Hematology Practice Questions

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.

Q: In the context of Fanconi's anemia, which investigation is least relevant?

Echocardiography. ***Echocardiography*** - While Fanconi's anemia can rarely be associated with **cardiac anomalies**, these are not primary or consistent features, making routine echocardiography **less relevant** for diagnosis or monitoring compared to other mentioned investigations. - The core pathology involves **bone marrow failure** and genomic instability, which are better assessed by hematological and genetic tests. *Bone marrow examination* - This is a crucial investigation for Fanconi's anemia, as it directly assesses the bone marrow for **hypoplasia**, **dysplasia**, and changes consistent with impending **myelodysplastic syndrome (MDS)** or acute myeloid leukemia (AML) [1]. - It helps confirm the presence and severity of the **pancytopenia** and bone marrow failure [1]. *Platelet count* - **Thrombocytopenia** (low platelet count) is a common manifestation of Fanconi's anemia, reflecting the progressive **bone marrow failure**. - Monitoring platelet counts is essential for assessing disease progression and guiding **transfusion support** to manage bleeding risk. *Karyotyping* - **Karyotyping** (especially chromosomal breakage studies with mitomycin C or diepoxybutane) is the **gold standard diagnostic test** for Fanconi's anemia. - It identifies the characteristic **chromosomal instability** and breaks, confirming the underlying genetic defect.

Question 1

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 2

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

Question 3

In the context of Fanconi's anemia, which investigation is least relevant?

Accepted Answer

Echocardiography

Answer

Bone marrow examination

Answer

Platelet count

Answer

Karyotyping

Hematology — MCQs

Hematology — MCQs

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