Hematopathology Practice Questions

Q: The following test is performed for which condition?

Paroxysmal nocturnal haemoglobinuria. ***Paroxysmal nocturnal haemoglobinuria*** - The image depicts the **Ham test**, also known as the acid serum lysis test, which is historically used to diagnose **paroxysmal nocturnal hemoglobinuria (PNH)**. - In PNH, red blood cells are abnormally susceptible to complement-mediated lysis in acidified serum, leading to **hemolysis** as seen in the test tube with red fluid. *Hemoglobinopathy* - Hemoglobinopathies are characterized by **abnormal hemoglobin structures** or synthesis, such as sickle cell anemia or thalassemia. - While they can cause hemolytic anemia, their diagnosis involves **hemoglobin electrophoresis** or genetic testing, not the Ham test. *Alkaptonuria* - Alkaptonuria is a rare genetic disorder of metabolism affecting the breakdown of **tyrosine and phenylalanine**, leading to accumulation of homogentisic acid. - It results in **dark urine** upon standing and pigmentation of connective tissues (ochronosis), and is not diagnosed by tests for hemolysis. *Paroxysmal cold haemoglobinuria* - This condition is characterized by **hemolysis following cold exposure**, mediated by anti-P cold agglutinin antibodies. - Diagnosis involves the **Donath-Landsteiner test**, which demonstrates hemolysis at cold temperatures and warming, rather than the Ham test.

Q: All are true about the blood component shown below except?

Random donor platelets are produced by apheresis. ***Random donor platelets are produced by apheresis*** - **Random donor platelets** are typically prepared from **whole blood donations** by centrifugation, not apheresis. - **Apheresis** is a process used to collect specific blood components, such as platelets, from a single donor, resulting in a single-donor apheresis platelet unit, not random donor platelets. *Infusion must be completed within 30 minutes* - The recommended infusion time for platelets is generally **30-60 minutes**. - Rapid infusion (under 30 minutes) is usually not recommended and could potentially lead to adverse reactions or compromise platelet viability. *Use of leukocyte filters helps in reducing allosensitization* - **Leukocyte reduction** significantly reduces the number of white blood cells in platelet products. - This helps to decrease the risk of **febrile non-hemolytic transfusion reactions** and **alloimmunization** (allosensitization) to HLA antigens. *Two units of transfused platelets per square meter body surface area are anticipated to increase the platelet count by approximately 10,000/μL* - This statement accurately describes the expected **platelet increment** from standard random donor platelet transfusions. - The increment can vary based on factors such as patient condition and consumption, but this is a general guideline for adequate response.

Q: Which of the following will be the most probable diagnostic finding in the patient with X-Ray skull shown below?

CD1A positivity. ***CD1A positivity*** - The skull X-ray shows multiple punched-out lytic lesions without sclerotic borders, characteristic of **Langerhans Cell Histiocytosis (LCH)**. - **Langerhans cells** in LCH express surface markers like **CD1a** and S100 protein, which are diagnostic when detected via **immunohistochemistry on tissue biopsy**. - CD1a positivity is the **gold standard** for confirming LCH diagnosis. *Elevated SAP* - **Serum Alkaline Phosphatase (SAP)** is typically elevated in conditions with increased osteoblastic activity or liver/biliary disease. - LCH lesions are generally **lytic** and not associated with increased osteoblastic activity, thus SAP elevation is not a specific or common finding. *Bence Jones proteins* - **Bence Jones proteins** (monoclonal light chains) are found in the urine of patients with **Multiple Myeloma**, which can also cause lytic bone lesions. - However, the image shows more discrete, "punched-out" lesions typical of LCH, whereas myeloma lesions are often more diffuse or "salt-and-pepper" in appearance, and LCH does not secrete Bence Jones proteins. *Elevated HbA2* - **Elevated HbA2** is a characteristic finding in **beta-thalassemia trait**, a genetic blood disorder. - There is no clinical or radiological indication in the provided image or question to suggest a connection to thalassemia.

Q: A 10-year-old Sindhi boy presents with recurrent episodes of bone pain. Specimen shows:

Spleen infarction. ***Spleen infarction*** - The image shows a **wedge-shaped, pale area** within the spleen, consistent with an **infarct**. Splenic infarctions are common in conditions leading to **vaso-occlusion**, such as **sickle cell disease**. - A 10-year-old Sindhi boy with **recurrent bone pain** suggests a **hemoglobinopathy**, particularly sickle cell disease (or a related condition like **thalassemia-sickle beta**), where red blood cells can obstruct splenic blood vessels leading to infarction. *Liver hematoma* - A **hematoma** is a collection of blood, often caused by trauma or hemorrhage. The image depicts a **necrotic area** (infarct) rather than a blood clot within the liver. - While liver issues can occur in some hematologic disorders, the characteristic wedge shape points away from a simple hematoma. *Heart infarction* - A **heart infarction** (myocardial infarction) affects the heart muscle. The specimen in the image is clearly an **abdominal organ**, identifiable as the spleen due to its characteristic shape and internal architecture. - The clinical presentation of bone pain is not directly indicative of a heart infarction. *Adrenal hemorrhage* - **Adrenal hemorrhage** would involve bleeding within the adrenal glands, which are small glands located on top of the kidneys. The organ shown in the image is much larger and shaped like a spleen. - Bone pain is not a typical symptom of adrenal hemorrhage.

Q: The image shows:

Thalassemia. ***Thalassemia*** - The image on the left shows a child with features suggestive of **thalassemia major**, specifically **frontal bossing** (prominent forehead), wide-set eyes, and potentially **malar prominence** due to **extramedullary hematopoiesis** in the facial bones. This patient also has abdominal distension, likely due to **hepatosplenomegaly**. - The peripheral blood smear on the right is characteristic of thalassemia, showing **microcytic hypochromic cells**, target cells, and **nucleated red blood cells** (erythroblasts) which indicate severe ineffective erythropoiesis and high red blood cell turnover. *Hereditary spherocytosis* - This condition is characterized by the presence of **spherocytes** (small, dense, spherical red blood cells without central pallor) on the peripheral blood smear. - While hereditary spherocytosis can cause anemia and some similar clinical features, the **marked dysmorphic features** of the child and the specific red cell morphology (especially target cells and nucleated RBCs, not spherocytes) visible in the smear are more indicative of thalassemia. *Autoimmune hemolytic anemia* - This condition can lead to anemic symptoms and an enlarged spleen, but the peripheral smear typically shows **spherocytes** and sometimes **polychromasia** from reticulocytosis, with a positive direct antiglobulin test (DAT). - The facial dysmorphism and the presence of numerous target cells and nucleated red blood cells are not typical for autoimmune hemolytic anemia. *G6PD* - G6PD deficiency results in episodes of **hemolytic anemia** triggered by oxid ative stress, such as certain drugs, fava beans, or infections. - The peripheral smear during a hemolytic crisis might show **Heinz bodies** (which require a special stain) and **bite cells** due to oxidant damage, but the chronic facial changes and the characteristic target cells/nucleated RBCs seen in the image are not typical for G6PD deficiency.

Q: A 6-year-old Afro-American boy presents with acute abdomen and nonhealing ulcer on medial malleolus. Peripheral smear is shown. The most likely cause of this condition is:

Substitution of single amino acid base. ***Substitution of single amino acid base*** - The image shows numerous **sickle cells** (elongated, crescent-shaped red blood cells), which are characteristic of **sickle cell anemia**. - Sickle cell anemia is caused by a **point mutation** (a single amino acid substitution) in the beta-globin gene, where valine replaces glutamic acid at the sixth position. *Trinucleotide repeat* - **Trinucleotide repeat disorders** (e.g., Huntington's disease, Fragile X syndrome) involve an abnormal expansion of three-nucleotide sequences. - They present with neurological or developmental symptoms, not the hematological features of sickle cell disease. *Antibody to RBC membrane* - **Antibodies to RBC membrane** cause conditions like **autoimmune hemolytic anemia**, where red blood cells are destroyed by the immune system. - While it can cause anemia, the characteristic **sickle shape** of the red blood cells, as seen in the smear, is not a feature of autoimmune hemolytic anemia. *Genomic imprinting* - **Genomic imprinting** is an epigenetic phenomenon where certain genes are expressed in a parent-of-origin-specific manner (e.g., Prader-Willi syndrome). - This mechanism is not involved in the pathogenesis of sickle cell disease.

Q: A 16-year-old female with pallor and hepatosplenomegaly presents to your clinic. Peripheral smear shows:

Thalassemia intermedia. ***Thalassemia intermedia*** - The peripheral smear shows significant **anisopoikilocytosis** with numerous **target cells** (indicated by the arrowhead), teardrop cells, and some irregularly contracted cells, which are characteristic of thalassemia. - The patient's presentation with **pallor** and **hepatosplenomegaly** (due to extramedullary hematopoiesis and increased red cell destruction) in a 16-year-old female points towards a chronic hemolytic anemia like thalassemia intermedia. *Iron-deficiency anemia* - While it causes **microcytic hypochromic anemia**, the peripheral smear would typically show predominantly small, pale red cells and not the extensive target cells, teardrop cells, or significant anisopoikilocytosis seen in the image. - **Hepatosplenomegaly** is not a common finding in uncomplicated iron-deficiency anemia. *Autoimmune hemolytic anemia* - This condition typically presents with **spherocytes** (small, dense red cells lacking central pallor) due to immune-mediated destruction, and sometimes **agglutination**, which are not the predominant findings in this smear. - While it can cause anemia and splenomegaly, the distinct red cell morphology in the image is not typical for AIHA. *Megaloblastic anemia* - The classic peripheral smear findings for megaloblastic anemia are **macrocytic red blood cells** and **hypersegmented neutrophils**, neither of which are observed in this image. - The presence of target cells and other poikilocytes seen here excludes megaloblastic anemia.

Q: A 7-year-old boy presents with fever and weight loss. On examination he had pallor with lymphadenopathy. Peripheral smear is shown below. Diagnosis is: (AIIMS May 2017)

ALL. ***ALL*** - The peripheral smear shows numerous **blasts** with **scanty cytoplasm**, **prominent nucleoli**, and a **high nuclear-to-cytoplasmic ratio**, which is characteristic of **acute lymphoblastic leukemia (ALL)**. - Clinical features such as **fever**, **weight loss**, **pallor**, and **lymphadenopathy** in a 7-year-old child are classic presentations of ALL, the most common childhood leukemia. *AML* - While AML also presents with acute symptoms and blasts, the blasts in AML typically have **more abundant cytoplasm**, and may contain **Auer rods**, which are not clearly visible here. - The **morphology of the blasts** in the image, with their uniformly high N/C ratio and immature appearance, points away from typical AML. *Aplastic anemia* - Aplastic anemia is characterized by **pancytopenia** in the peripheral blood and **hypocellular bone marrow**, meaning a significant reduction in all blood cell lines, and **lacks the presence of blasts**. - The image clearly displays a proliferation of immature cells (blasts), which is contrary to the pathology of aplastic anemia. *Juvenile myelomonocytic leukemia* - JMML is a rare disorder characterized by **monocytosis**, **splenomegaly**, and typically presents in very young children (median age 2 years), often with **rashes** and **adenopathy**. - While there is some overlap in symptoms, the predominant cell type in the smear does not suggest a significant monocytic component, and the clinical picture in a 7-year-old child is less typical for JMML without other defining features.

Q: A patient presents with a solitary bone lesion. Histopathological examination shows the following microscopic features. What is the most likely diagnosis?

Unifocal Langerhans cell histiocytosis. ***Unifocal Langerhans cell histiocytosis*** - This condition is characterized by a single lesion of **Langerhans cells**, often presenting as a **lytic bone lesion** in children or young adults [1]. - It is the most benign form of Langerhans cell histiocytosis and typically has an excellent prognosis after local treatment. *Monoclonal Gammopathy of unknown significance* - MGUS is an asymptomatic condition characterized by the presence of a **monoclonal protein** in the blood without evidence of multiple myeloma or related disorders. - It does not typically present with **lytic bone lesions** or symptoms of bone pain. *Multiple myeloma* - Multiple myeloma is a malignant proliferation of **plasma cells** in the bone marrow [3], leading to widespread **lytic bone lesions** [2,4], hypercalcemia, renal failure, and anemia. - While it causes lytic lesions, the presentation is usually more systemic and aggressive than a single bone lesion [4]. *Paget's disease* - Paget's disease is a chronic bone disorder characterized by abnormal bone remodeling, leading to enlarged and weakened bones. - It typically presents with **bone pain**, deformities, and increased bone turnover markers, but the lesions are often **sclerotic** or mixed, not purely lytic, and it's more common in older adults. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, p. 630. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, p. 608. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 606-607. [4] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 616-617.

Question 1

The following test is performed for which condition?

Accepted Answer

Paroxysmal nocturnal haemoglobinuria

Answer

Hemoglobinopathy

Answer

Alkaptonuria

Answer

Paroxysmal cold haemoglobinuria

Question 2

A 3-year-old child presents with bleeding from nose and Periorbital Ecchymosis. Sternal tenderness and bone pain is present. Peripheral smear shows presence of fragmented RBC and helmet cells. The most probable diagnosis is? (Recent NEET Pattern 2016-17)

Accepted Answer

Acute myeloid Leukemia

Answer

Acute Idiopathic thrombocytopenic purpura

Answer

Hemophilia A

Answer

Battered baby syndrome

Question 3

All are true about the blood component shown below except?

Accepted Answer

Random donor platelets are produced by apheresis

Answer

Infusion must be completed within 30 minutes

Answer

Use of leukocyte filters helps in reducing allosensitization

Answer

Two units of transfused platelets per square meter body surface area are anticipated to increase the platelet count by approximately 10,000/μL

Question 4

Which of the following will be the most probable diagnostic finding in the patient with X-Ray skull shown below?

Accepted Answer

CD1A positivity

Answer

Elevated SAP

Answer

Bence Jones proteins

Answer

Elevated HbA2

Question 5

A 10-year-old Sindhi boy presents with recurrent episodes of bone pain. Specimen shows:

Accepted Answer

Spleen infarction

Answer

Liver hematoma

Answer

Heart infarction

Answer

Adrenal hemorrhage

Question 6

The image shows:

Accepted Answer

Thalassemia

Answer

Hereditary spherocytosis

Answer

Autoimmune hemolytic anemia

Answer

G6PD

Question 7

A 6-year-old Afro-American boy presents with acute abdomen and nonhealing ulcer on medial malleolus. Peripheral smear is shown. The most likely cause of this condition is:

Accepted Answer

Substitution of single amino acid base

Answer

Trinucleotide repeat

Answer

Antibody to RBC membrane

Answer

Genomic imprinting

Question 8

A 16-year-old female with pallor and hepatosplenomegaly presents to your clinic. Peripheral smear shows:

Accepted Answer

Thalassemia intermedia

Answer

Iron-deficiency anemia

Answer

Autoimmune hemolytic anemia

Answer

Megaloblastic anemia

Question 9

A 7-year-old boy presents with fever and weight loss. On examination he had pallor with lymphadenopathy. Peripheral smear is shown below. Diagnosis is: (AIIMS May 2017)

Accepted Answer

ALL

Answer

AML

Answer

Aplastic anemia

Answer

Juvenile myelomonocytic leukemia

Question 10

A patient presents with a solitary bone lesion. Histopathological examination shows the following microscopic features. What is the most likely diagnosis?

Accepted Answer

Unifocal Langerhans cell histiocytosis

Answer

Monoclonal Gammopathy of unknown significance

Answer

Multiple myeloma

Answer

Paget's disease

Hematopathology — MCQs

Hematopathology — MCQs

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