash**, it is typically accompanied by signs of severe illness such as **fever, altered mental status, and hemodynamic instability**, which are absent in this child. - The rash in meningococcemia often progresses rapidly to **petechiae and purpura**, but the distribution and association with a prior viral URI are less specific than in HSP. *Hemolytic uremic syndrome* - This condition involves the triad of **microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury**, often following a diarrheal illness (e.g., E. coli O157:H7). - The patient's **normal kidney function tests** and the rash pattern are not consistent with HUS; rash in HUS is not typically a prominent feature. *Cutis marmorata* - This is a harmless, temporary phenomenon characterized by a **lace-like, reticulated reddish-blue discoloration of the skin** that occurs in response to cold exposure due to vasomotor instability. - Unlike the described rash, cutis marmorata **blanches with pressure** and is not a palpable purpuric rash associated with a systemic illness.

Q: A two-year-old boy is brought to the emergency department with severe anemia (haemoglobin level of 2 gm%) and features of congestive heart failure. The most appropriate immediate therapy for this boy would be –

Packed cell transfusion. ***Packed cell transfusion*** - **Packed red blood cells (PRBCs)** can be used for severe anemia with congestive heart failure, but must be administered with extreme caution. - PRBCs should be given **very slowly** (over 4-6 hours) in small aliquots of **5-10 mL/kg** to avoid volume overload. - **Concurrent diuretic therapy** (furosemide) is essential to prevent worsening of heart failure. - This approach increases oxygen-carrying capacity while minimizing risk of pulmonary edema. *Partial exchange transfusion* - This is actually considered the **gold standard** for severe anemia (Hb <4 g/dL) with cardiovascular compromise in pediatrics. - It simultaneously removes blood while replacing it with PRBCs, maintaining **normovolemia** and preventing heart failure exacerbation. - While technically more complex, it is the **safest approach** as it avoids any net volume expansion. - The explanation that it's only for sickle cell crisis is **incomplete** - it's also indicated for severe symptomatic anemia with cardiac decompensation. *Whole blood transfusion* - **Whole blood** contains plasma and all cellular components, significantly increasing circulating volume. - This can worsen **fluid overload** and precipitate acute pulmonary edema in a child already in congestive heart failure. - Whole blood is generally avoided in this setting due to high risk of cardiac decompensation. *Parenteral iron injection* - **Parenteral iron** treats iron deficiency anemia by stimulating erythropoiesis over days to weeks. - It has **no role in emergency management** of severe anemia requiring immediate improvement in oxygen delivery. - Cannot address acute heart failure and would be negligent as sole immediate therapy.

Q: A child presents with seborrheic dermatitis, lytic skull lesions, ear discharge, and hepatosplenomegaly; what is the likely diagnosis?

Histiocytosis X. ***Histiocytosis X*** - This constellation of symptoms, including **seborrheic dermatitis-like rash**, **lytic bone lesions** (especially in the skull), **ear discharge** (often due to mastoid involvement), and **hepatosplenomegaly**, is highly characteristic of multisystem **Langerhans cell histiocytosis (LCH)**, previously known as Histiocytosis X. - LCH involves the proliferation of **Langerhans cells**, which are a type of dendritic cell, leading to organ infiltration and dysfunction. *Leukemia* - While leukemia can cause hepatosplenomegaly and occasional bone pain or lytic lesions, it typically presents with features like **anemia, thrombocytopenia, and lymphadenopathy**, and the prominent seborrheic dermatitis-like rash is not a hallmark. - The combination of **lytic skull lesions** and **ear discharge** points away from typical leukemia presentations. *Lymphoma* - Lymphoma primarily affects the **lymphatic system**, causing lymphadenopathy, hepatosplenomegaly, and B symptoms (fever, weight loss, night sweats). - It does not commonly present with **seborrheic dermatitis-like rashes** or the specific combination of **lytic skull lesions** and **chronic ear discharge** seen here. *Multiple myeloma* - Multiple myeloma is a **plasma cell malignancy** typically affecting older adults, not children, and presents with **bone pain, hypercalcemia, renal failure, and anemia**. - It involves **destructive bone lesions** but does not usually cause seborrheic dermatitis or ear discharge.

Question 1

Common age of presentation of Henoch-Schönlein purpura is?

Accepted Answer

< 10 years

Answer

10-15 years

Answer

15-20 years

Answer

20-30 years

Question 2

According to the WHO criteria, anaemia in infants of 6 months of age is defined as haemoglobin less than:

Accepted Answer

110 gm/litre

Answer

115 gm/litre

Answer

100 gm/litre

Answer

105 gm/litre

Question 3

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?

Accepted Answer

PNH

Answer

Hereditary spherocytosis

Answer

G–6–PD deficiency

Answer

Sickle cell anemia

Question 4

Which of the following statements is true of hereditary spherocytosis?

Accepted Answer

About 50% of affected infants have moderately severe neonatal jaundice.

Answer

The disorder is usually due to autosomal recessive inheritance.

Answer

Diagnosis can be made in the neonatal period easily by examination of a blood film.

Answer

Intravascular hemolysis is a common feature.

Question 5

A 5-year-old child presents with non-blanching macules and papules on the skin. Skin biopsy reveals a perivascular IgA deposition. Which of the following is the most likely diagnosis?

Accepted Answer

Henoch-Schönlein purpura

Answer

Wegener's granulomatosis

Answer

Kawasaki disease

Answer

Drug-induced vasculitis

Question 6

Which of the following is NOT a characteristic of Fanconi anemia?

Accepted Answer

Hematologic abnormalities in infancy

Answer

Chromosome fragility

Answer

Pancytopenia

Answer

Skeletal anomalies

Question 7

A 7-year-old boy presents with a palpable, non-blanching rash that started 3 days prior and involves the lower limbs and buttocks, following a previous viral upper respiratory tract infection. His blood pressure is normal, and kidney function tests are normal. What is the diagnosis?

Accepted Answer

Henoch-Schonlein purpura

Answer

Meningococcemia

Answer

Hemolytic uremic syndrome

Answer

Cutis marmorata

Question 8

A two-year-old boy is brought to the emergency department with severe anemia (haemoglobin level of 2 gm%) and features of congestive heart failure. The most appropriate immediate therapy for this boy would be –

Accepted Answer

Packed cell transfusion

Answer

Partial exchange transfusion

Answer

Whole blood transfusion

Answer

Parenteral iron injection

Question 9

A child presents with seborrheic dermatitis, lytic skull lesions, ear discharge, and hepatosplenomegaly; what is the likely diagnosis?

Accepted Answer

Histiocytosis X

Answer

Leukemia

Answer

Lymphoma

Answer

Multiple myeloma

Hematology — MCQs

Hematology — MCQs

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